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Mitral Annular Disjunction in Advanced Myxomatous Mitral Valve Disease: Echocardiographic Detection and Surgical Correction
Abstract
Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus described by pathologists in association with mitral leaflet prolapse and defined as a separation between the atrial wall-mitral valve (MV) junction and the left ventricular attachment allowing for hypermobility of the MV apparatus. The transesophageal echocardiographic characteristics of this abnormality have not been previously described. In patients undergoing MV repair for myxomatous MV degeneration and evaluated using a standardized transesophageal echocardiographic protocol, annular disjunction (mean value 10 +/- 3 mm) was seen at the base of the posterior leaflet in 98% of patients with advanced, and in 9% of patients with mild/moderate MV degeneration. There was a significant correlation between the magnitude of disjunction and the number of segments with prolapse/flail (r = 0.397, P = .001). We found annular disjunction to be a common component of MV apparatus in advanced MV degeneration. Its recognition on transesophageal echocardiography is important to facilitate optimal MV repair. The modification of the repair technique allows surgical correction of the annular disjunction, which seems to optimize long-term results in these challenging cases.
... MAD can be assessed through various imaging modalities, such as echocardiography, computed tomography (CT), and magnetic resonance (MR) imaging [8,[10][11][12][18][19][20][21][22][23][24][25], with CT offering the advantage of detailed, high-resolution evaluation of the entire circumference of the mitral valve annulus [22,23,26]. In this study, we sought to determine the prevalence and morphological features of both a-MAD and v-MAD using three-dimensional reconstructions of the cardiac CT images. ...
... For instance, in transthoracic echocardiography, a misalignment ≥2 mm measured in systole was sufficient to diagnose the presence of a-MAD [8,25]. In transesophageal echocardiography, MAD was diagnosed if there was a wide separation of ≥5 mm in two-dimensional [18,28] and threedimensional studies [10]. For the cardiac MR or cardiac CT imaging, previous studies do not indicate any cut-off point, defining any displacement of the mitral leaflet hinge line as MAD, or using a 1 mm threshold [12,[20][21][22][23][24]. ...
... Finally, recognizing MAD, whether atrial or ventricular, seems crucial for patients undergoing mitral valve surgery. In patients with annular disjunctions, modifying the surgical technique may be necessary to avoid prosthetic valve replacement and ensure an optimal and long-lasting outcome of the repair [18,19]. ...
... MAD was identified as high as 92%, although little attention was paid by then as MAD was deemed with no significant clinical consequence. Till 2005, Eriksson et al reported that the recognition of MAD prior to MV repair [2], with modification of the surgical techniques to correct the annular disjunction, were essential to achieve durable long-term results. Subsequently, MAD started to gain more clinical interest as it became more frequently recognized by echocardiography. ...
... In transoesophageal echocardiogram (TEE), the degree of annular disjunction could be measured in the four-chamber mid-esophageal view at 0° during systole. When applying a threshold of ≥ 5 mm by Eriksson et al [2], MAD was detected in 98% of patients with advanced Barlow's disease, and the severity of the disjunction was significantly correlated with the number of prolapsing segments. ...
... The recognition of MAD is imperative in the surgical planning in MV surgery. With modification of repair techniques, complete resolution of MAD can be achieved in most patients after MV operation [2]. The annuloplasty ring or band should be sutured to affix the mitral annulus to the correct level of the myocardium in the LV, to obliterate the gap caused by the disjunction, rather than to be attached to LA wall (Fig. 2). ...
Mitral annular disjunction is a cardiac structural abnormality characterized by the distinct separation between the top of the left ventricular myocardium and the mitral annulus supporting the posterior mitral leaflet occurring during systole. It has recently gained wide attention due to the increasing recognition of the link between mitral annular disjunction and arrhythmogenic mitral valve prolapse, particularly, with the increased risks of ventricular arrhythmias resulting in sudden cardiac death. This review has summarized the recent progress in the diagnostic modalities, clinical implications of mitral annular disjunction, and its specific surgical considerations.
... It can occur in a 360 degree arc around the mitral valve annulus, but is most often visualized in the region of the infero-lateral myocardium directly under the posterior mitral valve leaflet, typically in the region of the P1 and P2 mitral valve scallops [1]. The diagnosis of MAD can reliably be made on non-invasive imaging modalities including transthoracic echocardiography, transoesophageal echocardiography, and cardiac magnetic resonance imaging [11,13,14]. On transthoracic echocardiography, this is most commonly seen in the parasternal long-axis view and to a lesser extent from the apical four chamber view, three chamber view and two-chamber view in the systolic phase. ...
... MAD distance was measured in the parasternal long-axis view at end-systole as the distance between the posterior scallop insertion into the LA wall and the systolic bulge of the ventricular myocardium [21] (Fig. 1). A disjunction distance of > 5mm was defined as mitral annular disjunction [13]. There is no universally accepted cut off for MAD, with variability in reporting between studies and modalities. ...
... Previous studies have suggested that MAD is associated with an increased risk of ventricular arrhythmias [8,9,11]. It has been postulated that areas of MAD generate mechanical stress of the papillary muscles and ventricular endocardium, which over time can cause myocardial fibrosis in some patients [13]. A recent large cohort study by Essayah et al. demonstrated MAD presence with MVP is associated with progressive excess incidence of clinical arrhythmic events after diagnosis without an increase in mortality within the first 10 years, and they highlighted the importance of arrhythmia monitoring in the follow up of these patients [21]. ...
Background
There is a paucity of literature regarding outcomes of patients with mitral valve prolapse (MVP) and mitral annular disjunction (MAD) after mitral surgery, with many unanswered questions including the post-surgical persistence of MAD, effect of MAD on mitral valve reparability, and incidence of arrhythmia after mitral valve surgery. We aimed to examine the prevalence, imaging characteristics and clinical associations of mitral annular disjunction among patients undergoing mitral valve surgery for mitral valve prolapse, as well as outcomes after surgery including the persistence of MAD, arrhythmic events and excess mortality.
Results
A retrospective analysis of 111 consecutive patients who underwent mitral valve surgery for MVP was performed. A total of 32 patients (28.8%) had MAD. Patients with MAD were younger (64 vs 67 yrs, p = 0.04), with lower rates of hypertension (21.9% vs 50.6%, p = 0.01) and hyperlipidaemia (25% vs 50.6%; p = 0.01) and were more likely to be female (43.8% vs 21.4%, p = 0.04) with myxomatous leaflets > 5mm (90.6% vs 15.2%, p = < 0.01) and bileaflet prolapse (31.3% vs 10.1%, p = 0.02). Mitral valve repair was performed in 29/32 patients (90.6%) in the MAD positive group, and no patients had the persistence of MAD post-surgery. Post-operative ventricular arrhythmia was higher in the MAD positive group (28.13% vs 11.69%, p = 0.04) with no difference in mortality, 30-day hospital re-admission, or post-operative mitral regurgitation between patients with and without MAD over 3.91 years of follow up.
Conclusion
In this study of consecutive patients with MVP undergoing surgery, MAD was a common finding (almost 1 in 3). MAD does not compromise mitral valve surgical reparability, and both repair and replacement are effective at correcting disjunction. Our data suggest that concurrent MAD in MVP patients undergoing surgery has no significant effect on post surgical outcomes. Further research as to whether this patient cohort requires post-surgical arrhythmia monitoring is warranted.
... Mitral valve prolapse (MVP) is the most frequent cause of primary or degenerative mitral regurgitation (MR) with estimated prevalence up to 3% in the general population (1,2). Mitral annulus disjunction (MAD) is frequently coexistent with MVP, although the reported prevalence of MAD varies due to the different imaging modalities, various cutoffs, heterogeneous subpopulations, and various MR-severity grades (3)(4)(5). ...
... The prevalence of MAD among patients with MVP varies between 20% and 58% (3,9,10). In a certain subset of patients with myxomatous MVP, MAD prevalence varies between 21.8% and 98% (3)(4)(5). In patients with MAD, the reported presence of MVP was 78% (6). ...
... The cornerstone for the diagnosis of MAD is cardiac imaging modalities. MAD can be detected easily in the long-axis views during end-systole using transthoracic echocardiography (TTE) (3)(4)(5)10), and cardiac magnetic resonance (CMR) (13). As an adjunctive, cardiac computed tomography (CT) can also diagnose MAD. ...
Mitral annulus disjunction (MAD) is defined as a systolic displacement between the ventricular myocardium and the posterior mitral annulus supporting the posterior mitral leaflet. This structural abnormality is associated with the loss of mechanical annular function manifested as an abnormal systolic excursion of the leaflet hinge point into the left atrium but with maintained electrical function, separating the left atrium and ventricle electrophysiologically. The mitro-aortic fibrous continuity limits MAD anteriorly, between the aortic cusps and the anterior leaflet of the mitral valve. Consequently, MAD has been observed only at the insertion of the posterior leaflet. It can extend preferentially at the central posterior scallop. The first diagnostic modality aiding the diagnosis is transthoracic echocardiography (TTE), although in some cases adjunctive cardiac imaging modality might be suggested. MAD carries a strong association with malignant ventricular arrhythmogenesis and a profound predisposition for sudden cardiac death (SCD). In this context, a thorough investigation of this morphological and functional abnormality is vital in estimating the risk assessment and stratification for optimal management and elimination of the risk of the patient for SCD. Based on the current scientific data and literature, we will discuss the diagnosis, clinical implications, risk stratification, and therapeutic management of MAD.
... In MAD, there is a discontinuation between the attachment of the mitral valve leaflet and the myocardium of the left ventricular (LV) free wall ("ventricular crest") ( Fig. 1) [24,25]. Although the original description is often credited to Hutchins et al. [24], other authors already documented this anatomic alteration several years before [26][27][28][29]. ...
... Transthoracic echocardiograpgy (TTE) is the most widely used imaging modality to assess MAD, usually in a parasternal long-axis view [10 ••, 21,23,[34][35][36]. However, MAD may affect up to two-thirds of the annular circumference, with regions of disjunction interspersed with normal tissue [24,25,30,[37][38][39][40]. Therefore, assessing MAD only in the standard parasternal long-axis view may not capture its true extent, and measuring its longitudinal distance alone may ignore the relevance of the circumferential extent of the disjunction with respect to outcome. ...
... The increased spatial resolution of intraoperative transoesophageal echocardiography (TOE), cardiac CT, or cardiac magnetic resonance imaging (CMR) has allowed a better visualization of the mitral annulus. However, different imaging modalities have yielded different cutoffs which may not be interchangeable [24,25,[41][42][43]. For instance, Dejgaard et al. reported a near complete concordance between CMR and TTE for the presence/absence of MAD, but a moderate correlation (R = 0.47, p < 0.001) for absolute longitudinal MAD distance between CMR vs. TTE [21]. ...
Purpose of Review
To provide an overview of mitral annular disjunction (MAD) and to discuss important challenges in diagnosis and management of MAD.
Recent Findings
MAD has regained interest in the context of sudden cardiac death (SCD) in patients with mitral valve prolapse (MVP), coined as the “arrhythmic” MVP syndrome. In addition, MAD in isolation was recently suggested to be associated with severe arrhythmia and SCD.
Summary
There is a lack of consensus on the definition of MAD and the imaging modality to be used for diagnosing MAD, and the therapeutic implications of MAD remain uncertain. Furthermore, the exact mechanism underlying the association of MAD with SCD remains largely unexplored.
... Mitral annular disjunction (MAD) is an anatomic abnormality which refers to a wide separation between the left atrial wall-mitral valve junction and the basal posterolateral ventricular myocardium that has been recently associated with malignant arrhythmias and sudden cardiac death (SCD) [1][2][3][4][5]. MAD as a sole entity as well as in the setting of the broader classification of "malignant mitral valve prolapse (MVP) syndrome" are considered potentially arrhythmogenic entitles [6]. ...
... MAD has been reported to exist in 6% of normal hearts, as a normal anatomic variant [1]. The first echocardiographic description comes from Erikson et al. [2] in 2005 where MAD was observed by transesophageal echocardiography in 99% of patients, referred for surgical repair for advanced mitral valve (MV) disease. ...
... In pathological studies, MAD has been mainly related to MVP with floppy mitral valve and myxomatous degeneration rather than FED [1,13,14]. MAD can be easily detected by transthoracic echocardiography (TTE) [2], and measured in the parasternal long axis view during endsystole (Fig. 1b). It mainly affects the area under the posterior mitral leaflet, which is more prone to mechanical stretch as opposed to the more rigid aorto-mitral continuity [14]. ...
Although mitral valve prolapse (MVP) is usually considered a benign clinical condition, it has been linked with ventricular arrhythmias and sudden cardiac death in patients with a certain “arrhythmic” phenotype, raising awareness and mandating a specific risk stratification protocol. Mitral annular disjunction (MAD) is considered a “red flag” in malignant MVP syndrome along with bileaflet myxomatous prolapse, female gender, negative or biphasic T waves in the inferior leads, fibrosis in the papillary muscles or inferobasal wall detected by cardiac magnetic resonance imaging and complex arrhythmias of right bundle branch morphology. MAD seems to play a critical role in the chain of morphofunctional abnormalities which lead to increased mechanical stretch and subsequent fibrosis mainly in the papillary muscles, forming the vulnerable anatomic substrate prone to arrhythmogenesis, and associated with long-term severe ventricular arrhythmias. Arrhythmogenesis in MVP/MAD patients is not fully understood but a combination between a substrate and a trigger has been established with premature ventricular contraction triggered ventricular fibrillation being the main mechanism of sudden cardiac death (SCD). Certain characteristics mostly recognized by non-invasive imaging modalities serve as risk factors and can be used to diagnose and identify high risk patients with MAD, while treatment options include catheter ablation, device therapy and surgical intervention. This review focuses on the clinical presentation, the arrhythmogenic substrate, and the incidence of ventricular arrhythmias and SCD in MAD population. The current risk stratification tools in MAD arrhythmogenic entity are discussed.
... Barlow's disease is a form of degenerative MV disease characterized by myxomatous thickening, billowing and prolapse of the mitral leaflets and pronounced annular dilation, often leading to significant MR (20). The association between Barlow's disease and MAD was first described more than two decades ago, and since then multiple studies have demonstrated a high prevalence of MAD in the setting of Barlow's disease (19,21,22). Lee et al. showed that MAD was associated with more diffuse myxomatous MV disease with more severe mitral leaflet deformity and larger leaflet areas and billow height and volume (9). ...
... Carmo et al. described the recognition of MAD by TTE using the length of the annular disjunction during end-systole on parasternal long axis view, which was defined as the measurement from the junction of the left atrial wall and MV posterior leaflet to the top of the LV posterior wall (19). MAD has also been described based upon intraoperative TEE, and was defined as a separation between the P2 insertion into the left atrial wall and the atrial/ventricular attachment performed in a 4-chamber mid-esophageal view (22). ...
... Mitral transcatheter edge-to-edge repair (TEER) with the MitraClip device was approved in the United States in 2013 for the treatment of primary MR. Surgical mitral valve repair can repair MAD in almost all patients likely due to the annuloplasty ring suture joining the mitral annulus to the LV myocardium and bridging the MAD gap, however this structural correction does not occur with TEER (7,13,22). Additionally, as mentioned above, although data on a large cohort of patients is lacking, smaller studies have shown improvement in ventricular arrhythmias after mitral valve surgery for MVP. It is unclear whether TEER provides the same benefit in this setting, however prior studies have shown a reduction in ventricular arrhythmias after MitraClip placement for secondary or functional MR (52, 53). ...
Mitral annular disjunction (MAD) is an abnormal displacement of the mitral valve leaflet onto the left atrial wall and is commonly found in patients with mitral valve prolapse (MVP). The diagnosis is usually made by transthoracic echocardiography (TTE) although findings can be subtle and further cardiac imaging may be necessary. MAD has been associated with a risk of malignant ventricular arrhythmias and sudden cardiac death, therefore recognition of this diagnosis and risk stratification are highly important. In this review, we will discuss the diagnosis, clinical implications, risk stratification and management of MAD based upon currently available literature, as well as provide a series of cases showing the heterogeneity in presentation and our experience with management of this rare but potentially fatal entity.
... The MAD trench is relatively easy to recognize in long-axis views by transthoracic-Echocardiography [42][43][44][45] or CMR. [45][46][47] Cardiac CT Scan can also diagnose MAD. ...
... The reported prevalence of MAD varies partly due to the different imaging modalities, various cut-offs, heterogeneous subpopulations, and various MR-severity grades. 42,43,45,[59][60][61] Amongst patients with MVP, the prevalence of MAD varies between 20 and 58% (reported pooled rate of 32.6% from three studies). 14,42,48,[61][62][63][64] In the specific subset of patients with myxomatous MVP, MAD prevalence varies between 21.8% and 98% (pooled rate of 50.8% from 3 studies). ...
... 14,42,48,[61][62][63][64] In the specific subset of patients with myxomatous MVP, MAD prevalence varies between 21.8% and 98% (pooled rate of 50.8% from 3 studies). 14,43,44,61,64,65 In patients with MAD, reported presence of MVP was 78%. 46 In syndromic MVP, MAD prevalence is reported between 34% 66 in patients with Marfan syndrome and 40% in carriers of Loeys-Dietz syndrome. ...
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Risk stratification scheme. Risk stratification aiming at assessing the risk of VAs and SCD in patients with MVP, involving two phases based on the clinical and imaging context and the uncovered arrhythmia. In the absence of ventricular tachycardia, phenotypic risk features will trigger the intensity of screening for arrhythmia. Green boxes indicate green heart consensus statements and yellow boxes indicate yellow heart consensus statements. High risk - sustained VT, polymorphic NSVT, fast (>180 bpm) NSVT, VT/NSVT resulting in syncope. ICD = implantable cardioverter defibrillator; LA = left atrium; LGE = late gadolinium enhancement; LV-EF = left ventricular ejection fraction; MAD = mitral annular disjunction; MV = mitral valve; PVCs = premature ventricular contractions; TWI = T-wave inversion; VT = ventricular tachycardia. #Additional ECG monitoring method may be used such as loop recorders.
... Mitral valve repair or mitral valve replacement in patients with severe mitral valve disease is associated with good long term patient outcomes [5,6]. However, less favourable surgical outcomes have been reported in patients who have excessive mobility of the mitral leaflet or apparatus [7]. Echocardiography represent an effective method of evaluating MAD and mitral valve disease as this imaging modality has a good balance of temporal and spatial resolution. ...
... We found that MAD is common in patients undergoing mitral valve surgery which has been described before. Eriksson et al. reported a much higher prevalence of MAD of 97% in advanced myxomatous mitral valve disease and 9% in mild to moderate myxomatous mitral valve disease [7]. The difference in rate in Eriksson et al. likely related to the difference in the evaluated population. ...
... The current study includes all aetiologies for mitral valve disease requiring surgery rather than advanced myxomatous mitral valve disease alone and the current study used TTE rather than transoesophageal echocardiography. Our study results are more consistent with the 38% rate of ≥ 5 mm MAD evaluated by TTE in a cohort of 64 patients with Barlow's disease undergoing mitral valve surgery reported by Hiemstra et al [5] and the rate of 35% in 89 patients with mitral valve prolapse detected on cardiac magnetic resonance imaging described by Essayagh et al. [10] To the best of our knowledge only one study by Eriksson et al. [7] has investigated patients with and without MAD pre and post mitral valve surgery. Whilst there were similarities between Eriksson et al. [7] and our evaluation in terms patient gender (66% male vs 72.2% male) and MAD length (10 ± 3 mm vs 8.4 ± 3.9 mm). ...
Background
Mitral annular disjunction (MAD) is a structural abnormality characterized by the distinct separation of the mitral valve annulus/left atrium wall and myocardium. Little is known about the significance of MAD in patients requiring mitral valve surgery. This evaluation evaluates the echocardiographic characteristics and patient outcomes for patients with and without MAD who require mitral valve surgery.
Methods
All patients who underwent mitral valve surgery and who had a pre-surgical transthoracic echocardiogram between 2013 and 2020 were included. Patient demographics and clinical outcomes were collected on review of patient electronic records.
Results
A total of 185 patients were included in the analysis of which 32.4% had MAD (average MAD length 8.4 mm). MAD was seen most commonly in patients with mitral valve prolapse and myxomatous mitral valves disease (90% and 60% respectively). In the patients with MAD prior to mitral valve surgery, only 3.9% had MAD post mitral valve surgery. There were no significant difference in the severity of post-operative mitral regurgitation, arrhythmic events or major adverse cardiovascular events in patients with and without MAD.
Conclusions
MAD is common in patients who undergo mitral valve surgery. Current surgical techniques are able to correct the MAD abnormality in the vast majority of patients. MAD is not associated with an increased risk of adverse clinical outcomes post mitral valve surgery.
... Results comparing subgroups of patients according to the presence or absence of MAD and/or MVP are reported in Table 3. Of the 2611 patients, 90 (3.4%) patients had MAD without 30 [27][28][29][30][31][32][33] 27 [25][26][27][28][29][30] .057 ...
... We found a MAD length greater than 5 mm to be associated with arrhythmic risk (OR 3.96, 95% CI: 1.93, 8.15; P < .001). To date, a definite cutoff for pathologic MAD length has not been established, with several previous attempts yielding values ranging from 4.8 mm (10) to 10 mm (26). Specifically, a MAD length greater than 8.5 mm has previously been linked to nonsustained ventricular tachycardia (27), even in patients without mitral valve regurgitation (10). ...
Mitral annular disjunction (MAD) was a frequent incidental finding at cardiac MRI examinations performed for various clinical indications; longer MAD length and coexisting bileaflet mitral valve prolapse were associated with a higher prevalence of arrhythmia.
... MAD is characterized by a separation of the mitral valve annulus-left atrial wall and the basal portion of the posterolateral ventricular myocardium. It can have a variable distance ranging from a few millimeters (mm) to more than 10 mm [1]. It is common in myxomatous mitral valve disease and MVP [2]. ...
... The role of surgical intervention in this cohort remains limited to those patients with concomitant severe MR. The feasibility of mitral valve repair with MAD was first reported by Erikksson and colleagues in a 2005 study where transesophageal echocardiography was used to diagnose MAD and assess the mitral valve apparatus [1]. A more contemporary study reported on mitral valve repair in 60 patients with MVP (27 with MAD) and found no difference in surgical characteristics (bypass, clamp times, post-operative MR etc.) between MAD and no MAD groups [48]. ...
Purpose of Review
The purpose of this manuscript is to provide a comprehensive review for the clinician regarding the epidemiology, pathophysiology, diagnosis, and management of patients with mitral valve prolapse and disjunction. We will start the case with an illustrative case which consolidates these points.
Recent Findings
After providing a historical background, we will also update the reader on newer studies in approaching catheter ablation of ventricular arrhythmias in these patients and the impact of surgical repair on outcomes. We then provide our own framework and risk stratification schema to observe, treat, and manage this heterogeneous patient cohort.
Summary
The association of mitral valve dysplasia and ventricular arrhythmias and sudden cardiac death has long been recognized. The most important high-risk features include the presence of thickened mitral valve leaflets with bileaflet prolapse, fast, non-sustained ventricular tachycardia, late gadolinium enhancement in cardiac MRI, and the presence of mitral annular disjunction. Although marked progress has been made in recognizing this entity and managing these patients, more insights into arrhythmia mechanisms and prospective data evaluating the impact of treatments are needed.
... The magnitude of disjunction appears to be correlated with the number of proleptic valve segments. 1 The prevalence of MAD varies in the literature, presumably due to different definitions and imaging modalities. [1][2][3] The reported prevalence in a hospital-based transthoracic echocardiography (TTE) European Heart Journal -Case Reports (2024) 8, ytae372 https://doi.org/10.1093/ehjcr/ytae372 ...
... 1 The prevalence of MAD varies in the literature, presumably due to different definitions and imaging modalities. [1][2][3] The reported prevalence in a hospital-based transthoracic echocardiography (TTE) European Heart Journal -Case Reports (2024) 8, ytae372 https://doi.org/10.1093/ehjcr/ytae372 ...
Background
Arrhythmic mitral valve prolapse syndrome (ARMV) is a recognized but underdiagnosed disease pattern. Risk factors for ARMV are established but not very well known, and the association of the structural abnormality with ventricular arrhythmias is incompletely understood.
Case summary
Here, we present the case of a young man who presented at our hospital for radiofrequency catheter ablation and mitral valve surgery after two episodes of survived sudden cardiac arrest. We discuss the diagnostic and therapeutic strategies that were used. We shine light on the risk factors for ARMV and why early identification is crucial. We address the topic of primary prevention and its limitations. Finally, we discuss different treatment modalities for patients with ARMV.
Discussion
More awareness for ARMV is crucial. A consensus statement on clinical management exists, but scientific gaps in prospective data for primary prevention need to be filled and there is a need for a better understanding of the pathogenesis of ARMV.
... It is easily identifiable in long-axis view with transthoracic echocardiography or CMR ( Figure 1). [20][21][22][23] During systole, the posterior mitral annulus shows a brisk upward motion, which is called 'curling' and is studied frame-by-frame to detect the precise location of the leaflet insertion. The lower limit of MAD is therefore defined at the level of the LV myocardium, whereas the higher limit is established at the level of PML insertion on the annulus/left atrial wall. ...
... Indeed, the exact relationship between MAD and other MVP characteristics remains uncertain and MAD has been interpreted as preceding MVP diagnosis, 11 unrelated to MVP, 20,34 or as a consequence of myxomatous MVP itself. 21,35 Moreover, studies about MAD outside the context of MVP contributed to the uncertainty in dealing with this morphological entity. Two observational studies on Japanese subjects reported a significant prevalence of MAD in structurally normal hearts (8.7% with echocardiography and 96% with cardiac computerized tomography) with a higher prevalence in patients with MV. 20,36 A recent analysis from the Biobank Imaging Study reported disjunction in 76% of the 2607 participants, more frequently at the anterior and the inferior ventricular wall. ...
Mitral valve prolapse (MVP) is usually regarded as a benign condition though the proportion of patients with a life-threatening arrhythmic MVP form remains undefined. Recently, an experts’ consensus statement on arrhythmic MVP has proposed approaches for risk stratification across the spectrum of clinical manifestation. However, sudden cardiac death may be the first presentation, making clinicians focused to early unmasking this subset of asymptomatic patients. Growing evidence on the role of cardiac imaging in the in-deep stratification pathway has emerged in the last decade. Pathology findings have suggested the fibrosis of papillary muscles and inferobasal left ventricular wall as the malignant hallmark. Cardiac magnetic resonance, while of limited availability, allows the identification of this arrhythmogenic substrate. Therefore, speckle tracking echocardiography may be a gateway to prompt referring patients to further advanced imaging investigation. Our review aims to summarize the phenotypic features linked to the arrhythmic risk and to propose an image-based algorithm intended to help stratifying asymptomatic MVP patients.
... MAD is a structural abnormality of the mitral annulus and is defi ned as a spatial displacement between the LA wall, the attachment of the mitral leafl ets, and the top of the LV free wall, manifested as a wide separation between the atrial wall-mitral valve junction and the top of the LV free wall [23,24]. The mitral annu-lus position is best recognized in the long axis view by transthoracic echocardiography zoomed on the mitral valve using the highest frame rate possible and by reviewing the images frame by frame. ...
... In the 2000s Erikson et al. [23] and Carmo et al. [24] found an increased frequency of VPBs and non-sustained VT (NSVT) in patients with MAD in comparison to those without MAD. Carmo et al. evaluated 38 patients with MVP. ...
Mitral valve prolapse (MVP) and mild mitral regurgitation (MR) are ones of the most common structural changes of the heart and affect many young individuals, who aspire to partake in competitive sport or high intensity recreational exercise. These two conditions are associated with a different prognosis and possible omplications such as heart failure (HF), malignant arrhythmias and sudden cardiac death (SCD). Therefore it is essential to determine the risk factors predicting these complications and to define a follow-up algorithm. The objective is to present a literature review of consensus recommendations addressing criteria for eligibility and disqualification from organized competitive sports for the purpose of ensuring the health and safety of young athletes.
... Several studies were dedicated to revealing potential predictors of life-threatening arrhythmias in MVP patients [8,14,16,17,22,26,27,33,34]. MAD, as an accessible echocardiographic parameter, has been considered one of the central morphologies as it correlates with VAs [17,33,46]. Therefore, in the first step of our study, we analyzed our patients according to the presence of this morphological feature, showing that MAD+ patients were characterized by the more frequent occurrence of different VAs (Table 2). ...
... For instance, Ermakov et al. showed an increase in the PSD value, due to local myocardium hypermobility, in MVP patients with VAs and indicated that the parameter was the only significant predictor of arrhythmic risk on multivariate analysis (OR 1.1, 95% CI 1.02 to 1.11, p = 0.006) [50]. In our study, PSD was significantly higher in MVP patients compared to healthy controls (44 (34 54) and 34 (27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38) ms, respectively, p = 0.005), and a little higher in MAD+ compared to MAD− persons (46 (36-56) and 38 (31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41)(42)(43)(44)(45)(46) ms respectively, p = 0.051). However, that parameter did not have adequate discrimination power in the ROC analysis. ...
Mitral valve prolapse (MVP) could associate with malignant ventricular arrhythmias (VAs). Mitral annular disjunction, a putative mechanism for an arrhythmic substrate, leads to excessive mobility, stretch, and damage of some segments. Speckle tracking echocardiography (STE), with particular attention to the segmental longitudinal strain and myocardial work index (MWI), could be an indicator of the segments we aimed to check. Seventy-two MVP patients and twenty controls underwent echocardiography. Complex VAs documented prospectively after the enrollment was qualified as the primary endpoint, which was noticed in 29 (40%) patients. Pre-specified cut-off values for peak segmental longitudinal strain (PSS) and segmental MWI for basal lateral (−25%, 2200 mmHg%), mid-lateral (−25%, 2500 mmHg%), mid-posterior (−25%, 2400 mmHg%), and mid-inferior (−23%, 2400 mmHg%) segments were accurate predictors of complex VAs. A combination of PSS and MWI increased the probability of the endpoint, reaching the highest predictive value for the basal lateral segment: odds ratio 32.15 (3.78–273.8), p < 0.001 for PSS ≥ −25% and MWI ≥ 2200 mmHg%. STE may be a valuable tool for assessing the arrhythmic risk in MVP patients. Excessively increased segmental longitudinal strain with an augmented regional myocardial work index identifies patients with the highest risk of complex VAs.
... There is evidence that mitral valve surgery can be beneficial in younger patients (<42 years). [45][46][47][48][49] However, data are limited and surgery may not effectively reduce the rate of malignant arrhythmias. 50 Due to the benign clinical course of the condition in most people, asymptomatic patients with mild or moderate MR can participate in competitive and informal sports. ...
... Due to the nature of the disease, most of the data regarding the incidence, diagnosis and possible treatment options come from inadequately powered studies either due to confounding factors or low sample number. [7][8][9][22][23][24][25]27,32,[43][44][45][46][47][48][49][50] The scarcity of large-scale, randomised, double-blind studies on the subject should also be noted. Further studies will help clear doubts by adding solid data to the current knowledge on MVP. ...
Mitral valve prolapse (MVP) is a common condition present in 1–3% of the population. There has been evidence that a subset of MVP patients is at higher risk of sudden cardiac death. The arrhythmogenic mechanism is related to fibrotic changes in the papillary muscles caused by the prolapsing valve. ECG features include ST-segment depression, T wave inversion or biphasic T waves in inferior leads, and premature ventricular contractions arising from the papillary muscles and the fascicular system. Echocardiography can identify MVP and mitral annular disjunction, a feature that has significant negative prognostic value in MVP. Cardiac MRI is indicated for identifying fibrosis. Patients with high-risk features should be referred for further evaluation. Catheter ablation and mitral valve repair might reduce the risk of malignant arrhythmia. MVP patients with high-risk features and clinically documented ventricular arrhythmia may also be considered for an ICD.
... • mitral valve prolapse (MVP) ○ particularly bileaflet prolapse [22] • myxomatous degeneration of mitral leaflets • sudden cardiac death ○ ventricular ectopy and dysrhythmias Classification I. A suggested classification for subgrouping MAD by measured degree of disjunction was proposed in 2013 by Konda et al. [23]: • type I: excessive annular mobility with an absence of a visualized separation between annulus and basal left ventricular myocardium • type II: annulus-ventricular separation (i.e. ...
Mitral annular disjunction (MAD) is an easily identifiable entity on transthoracic echocardiography, but is still poorly recognized or ignored. It is often associated with mitral valve prolapse and is itself a risk marker for ventricular arrhythmias and sudden cardiac death. We present here a case of 54 year Indian male presenting with MVP accompanied by significant mitral annulus disjunction (MAD) in whom a comprehensive transthoracic echocardiography (TTE) and 4Dimensional X Strain speckle tracking imaging was conducted to estimate the MAD distance and importantly, various LV strain parameters were evaluated to discover any early markers of LV aberration by LV strain imaging by 4Dimensional X Strain speckle tracking echocardiography (4DXStrain STE). Keywords: Mitral annulus disjunction, MAD, Mitral annulus prolapse, Ventricular arrhythmias in MAD, SCD in MAD, LV strain imaging in MAD.
... MAD is not uniformly observed in MVP, detectable by echocardiography in approximately one of three patients [7]. Diagnosis requires high spatial/temporal resolution imaging in long-axis views by transthoracic-echocardiography [10][11][12][13] or cardiovascular magnetic resonance (CMR) [8,13,14] through dynamic frame-by-frame analysis with careful examination of mitral annulus position ( Fig. 1) [15,16]. Clinical macroscopic MAD is associated with partial loss of mechanical annular function with late systolic expansion, while electrical isolation of LA/ventricle is maintained and the impact on atrial function remains undefined, although the LA is generally notably dilated with MAD [7,17]. ...
... 7 Whether the annular structural integrity and potential disappearance of MAD achieved by MV surgery (MVS) may reduce VA burden further, due to the suturing of the ring and prosthesis and the joining of the annulus to the LV myocardium, can at this stage only be hypothesised. 50,51 Similarly, whether additive therapy for MAD and stabilisation of the mitral annulus might further reduce VA burden in those MVP patients who undergo percutaneous edge-to-edge mitral valve repair warrants clarification. 52 ...
Ventricular arrhythmias associated with mitral valve prolapse (MVP) and the capacity to cause sudden cardiac death (SCD), referred to as ‘malignant MVP’, are an increasingly recognised, albeit rare, phenomenon. SCD can occur without significant mitral regurgitation, implying an interaction between mechanical derangements affecting the mitral valve apparatus and left ventricle. Risk stratification of these arrhythmias is an important clinical and public health issue to provide precise and targeted management. Evaluation requires patient and family history, physical examination and electrophysiological and imaging-based modalities. We provide a review of arrhythmogenic MVP, exploring its epidemiology, demographics, clinical presentation, mechanisms linking MVP to SCD, markers of disease severity, testing modalities and management, and discuss the importance of risk stratification. Even with recently improved understanding, it remains challenging how best to weight the prognostic importance of clinical, imaging and electrophysiological data to determine a clear high-risk arrhythmogenic profile in which an ICD should be used for the primary prevention of SCD.
... Myxomatous degeneration is more common and occurs owing to myxoid infiltration of the leaflet's spongiosa (middle) layer, leading to thick redundant leaflets, annular dilatation, and irregular elongated chordae. Myxomatous degeneration can be diffuse (Barlow disease) or localized, usually involves prominent annulus dilatation and bileaflet prolapse, and has a strong (97% of cases) association with MAD, especially in severe disease (54). Fibroelastic deficiency derives from impaired production of connective tissue, leading to segmental leaflet thinning. ...
Accurate evaluation of the mitral valve (MV) apparatus is essential for understanding the mechanisms of MV disease across various clinical scenarios. The mitral annulus (MA) is a complex and crucial structure that supports MV function; however, conventional imaging techniques have limitations in fully capturing the entirety of the MA. Moreover, recognizing annular changes might aid in identifying patients who may benefit from advanced cardiac imaging and interventions. Multimodality cardiovascular imaging plays a major role in the diagnosis, prognosis, and management of MV disease. Transthoracic echocardiography is the first-line modality for evaluation of the MA, but it has limitations. Cardiac MRI (CMR) has emerged as a robust imaging modality for assessing annular changes, with distinct advantages over other imaging techniques, including accurate flow and volumetric quantification and assessment of variations in the measurements and shape of the MA during the cardiac cycle. Mitral annular disjunction (MAD) is defined as atrial displacement of the hinge point of the MV annulus away from the ventricular myocardium, a condition that is now more frequently diagnosed and studied owing to recent technical advances in cardiac imaging. However, several unresolved issues regarding MAD, such as the functional significance of pathologic disjunction and how this disjunction advances in the clinical course, require further investigation. The authors review the role of CMR in the assessment of MA disease, with a focus on MAD and its functional implications in MV prolapse and mitral regurgitation.
... Dynamic frame-by-frame evaluation of systolic images is required to spot excessive posterior leaflet tissue of a normally implanted annulus that could incorrectly be interpreted as MAD [3]. MAD is a common, but not exclusive, feature of patients with MVP [54]. MAD length > 8.5 mm has been associated with non-sustained ventricular tachycardia [55], even in patients without mitral regurgitation [56]. ...
Mitral valve prolapse (MVP) is the most common valve disease in the western world and recently emerged as a possible substrate for sudden cardiac death (SCD). It is estimated an annual risk of sudden cardiac death of 0.2 to 1.9% mostly caused by complex ventricular arrhythmias (VA). Several mechanisms have been recognized as potentially responsible for arrhythmia onset in MVP, resulting from the combination of morpho-functional abnormality of the mitral valve, structural substrates (regional myocardial hypertrophy, fibrosis, Purkinje fibers activity, inflammation), and mechanical stretch. Echocardiography plays a central role in MVP diagnosis and assessment of severity of regurgitation. Several abnormalities detectable by echocardiography can be prognostic for the occurrence of VA, from morphological alteration including leaflet redundancy and thickness, mitral annular dilatation, and mitral annulus disjunction (MAD), to motion abnormalities detectable with "Pickelhaube" sign. Additionally, speckle-tracking echocardiography may identify MVP patients at higher risk for VA by detection of increased mechanical dispersion. On the other hand, cardiac magnetic resonance (CMR) has the capability to provide a comprehensive risk stratification combining the identification of morphological and motion alteration with the detection of myocardial replacement and interstitial fibrosis, making CMR an ideal method for arrhythmia risk stratification in patients with MVP. Finally, recent studies have suggested a potential role in risk stratification of new techniques such as hybrid PET-MR and late contrast enhancement CT. The purpose of this review is to provide an overview of the mitral valve prolapse syndrome with a focus on the role of imaging in arrhythmic risk stratification. CLINICAL RELEVANCE STATEMENT: Mitral valve prolapse is the most frequent valve condition potentially associated with arrhythmias. Imaging has a central role in the identification of anatomical, functional, mechanical, and structural alterations potentially associated with a higher risk of developing complex ventricular arrhythmia and sudden cardiac death. KEY POINTS: • Mitral valve prolapse is a common valve disease potentially associated with complex ventricular arrhythmia and sudden cardiac death. • The mechanism of arrhythmogenesis in mitral valve prolapse is complex and multifactorial, due to the interplay among multiple conditions including valve morphological alteration, mechanical stretch, myocardial structure remodeling with fibrosis, and inflammation. • Cardiac imaging, especially echocardiography and cardiac magnetic resonance, is crucial in the identification of several features associated with the potential risk of serious cardiac events. In particular, cardiac magnetic resonance has the advantage of being able to detect myocardial fibrosis which is currently the strongest prognosticator.
... MAD is only detectable, when the mitral annulus presents itself abnormally during ventricular systole. It appears as separated from the ventricular myocardium by a variable distance, from a few to more than 10 mm [48]. MAD, also known as the atrialization of the posterior leaflet base, can involve either a small or large portion of the mitral annulus, typically in the region of the P1 and P2 mitral valve scallops [49]. ...
Mitral valve prolapse (MVP) is a relatively common mitral valvulopathy and the most common cause of isolated primary mitral regurgitation (MR) requiring surgical repair. It affects about 1–3% of the general population. Although MVP is viewed as a benign condition, the association between MVP and sudden cardiac death (SCD) has been proven. Patients with MVP have a three times higher risk of SCD than the general population. The underlying mechanisms and predictors of arrhythmias, which occur in patients with MVP, are still poorly understood. However, some echocardiographic features such as mitral annulus disjunction (MAD), bileaflet MVP (biMVP), and papillary muscle (PM) fibrosis were frequently linked with increased number of arrhythmic events and are referred to as “arrhythmogenic” or “malignant”. Arrhythmogenic MVP (AMVP) has also been associated with other factors such as female sex, polymorphic premature ventricular contraction (PVC), abnormalities of T-waves, and Pickelhaube sign on tissue Doppler tracing of the lateral part of the mitral annulus. Cardiac magnetic resonance (CMR) imaging and speckle tracking echocardiography are new tools showing significant potential for detection of malignant features of AMVP. This paper presents various data coming from electrocardiography (ECG) analysis, echocardiography, and other imaging techniques as well as compilation of the recent studies on the subject of MVP.
... 47 While MAD has been hypothesized as causing MVP, its inconsistent presence argues in favor of MAD and MVP being variable consequences of myxomatous degeneration. 69 Diagnosis requires high spatial/temporal resolution imaging, in long-axis views by transthoracic echocardiography 59,[70][71][72] or MRI 40,67,72 through dynamic frame-by-frame analysis with careful examination of mitral annulus position 20,44 ( Figure 5). Mitral annular disjunction depth measurement begins at posterior leaflet insertion on the annulus/left atrial wall and ends at the detached LV myocardium in systole. ...
Mitral valve prolapse (MVP) is the most frequent valve condition but remains a conundrum in many aspects, particularly in regard to the existence and frequency of an arrhythmic form (AMVP) and its link to sudden cardiac death. Furthermore, the presence, frequency, and significance of the anatomic functional feature called mitral annular disjunction (MAD) have remained widely disputed. Recent case series and cohorts have shattered the concept that MVP is most generally benign and have emphasized the various phenotypes associated with clinically significant ventricular arrhythmias, including AMVP. The definition, evaluation, follow-up, and management of AMVP represent the focus of the present review, strengthened by recent coherent studies defining an arrhythmic MVP phenotypic that would affect a small subset of patients with MVP at concentrated high risk. The role of MAD in this context is of particular importance, and this review highlights the characteristics of AMVP phenotypes and MAD, their clinical, multimodality imaging, and rhythmic evaluation. These seminal facts lead to proposing a risk stratification clinical pathway with consideration of medical, rhythmologic, and surgical management and have been objects of recent expert consensus statements and of proposals for new research directions.
... Its recognition in transthoracic and confirmation on transesophageal echocardiography is important to facilitate optimal mitral valve repair. The modification of the repair technique allows surgical correction of the annular disjunction, which seems to optimize long-term results in these challenging cases [2]. The importance of genetically diagnosed variations of Loeys-Dietz syndrome should be highlighted, as in rare cases, the combination of mitral annular disjunction can be of clinical significance or even fatal if not treated. ...
In this case report, we present 31-year-old twin sisters diagnosed with severe Barlow mitral valve prolapse, mitral annular disjunction and presence of lateral mid-wall fibrosis diagnosed on MRI as well as ventricular arrhythmias, and a very rare variant of Loeys-Dietz syndrome, being referred to our center for surgical repair. Genetic testing detected pathogenic variants of clinical significance in SMAD3 and KCNH2 genes that are associated with autosomal dominant disease of Loeys-Dietz syndrome. Due to the presence of severe mitral valve regurgitation, the first patient was referred for minimally invasive mitral valve repair that was performed successfully. Before discharge, a subcutaneous ICD implantation was performed as primary prevention against malignant ventricular arrhythmias and sudden cardiac death. Her twin sister presented with the identical diagnosis and underwent the same surgical procedure with S-ICD implantation a few months later.
... As the posterolateral myocardium contracts during systole, the annulus "slides" and separates from the ventricular myocardium by a variable distance, ranging from a few millimetres to more than a centimeter. 4 The movement of MV annulus is located near the LV apex in systole and near the LA in diastole. During early systole mitral valve annulus contracts and assumes a deep saddle shape. ...
Objective: To determine the prevalence of Echocardiographically-recognizable Mitral Annular Disjunction in patients of Myxomatous Mitral Valve Disease/Mitral Leaflet Prolapse. Study design: Analytical Cross sectional . Place & Duration of study: Armed Forces Institute of Cardiology/National Institute of Heart Disease (AFIC/NIHD),Rawalpindi Pakistan from Jul 2021 to Sep 2021. Methodology: A total (n=45) diagnosed patients of Myxomatous Mitral Valve disease, were included through non-probability consecutive sampling. Mitral Annular Disjunction (MAD) was assessed by 2D TTE imaging as the distance between the point of insertion of the posterior leaflet into the left atrial wall (upper boundary of the disjunction) and the link between the left atrium and the left ventricle myocardium (lower border of the disjunction)at end-systole in parasternal long axis view. A distance equal to or greater than 2mm was used as a threshold for diagnosing the presence of MAD. The data analysis was done with the help of computer software programme SPSS version 24. Results: Total number of patients were 45 patients with males being 32 (71.11%) while females being 13 (28.88%), with a mean age of 30.24 + 5.21 years. MAD was present in 26 (57.8%) of the patients with mean length of 2.88mm + 2.77 mm. Patients with MAD had more chest pain, palpitations and dyspnoea than those without MAD. Mitral regurgitation was more severe in patients with MAD than without. The MAD severity correlated with the presence of Non Sustained Ventricular Tachycardia. Conclusion: MAD is not an uncommon finding in patients having myxomatous mitral valve disease/mitral valve prolapse........
... In classic MVP, due to myxoid infiltration, leaflet thickness is at least 5 mm and leaflet prolapse in the left atrium is 2 mm or more. MAD can be associated with MVP, easily detected by TTE, with a prevalence that varies from 15% to 55% based on the different imaging modality used and population considered, strongly associated with myxomatous mitral valve disease [13][14][15][16]. MAD is visualized during ventricular systole as a distinct separation of the mitral valve annulus-left atrial wall and the basal region of the posterolateral LV myocardium, when the mitral annulus "slides" into the atrium, also defined "atrialization", ranging from a few millimetres to more than 10 mm is the distance (Fig. 1). ...
Mitral valve prolapse (MVP) is the most frequent valvulopathy in the general population, with usually a favourable prognosis. Although it can be associated with some complications, ventricular arrhythmias (VA) and sudden cardiac death (SCD) are the most worrying. The estimated risk of SCD in MVP is between 0.2% to 1.9% per year, including MVP patients with and without severe mitral regurgitation (MR). The association between SCD and MVP is expressed by a phenotype called "malignant MVP" characterized by transthoracic echocardiography (TTE) findings such as bileaflet myxomatous prolapse and mitral annulus disjunction (MAD), ECG findings such as repolarization abnormalities, complex ventricular arrhythmias (c-VAs) and LV fibrosis of papillary muscles (PMs) and inferobasal wall visualized by late gadolinium enhancement cardiac magnetic resonance (LGE-CMR). Therefore, attention is raised for patients with "arrhythmic MVP" characterized from an ECG point of view by frequent premature ventricular contractions (PVCs) arising from one or both PMs as well as by T-wave inversion in the inferolateral leads. In athletes, SCD is the most frequent medical cause of death and in young subjects (< 35 years) usually is due to electrical mechanism affecting who has a silent cardiovascular disease and are not considered per se a cause of increased mortality. In MVP, SCD was reported to happen during sports activity or immediately after and valve prolapse was the only pathological aspect detected. The aim of the present paper is to explore the association between SCD and MVP in athletes, focusing attention on ECG, TTE in particular, and CMR findings that could help to identify subjects at high risk for complex arrhythmias and eventually SCD. In addition, it is also examined if sports activity might predispose patients with MVP to develop major arrhythmias.
... 8 The prevalence of MAD varies in recent studies from 98% to 16% dependent upon the severity of MVP and MR present and the diagnostic modality used. 12,13 It is more prevalent in populations with severe MVP/MR and those studied by transesophageal echo. MAD was detected in both "Barlow disease" (BD) and fibroelastic deficiency (FED) patients, suggesting that it is a primary anatomic abnormality of the annulus. ...
Mitral valve prolapse (MVP) is the most common nonischemic mitral regurgitation etiology and mitral abnormality requiring surgery in the Western world. There is an increasing awareness that pathological findings in MVP are not confined to the valve tissue; rather, it is a complex disease, involving the mitral valve apparatus, cardiac hemodynamics, and cardiac structure. Imaging has played a fundamental role in the understanding of the diagnosis, prevalence, and consequences of MVP. The diagnosis of MVP by imaging is based upon demonstrating valve leaflets ascending into the left atrium through the saddle-shaped annulus. Transthoracic and transesophageal echocardiography are the primary modalities in the diagnosis and assessment of MVP patients and must include careful assessment of the leaflets, annulus, chords, and papillary muscles. High-spatial-resolution imaging modalities such as cardiac magnetic resonance images and cardiac computed tomography play a secondary role in this regard and can demonstrate the anatomical relation between the mitral valve annulus and leaflet excursion for appropriate diagnosis. Ongoing development of new methods of cardiac imaging can help us to accurately understand the mechanism, diagnose the disease, develop an appropriate treatment plan, and estimate the risk for sudden death. Recently, several new observations with respect to prolapse have been derived from cardiac imaging including three-dimensional echocardiography and tissue-Doppler imaging. The aim of this article is to present these new imaging-derived insights for the diagnosis, risk assessment, treatment, and follow-up of patients with MVP.
... On 2D-TTE, MAD distance was measured from the parasternal long axis view, as the longitudinal distance from the left atrial wall-mitral valve leaflet junction to the top of the LV posterolateral wall during end-systole, based on original histologic description by Hutchins et al. [3] and echocardiographic description by Eriksson et al. [26]. ...
Purpose
To evaluate the possible influence of chest wall conformation on myocardial strain parameters in a cohort of mitral valve prolapse (MVP) patients with and without mitral annular disjunction (MAD).
Methods
All consecutive middle-aged patients with MVP referred to our Outpatient Cardiology Clinic for performing two-dimensional (2D) transthoracic echocardiography (TTE) as part of work up for primary cardiovascular prevention between March 2018 and May 2022, were included into the study. All patients underwent clinic visit, physical examination, modified Haller index (MHI) assessment (the ratio of chest transverse diameter over the distance between sternum and spine) and conventional 2D-TTE implemented with speckle tracking analysis of left ventricular (LV) global longitudinal strain (GLS) and global circumferential strain (GCS). Independent predictors of MAD presence on 2D-TTE were assessed.
Results
A total of 93 MVP patients (54.2 ± 16.4 yrs, 50.5% females) were prospectively analyzed. On 2D-TTE, 34.4% of MVP patients had MAD (7.3 ± 2.0 mm), whereas 65.6% did not. Compared to patients without MAD, those with MAD had: 1) significantly shorter antero-posterior (A-P) thoracic diameter (13.5 ± 1.2 vs 14.8 ± 1.3 cm, p < 0.001); 2) significantly smaller cardiac chambers dimensions; 3) significantly increased prevalence of classic MVP (84.3 vs 44.3%, p < 0.001); 4) significantly impaired LV-GLS (-17.2 ± 1.4 vs -19.4 ± 3.0%, p < 0.001) and LV-GCS (-16.3 ± 4.1 vs -20.4 ± 4.9, p < 0.001), despite similar LV ejection fraction (63.7 ± 4.2 vs 63.0 ± 3.9%, p = 0.42). A-P thoracic diameter (OR 0.25, 95%CI 0.10–0.82), classic MVP (OR 3.90, 95%CI 1.32–11.5) and mitral annular end-systolic A-P diameter (OR 2.76, 95%CI 1.54–4.92) were the main independent predictors of MAD. An A-P thoracic diameter ≤ 13.5 cm had 59% sensitivity and 84% specificity for predicting MAD presence (AUC = 0.81). In addition, MAD distance was strongly influenced by A-P thoracic diameter (r = − 0.96) and MHI (r = 0.87), but not by L-L thoracic diameter (r = 0.23). Finally, a strong inverse correlation between MHI and both LV-GLS and LV-GCS was demonstrated in MAD patients (r = − 0.94 and − 0.92, respectively), but not in those without (r = − 0.51 and − 0.50, respectively).
Conclusions
A narrow A-P thoracic diameter is strongly associated with MAD presence and is a major determinant of the impairment in myocardial strain parameters in MAD patients, in both longitudinal and circumferential directions.
Mitral valve prolapse is a common valve disorder that usually has a benign prognosis unless there is significant regurgitation or LV impairment. However, a subset of patients are at an increased risk of ventricular arrhythmias and sudden cardiac death, which has led to the recognition of “arrhythmic mitral valve prolapse” as a clinical entity. Emerging risk factors include mitral annular disjunction and myocardial fibrosis. While echocardiography remains the primary method of evaluation, cardiac magnetic resonance has become crucial in managing this condition. Cine magnetic resonance sequences provide accurate characterization of prolapse and annular disjunction, assessment of ventricular volumes and function, identification of early dysfunction and remodeling, and quantitative assessment of mitral regurgitation when integrated with flow imaging. However, the unique strength of magnetic resonance lies in its ability to identify tissue changes. T1 mapping sequences identify diffuse fibrosis, in turn related to early ventricular dysfunction and remodeling. Late gadolinium enhancement sequences detect replacement fibrosis, an independent risk factor for ventricular arrhythmias and sudden cardiac death. There are consensus documents and reviews on the use of cardiac magnetic resonance specifically in arrhythmic mitral valve prolapse. However, in this article, we propose an algorithm for the broader use of cardiac magnetic resonance in managing this condition in various scenarios. Future advancements may involve implementing techniques for tissue characterization and flow analysis, such as 4D flow imaging, to identify patients with ventricular dysfunction and remodeling, increased arrhythmic risk, and more accurate grading of mitral regurgitation, ultimately benefiting patient selection for surgical therapy.
Background
Clinical importance of mitral annulus disjunction (MAD) is not well established.
Purpose
Characterize a population of MAD all‐comers diagnosed by cardiac magnetic resonance imaging (MRI).
Study Type
Retrospective.
Population
MAD confirmed in 222 patients, age of 49.2 ± 19.3 years, 126 (56.8%) males.
Field Strength/Sequence
1.5 T and 3 T/steady‐state free precession and inversion recovery.
Assessment
Clinical history, outcomes, imaging, and arrhythmia data. MAD defined as a separation ≥2 mm between left ventricular myocardium and mitral annulus. Presence and pattern of late gadolinium enhancement (LGE) were analyzed. LGE in the papillary muscles and adjacent to MAD were identified as MAD related. Ventricular arrhythmias (VA) were grouped into non‐sustained ventricular arrhythmias (NSVA) or sustained. Cardiovascular death assessed.
Statistical Tests
Differences between baseline characteristics were compared. Univariate regression was used to investigate possible associations between ventricular arrhythmia and cardiovascular death with characteristics associated with the severity of MAD. A multivariable logistic regression included significant variables from the univariate analysis and was performed for MAD‐related and global LGE.
Results
MAD extent 5.0 ± 2.6 mm. MV annulus expanded during systole for MAD ≥6 mm. Systolic expansion associated with prolapse, billowing, and curling. LGE present in 82 patients (36.9%). Twenty‐three patients (10.4%) showed MAD‐related LGE by three different observers. No association of LGE with MAD extent ( P = 0.545) noted. Follow‐up 4.1 ± 2.4 years. No sustained VA observed. In univariable analysis, NSVA was more prevalent in patients with MAD ≥6 mm (33.3% vs. 9.9%), but this was attenuated on multivariate analysis ( P = 0.054). The presence of NSVA was associated with global LGE but not MAD‐related LGE in isolation ( P = 0.750). Three patients died of cardiovascular causes (1.4%) and none had MAD‐related LGE. None died of sudden cardiac arrest.
Conclusion
In patients referred for cardiac MRI, mitral valve dysfunction was associated with MAD severity. Scar was not related to the extent of MAD, but associated with NSVA. The risk of sustained arrhythmias and cardiovascular death was low in this population.
Evidence Level
4
Technical Efficacy
Stage 2
Presentamos un caso de Síndrome de Marfan con insuficiencia aortica severa en el que se demuestra disyunción de anillo mitral. La disyunción del anillo mitral (DAM) se caracteriza por un desplazamiento entre la unión del anillo con el miocardio ventricular. La importancia de esta anormalidad es su asociación con las arritmias ventriculares y la muerte súbita cardíaca. Existe una relación entre el tamaño de la disyunción y el riesgo de taquicardia ventricular; se ha identificado que cuanto más desplazamiento hay, mayor es el riesgo de muerte súbita cardíaca.
Mitral annular disjunction (MAD) refers to atrial displacement of the hinge point of the mitral valve annulus from the ventricular myocardium. MAD leads to paradoxical expansion of the annulus in systole and may often be associated with mitral valve prolapse (MVP), leaflet degeneration, myocardial and papillary muscle fibrosis, and, potentially, malignant cardiac arrhythmias. Patients with MAD and MVP may present similarly, and MAD is potentially the missing link in explaining why some patients with MVP experience adverse outcomes. Patients with a 5 mm or longer MAD distance have an elevated risk of malignant cardiac arrhythmia compared with those with a shorter MAD distance. Evaluation for MAD is an important component of cardiac imaging, especially in patients with MVP and unexplained cardiac arrhythmias. Cardiac MRI is an important diagnostic tool that aids in recognizing and quantifying MAD, MVP, and fibrosis in the papillary muscle and myocardium, which may predict and help improve outcomes following electrophysiology procedures and mitral valve surgery. This article reviews the history, pathophysiology, controversy, prevalence, clinical implications, and imaging considerations of MAD, focusing on cardiac MRI. Keywords: MR-Dynamic Contrast Enhanced, Cardiac, Mitral Valve, Mitral Annular Disjunction, Mitral Valve Prolapse, Floppy Mitral Valve, Cardiac MRI, Arrhythmia, Sudden Cardiac Death, Barlow Valve © RSNA, 2023.
Objective
This study investigates mitral annular disjunctions (MAD) in the atrial wall-mitral annulus-ventricular wall junction along the mural mitral leaflet and commissures.
Methods
We examined 224 adult human hearts (21.9% females, 47.9±17.6 years) devoid of cardiovascular diseases (especially mitral valve disease). These hearts were obtained during forensic medical autopsies conducted between January 2018 and June 2021. MAD was defined as a spatial displacement (≥2 mm) of the leaflet hinge line towards the left atrium. We provided a detailed morphometric analysis (disjunction height) and histological examination of MADs.
Results
MADs were observed in 19.6% of all studied hearts. They appeared in 12.1% of mural leaflets. The P1 scallop was the primary site for disjunctions (8.9%), followed by the P2 scallop (5.4%) and P3 scallop (4.5%). MADs were found in 9.8% of all superolateral and 5.8% of all inferoseptal commissures. The average height for leaflet MADs was 3.0±0.6 mm, whereas that for commissural MADs was 2.1±0.5 mm (p<0.0001). The microscopical arrangement of MADs in both the mural leaflet and commissures revealed a disjunction shifted towards left atrial aspect, filled with connective tissue and covered by elongated valve annulus. The size of the MAD remained remarkably uniform and showed no correlation with other anthropometric factors (all p>0.05).
Conclusions
In the cohort of the patients with healthy hearts, MAD is present in about 20% of all studied hearts. The MADs identified tend to be localised, confined to a single scallop. Moreover, MADs in the commissures are notably smaller than those in the mural leaflet.
Mitral annular disjunction (MAD) is the atrial displacement of the mitral valve (MV) hinge point, especially along the posterior mitral leaflet, which leads to inhomogeneous blood flow into the left ventricle, causing chronic fibrotic changes, malignant arrhythmias, and even sudden cardiac arrest. Some studies suggest that MAD is a part of normal heart morphology; however, the origin is still controversial. MAD commonly occurs with MV prolapse and myxomatous degenerative MV disease. In almost 20% of cases, MAD can occur independently as well. The prevalence of MAD in normal hearts varies from 8.6% to 96%, depending on the imaging modality and the cutoff used to define MAD. Transthoracic echocardiography is often the initial screening test, but the low sensitivity of transthoracic echocardiography to identify MAD makes it easy to miss the diagnosis altogether. More advanced imaging, especially cardiac MRI, is the gold standard for diagnosing MAD and risk stratification. MAD is an independent predictor of malignant arrhythmia. Among patients with MAD, risk stratification is based on the age at diagnosis, previous syncopal attacks, premature ventricular contractions, papillary muscle fibrosis, and longitudinal disjunction distance. Most asymptomatic patients are managed conservatively; however, radiofrequency ablation should be considered in patients with high-risk or symptomatic MAD due to the risk of ventricular arrhythmias and sudden cardiac death.
Mitral annular disjunction (MAD) is an easily identifiable entity on transthoracic echocardiography, but is still poorly recognized or ignored. It is often associated with mitral valve prolapse and is itself a risk marker for ventricular arrhythmias and sudden cardiac death, but the management and risk stratification of these patients is not systematized. Two clinical cases of MAD associated with mitral valve prolapse and ventricular arrhythmias are presented. The first case is of a patient with a history of surgical intervention on the mitral valve due to Barlow's disease. He presented to the emergency department with sustained monomorphic ventricular tachycardia requiring emergent electrical cardioversion. MAD with transmural fibrosis at the level of the inferolateral wall was documented. The second report is of a young woman with palpitations and frequent premature ventricular contractions on Holter with documentation of valvular prolapse and MAD, and focuses on the risk stratification approach. The present article offers a review of the literature regarding the arrhythmic risk of MAD and mitral valve prolapse, as well as a review of risk stratification in these patients.
Objectives
This study aimed to describe the heterogeneous extension of mitral annular disjunction (MAD) and assess the hypotesis that different phenotypes of disjunction are not associated with increased surgical challenges.
Background
Mitral regurgitation (MR) is the most common end-stage scenario of degenerative mitral valve disease (DMVD). Few data exist on the three-dimensional extension and geometry of MAD, as well as for its role in valvular dynamic and coaptation.
Methods
A total of 85 consecutive subjects, who underwent elective mitral valve repair (MVR) for MMVD at our Institution between November 2019 and October 2021, were studied retrospectively. The extension and geometry of MAD was assessed using the digitally stored volumetric datasets of real-time 3D transesophageal echocardiography (TEE). Annular phenotypes and surgical repair techniques were analyzed.
Results
Mitral annular disjunction was diagnosed in 50 out of 85 patients (59%) with Barlow disease (BD). A detailed analysis of MAD extension was conducted on 33 patients. Two pattern of disjunction were identified: a bimodal shape was highlighted in 21 patients, while a more uniform distribution of the disjuncted annulus was observed in 12 patients. The bimodal pattern was characterized by lower disjunction distance (DD) at the 140°–220° arch (3.6 ± 2.2 mm), while a more regular DD was measured in the remaining patients. All patients successfully underwent MVR. Triangular leaflet resection was performed in 58% of the cases, neochordae implantation in 9%, and notably a 27% received an isolated annuloplasty.
Conclusion
Rather than a binary feature, MAD should be taken into account in its complex and heterogeneous morphology, where two major phenotypes can be identified. Despite its anatomical complexity, MAD was not associated with an increased surgical challenge; conversely a peculiar subgroup of patient was successfully treated with an isolated annuloplasty.
Background
Mitral annular disjunction is the atrial displacement of the mural mitral valve leaflet hinge point within the atrioventricular junction. Said to be associated with malignant ventricular arrhythmias and sudden death, its prevalence in the general population is not known.
Objectives
The purpose of this study was to assess the frequency of occurrence and extent of mitral annular disjunction in a large population cohort.
Methods
The authors assessed the cardiac magnetic resonance (CMR) images in 2,646 Caucasian subjects enrolled in the UK Biobank imaging study, measuring the length of disjunction at 4 points around the mitral annulus, assessing for presence of prolapse or billowing of the leaflets, and for curling motion of the inferolateral left ventricular wall.
Results
From 2,607 included participants, the authors found disjunction in 1,990 (76%) cases, most commonly at the anterior and inferior ventricular wall. The authors found inferolateral disjunction, reported as clinically important, in 134 (5%) cases. Prolapse was more frequent in subjects with disjunction (odds ratio [OR]: 2.5; P = 0.02), with positive associations found between systolic curling and disjunction at any site (OR: 3.6; P < 0.01), and systolic curling and prolapse (OR: 71.9; P < 0.01).
Conclusions
This large-scale study shows that disjunction is a common finding when using CMR. Disjunction at the inferolateral ventricular wall, however, was rare. The authors found associations between disjunction and both prolapse and billowing of the mural mitral valve leaflet. These findings support the notion that only extensive inferolateral disjunction, when found, warrants consideration of further investigation, but disjunction elsewhere in the annulus should be considered a normal finding.
The article presents a clinical case of a combination of mitral valve prolapse (MVP), mitral annular disjunction (MAD), and ventricular arrhythmia. The presence of MAD worsens the prognosis in MVP and predisposes to life-threatening ventricular arrhythmias. In a 42-year-old patient, MAD was detected during echocardiography to determine the indications for surgical correction of mitral insufficiency in MVP. Severe myxomatous degeneration of the mitral valve leaflets, polysegmental prolapse, and typical auscultatory pattern (systolic click followed by systolic murmur in the second half of systole) were the indications for the targeted search for MAD. Multi-day (ECG) monitoring recorded nonsustained ventricular tachycardias and premature ventricular complexes (PVCs). Cardiac magnetic resonance imaging was performed for confirmation the diagnosis and searched for left ventricular myocardial fibrosis accompanying MAD. Finally, MAD was confirmed, but myocardial fibrotic changes were not detected. Owing to the absence of myocardial fibrosis, the patient was treated conservatively with a beta-adrenoblocker (25 mg/day slow-release metoprolol succinate) in combination with 25 mg/day allaforte. Repeated 24-h ECG monitoring did not detect ventricular tachycardias and nonsustained registered a significant decrease of number of PVCs. The patient is followed up prospectively due to high risk factors for fibrosis and worsening prognosis, which may require surgical correction of the existing disturbances and/or implantation of a cardioverter-defibrillator.
Mitral annular disjunction is increasingly recognized as an important anatomic feature of mitral valve disease. The presence of mitral annular disjunction, defined as separation between the left atrial wall at the point of mitral valve insertion and the left ventricular free wall, has been associated with increased degeneration of the mitral valve and increased incidence of sudden cardiac death. The clinical importance of this entity necessitates standard reporting on cardiovascular imaging reports if patients are to receive adequate risk stratification and management. We provide a narrative review of the literature pertaining to mitral annular disjunction, its clinical implications, and areas needing further research.
Sudden cardiac death is reported as the leading cause of mortality in developed nations. Arrhythmic mitral valve disease, encompassing mitral valve prolapse and/or mitral annular disjunction, is thought to be responsible in a sizable portion of these deaths. Despite this evidence, there are no reliable methods nor clinically useful risk stratification schemes to determine which group of patients are at higher risk or may benefit from interventions like catheter ablation or prophylactic implantation of a defibrillator. The reasons for this lack of guidance include our incomplete understanding of the mechanisms of ventricular arrhythmias and the fact that mitral valve prolapse and disjunction are frequently diagnosed, yet carry an overall low risk of sudden cardiac death. This heterogeneity makes the development of a reliable prediction model based on the presence of common risk factors very difficult. In this review, we summarize the relevant literature regarding the epidemiology, diagnosis, pathophysiology, and management of mitral valve prolapse and mitral annular disjunction and elucidate their role in sudden cardiac death.
Background
Mitral annular disjunction (MAD) has recently been recognized as an arrhythmogenic entity. Data on the electrophysiological substrate as well as the outcomes of catheter ablation of ventricular arrhythmias in patients with MAD is limited.
Methods
Forty patients with MAD (mean age 47±15 years; 70% female) underwent catheter ablation for ventricular arrhythmias. Detailed clinical, electrocardiographic, cardiac imaging, and procedural data were collected. Clinical outcomes were compared between patients who had substrate modification in the MAD area and those who did not.
Results
Twenty-three (57.5%) patients had ablation for premature ventricular contractions, 10 (25%) patients for sustained ventricular tachycardia, and 7 (17.5%) patients for premature ventricular contraction-triggered ventricular fibrillation ablation. Mean end-systolic MAD length was 10.58±3.49 mm on transthoracic echocardiography. Seventeen (42.5%) patients had preprocedural cardiac magnetic resonance imaging, and 5 (29%) patients had late gadolinium enhancement. Among the 18 (45%) patients who had abnormal local electrograms (low voltage, long-duration, fractionated, isolated mid-diastolic potentials) during electroanatomical mapping, 10 (25%) patients had abnormal electrograms in the anterolateral mitral annulus or MAD area. Substrate modification was performed in 10 (25%) patients. Catheter ablation was acutely successful in 36 (90%) patients (elimination of premature ventricular contraction or noninducibility of ventricular tachycardia). After a median follow-up duration of 54.08 (interquartile range, 10.67–89.79) months, premature ventricular contraction burden decreased from a median of 9.75% (interquartile range, 3.25–14) before the ablation to a median of 4% (interquartile range, 1–7.75) after the ablation ( P =0.03 [95% CI, 0.055–6.5]). Eight (20.5%) patients had repeat ablation for ventricular arrhythmias. Substrate modification of the MAD was associated with a trend toward lower rates of repeat ablation (0% versus 26.7%; P =0.16).
Conclusions
Patients with MAD have a complex arrhythmogenic substrate, and catheter ablation is effective in reducing recurrence of ventricular arrhythmias. Substrate mapping and ablation may be considered in these patients.
Primary myxomatous degeneration of cardiac valves is a rare cardiac malformation. We discovered a case of fetal primary myxomatous degeneration of cardiac valves during routine prenatal ultrasound examination. This is the first time such a case has been detected on prenatal ultrasound.
Background:
Mitral annular disjunction (MAD) represents the detachment of the mitral leaflet hinge-point from the ventricular myocardium. Its role in patients with ST-segment-elevation myocardial infarction (STEMI) is unknown. This study aims to investigate the prevalence of MAD by cardiac magnetic resonance imaging (CMR) in STEMI-patients and its association with serious adverse events.
Methods:
STEMI-patients (n = 621) underwent CMR 4 days [interquartile range (IQR) 2-5] after percutaneous coronary intervention. Presence and longitudinal extent of MAD were obtained in long-axis cine-images, infarct characteristics in late gadolinium enhancement-images. During a median follow-up time of 366 days (IQR 136-454), patients were observed for the occurrence of major adverse cardiac events (MACE), comprising death, myocardial reinfarction, and congestive heart failure.
Results:
Overall, 307 patients (49 %) had MAD. Longitudinal MAD-distance was 4.6 ± 1.7 mm and the P3-segment was affected most frequently (n = 262, 85 % of MAD-patients). MAD-patients had a significantly smaller infarct size, lower prevalence of microvascular obstruction, and intramyocardial hemorrhage as well as a higher ejection fraction (all p < 0.03). During follow-up period, MACE occurred in 52 patients (8 %) and did not show significant difference between patients with and without MAD (7 % vs. 9 %, p = 0.424). Cardiovascular death occurred significantly more often in patients without MAD (n = 10, 3.2 % vs. n = 2, 0.7 %, p = 0.021).
Conclusion:
MAD is a rather common finding in patients presenting with STEMI. Patients with MAD had less severe infarct characteristics, however, they were not more commonly affected by MACE. Further confirmation and longer follow-up intervals are necessary to define the exact role of MAD in STEMI patients.
The mitral annulus is the point at which the atrial and ventricular walls meet the base of the mitral valve cusps. The suggestion that a variant of this arrangement termed "disjunction" was associated with prolapse of the leaflets prompted examination of the mitral atrioventricular junctions in seven normal hearts and six with prolapse owing to floppy mitral valves. A complete cord-like ring of connective tissue that encircled the atrioventricular junction and into which the three components were inserted at the same point was found in only one heart. The remaining hearts all showed a mixture of segments in which either the three components were inserted into a cord or simply met. Disjunction, defined as a separation of the atrial wall-mitral valve junction from the other component, the left ventricular wall, can occur both with and without a cord-like annulus. There was no significant difference in the number of segments around the left atrioventricular junction which showed disjunction in hearts with normal or prolapsing leaflets. The feature termed disjunction is an anatomical variation of the normal morphological characteristics of the left atrioventricular junction.
We evaluated 147 patients with adequate color Doppler and angiographic studies for mitral regurgitation. Sixty-five patients had no mitral regurgitation by both color Doppler and angiography and 82 patients had mitral regurgitation by both techniques. Thus the sensitivity and specificity of color Doppler for the detection of mitral regurgitation was 100%. Three two-dimensional echocardiographic planes (parasternal long and short axis, apical four-chamber view) were used to analyze variables of the mitral regurgitant jet signals in the left atrium. The best correlation with angiography was obtained when the regurgitant jet area (RJA) (maximum or average from three planes) expressed as a percentage of the left atrial area (LAA) obtained in the same plane as the maximum regurgitant area was considered. The maximum RJA/LAA was under 20% in 34 of 36 patients with angiographic grade I mitral regurgitation, between 20% and 40% in 17 of 18 patients with grade II mitral regurgitation, and over 40% in 26 of 28 patients with severe mitral regurgitation. Maximum RJA/LAA also correlated with angiographic regurgitant fractions (r = .78) obtained in 21 of 40 patients in normal sinus rhythm and with no evidence of associated aortic regurgitation. Other variables of the regurgitant jet such as maximal linear and transverse dimensions, maximal area, or maximal area expressed as a percentage of the LAA in one or two planes correlated less well with angiography. Color Doppler is a useful noninvasive technique that is not only highly sensitive and specific in the identification of mitral regurgitation but also provides accurate estimation of its severity.
Mitral-valve prolapse has been described as a common disease with frequent complications. To determine the prevalence of mitral-valve prolapse in the general population, as diagnosed with the use of current two-dimensional echocardiographic criteria, we examined the echocardiograms of 1845 women and 1646 men (mean [+/-SD] age, 54.7+/-10.0 years) who participated in the fifth examination of the offspring cohort of the Framingham Heart Study.
Classic mitral-valve prolapse was defined as superior displacement of the mitral leaflets of more than 2 mm during systole and as a maximal leaflet thickness of at least 5 mm during diastasis, and nonclassic prolapse was defined as displacement of more than 2 mm, with a maximal thickness of less than 5 mm.
A total of 84 subjects (2.4 percent) had mitral-valve prolapse: 47 (1.3 percent) had classic prolapse, and 37 (1.1 percent) had nonclassic prolapse. Their age and sex distributions were similar to those of the subjects without prolapse. None of the subjects with prolapse had a history of heart failure, one (1.2 percent) had atrial fibrillation, one (1.2 percent) had cerebrovascular disease, and three (3.6 percent) had syncope, as compared with unadjusted prevalences of these findings in the subjects without prolapse of 0.7, 1.7, 1.5, and 3.0 percent, respectively. The frequencies of chest pain, dyspnea, and electrocardiographic abnormalities were similar among subjects with prolapse and those without prolapse. The subjects with prolapse were leaner (P<0.001) and had a greater degree of mitral regurgitation than those without prolapse, but on average the regurgitation was classified as trace or mild.
In a community based sample of the population, the prevalence of mitral-valve prolapse was lower than previously reported. The prevalence of adverse sequelae commonly associated with mitral-valve prolapse in studies of patients referred for that diagnosis was also low.
Among 206 consecutive patients having undergone mitral valve repair with a prosthetic ring between 1972 and 1979 in our institution, the 195 patients (94.5%) who survived the operation were studied to assess the long-term function of this method of repair. Patients' ages ranged from 18 to 79 years (mean age 48.7 years). Mitral valve insufficiency was due to degenerative disease in 113 patients (58%), rheumatic disease in 74 (38%), ischemia and other causes in eight patients (4%). A total of 188 patients (9.7%) were in New York Heart Association class III or IV preoperatively and 94 (48%) had atrial fibrillation. The patients were divided into three functional groups: type I (normal leaflet motion), 35 patients (18%); type II (leaflet prolapse), 147 patients (75%); and type III (restricted leaflet motion), 13 patients (7%). The techniques included prosthetic ring annuloplasty (185 patients), leaflet resection (158 patients), chordal shortening (89 patients), leaflet mobilization (10 patients) and papillary muscle reimplantation (2 patients). Long-term follow-up was available in 189 patients (96.8%), for a rate of 2316 patients per year. The 15-year actuarial and valve-related survival rates were 72.4% and 82.8%, respectively. At 15 years, 93.9% of the patients were free from thromboembolism, 96.6% free from endocarditis, 95.6% free from anticoagulant-related hemorrhage, and 87.38% free from reoperation. Actuarial rate of freedom from reoperation was higher in the group with degenerative disease (92.7%) than in the group with rheumatic disease (76.12%). Among the 157 survivors, 117 (74%) were in New York Heart Association class I and class II and 105 (66%) were in sinus rhythm. Doppler echocardiographic studies showed normal ventricular contractility in 134 patients (84.5%), absence of mitral regurgitation in 112 (74%), trivial regurgitation in 27 (17%), and significant regurgitation in 4 patients (2.5%).
Although the role of multiplane transesophageal echocardiography in the diagnosis of flail mitral valve leaflet is well described, the accuracy of this modality in localizing the involved posterior leaflet scallop (medial, middle, or lateral) has never been validated. For 54 patients undergoing intraoperative transesophageal echocardiography for severe mitral regurgitation due to flail mitral valve leaflet, we assessed the accuracy of a systematic approach to localization of the flail mitral valve leaflet. Surgical confirmation was performed for all patients. At blinded review, a sensitivity of 78%, specificity of 92%, and overall diagnostic accuracy of 88% were achieved for correct localization of the flail posterior leaflet scallop. The middle scallop was most commonly affected in this series. The medial scallop was affected least often, and diagnosis of lesions in that area was least accurate. This diagnostic approach appears to be accurate and feasible and may assist in planning specific surgical therapy for this disorder. (J Am Soc Echocardiogr 1998;11:966-71.)
Background:
The Cosgrove-Edwards Annuloplasty System includes a universally flexible band that corrects mitral annular dilatation via measured plication of the posterior annulus. The purpose of this study was to evaluate midterm clinical and functional results in the first 197 patients receiving this flexible annuloplasty band at mitral valve repair.
Methods:
From February 1993 to July 1994, 197 consecutive patients with mitral regurgitation had mitral valve repair using this system. Valve disease was degenerative in 73%, rheumatic in 15%, ischemic in 5%, infectious in 2.5%, and other in 4%.
Results:
Immediately after repair, echocardiographic mitral regurgitation was none or trivial in 92%, 1+ in 5%, and 2+ in 3%. There were no hospital deaths. Late follow-up was available in 195 patients (99%), with 661 patient-years of follow-up available for analysis. Four-year actuarial survival was 93%, freedom from thromboembolism 94%, from endocarditis 98%, and from reoperation 95%. At a mean interval of 18 months, echocardiography in 157 patients demonstrated no or trace mitral regurgitation in 56%, 1+ in 24%, 2+ in 9%, 3+ in 6%, and 4+ in 3%. At a mean of 61 +/- 5 months, reconstruction of the mitral annulus from real-time three-dimensional echocardiographic images in 10 patients confirmed preserved nonplanar shape and sphincter mechanism of the mitral annulus. Annular orifice area decreased 28% +/- 11% during the cardiac cycle from a mean of 10.1 +/- 3.9 cm2 in diastole to 7.2 +/- 2.8 cm2 in systole.
Conclusions:
This annuloplasty system is effective for repair of mitral regurgitation secondary to all causes and preserves mitral annular flexibility and function at 5-year follow-up.
Multiplane transesophageal echocardiography (TEE) is useful in providing a detailed anatomic map for successful mitral valve repair. This report describes an approach, developed over the past two to three years, which helps to delineate valve anatomy in specific detail. Mid-esophageal views are selected to view different segments of the valve leaflets. When correlated with surgical anatomy, this approach is found to be both practical and useful.
Echocardiographic quantitative assessment of the atrioventricular plane displacement (AVPD) in systole towards the apex has been used to estimate global left ventricular (LV) function. The study population consisted of 106 patients with coronary artery disease (CAD) with or without previous myocardial infarction and 40 age-matched healthy subjects. The AVPD was recorded from the apical four- and two-chamber views at four sites corresponding to the septal, lateral, anterior and posterior walls of the left ventricle. A mean displacement (AVmean) was calculated from the above sites. AVmean was significantly decreased in patients with CAD compared to healthy subjects (P less than 0.001). In patients in whom the left ventricular ejection fraction (LVEF) was calculated from cineangiograms a good correlation between AVmean and LVEF was found (r = 0.89, P less than 0.001, SEE = 6.4). Selecting an AVmean of 10 mm or more to define a normal LVEF (greater than or equal to 55%) resulted in a sensitivity of 92% and a specificity of 87% in predicting a normal versus abnormal left ventricular systolic function. It is concluded that the ease of recording the AVPD by echocardiography provides a simple and valuable noninvasive method to assess global left ventricular function in patients with CAD.
Pulmonary venous flow varies with different cardiac conditions. Flow patterns in response to mitral regurgitation have not been well studied, but flows may vary enough to differentiate among different grades of regurgitation. Accordingly, pulmonary venous flow velocities were recorded in 50 consecutive patients referred for outpatient (n = 26) or intraoperative (mitral valve repair; n = 24) echocardiographic examination for mitral regurgitation. Recordings were made of right and left upper pulmonary veins with pulsed wave Doppler transesophageal echocardiography. Mitral regurgitation was graded from 1+ to 4+ by an independent observer using transesophageal color flow mapping. The results of cardiac catheterization performed 5 weeks earlier in 43 of the patients were also graded for mitral regurgitation by an independent observer. Pulmonary venous flow patterns, the presence of reversed systolic flow and peak systolic and diastolic flow velocities were compared with the severity of mitral regurgitation indicated by each technique. Of the 28 patients with 4+ regurgitation by transesophageal color flow mapping, 26 (93%) had reversed systolic flow. The sensitivity of reversed systolic flow in detecting 4+ mitral regurgitation by transesophageal color flow mapping was 93% and the specificity was 100%. The sensitivity and specificity of reversed systolic flow in detecting 4+ mitral regurgitation by cardiac catheterization were 86% and 81%, respectively. Discordant flows were observed in 9 (24%) of 38 patients; the left vein usually showed blunted systolic flow and the right showed reversed systolic flow. In 22 intraoperative patients, there was "normalization" of pulmonary venous systolic flow after mitral valve repair in the postcardiopulmonary bypass study compared with the prebypass study after the mitral regurgitant leak was corrected.(ABSTRACT TRUNCATED AT 250 WORDS)
Among 206 consecutive patients having undergone mitral valve repair with a prosthetic ring between 1972 and 1979 in our institution, the 195 patients (94.5%) who survived the operation were studied to assess the long-term function of this method of repair. Patients' ages ranged from 18 to 79 years (mean age 48.7 years). Mitral valve insufficiency was due to degenerative disease in 113 patients (58%), rheumatic disease in 74 (38%), ischemia and other causes in eight patients (4%). A total of 188 patients (9.7%) were in New York Heart Association class III or IV preoperatively and 94 (48%) had atrial fibrillation. The patients were divided into three functional groups: type I (normal leaflet motion), 35 patients (18%); type II (leaflet prolapse), 147 patients (75%); and type III (restricted leaflet motion), 13 patients (7%). The techniques included prosthetic ring annuloplasty (185 patients), leaflet resection (158 patients), chordal shortening (89 patients), leaflet mobilization (10 patients) and papillary muscle reimplantation (2 patients). Long-term follow-up was available in 189 patients (96.8%), for a rate of 2316 patients per year. The 15-year actuarial and valve-related survival rates were 72.4% and 82.8%, respectively. At 15 years, 93.9% of the patients were free from thromboembolism, 96.6% free from endocarditis, 95.6% free from anticoagulant-related hemorrhage, and 87.38% free from reoperation. Actuarial rate of freedom from reoperation was higher in the group with degenerative disease (92.7%) than in the group with rheumatic disease (76.12%). Among the 157 survivors, 117 (74%) were in New York Heart Association class I and class II and 105 (66%) were in sinus rhythm. Doppler echocardiographic studies showed normal ventricular contractility in 134 patients (84.5%), absence of mitral regurgitation in 112 (74%), trivial regurgitation in 27 (17%), and significant regurgitation in 4 patients (2.5%).
Mitral valve prolapse has been diagnosed by two-dimensional echocardiographic criteria with surprising frequency in the general population, even when preselected normal subjects are examined. In most of these individuals, however, prolapse appears in the apical four-chamber view and is absent in roughly orthogonal long-axis views. Previous studies of in vitro models with nonplanar rings have shown that systolic mitral annular nonplanarity can potentially produce this discrepancy. However, to prove directly that apparent leaflet displacement in a two-dimensional view does not constitute true displacement above the three-dimensional annulus requires reconstruction of the entire mitral valve, including leaflets and annulus. Such reconstruction would also be necessary to explore the complex geometry of the valve and to derive volumetric measures of superior leaflet displacement. A technique was therefore developed and validated in vitro for three-dimensional reconstruction of the entire mitral valve. In this technique, simultaneous real-time acquisition of images and their spatial locations permits reconstruction of a localized structure by minimizing the effects of patient motion and respiration. By applying this method to 15 normal subjects, a coherent mitral valve surface could be reconstructed from intersecting scans. The results confirm mitral annular nonplanarity in systole, with a maximum deviation of 1.4 +/- 0.3 cm from planarity. They directly show that leaflets can appear to ascend above the mitral annulus in the apical four-chamber view, as they did in at least one view in all subjects, without actual leaflet displacement above the entire mitral valve in three dimensions, thereby challenging the diagnosis of prolapse by isolated four-chamber view displacement in otherwise normal individuals. This technique allows us to address a uniquely three-dimensional problem with high resolution and provide new information previously unavailable from the two-dimensional images. This new appreciation should enhance our ability to ask appropriate clinical questions relating mitral valve shape and leaflet displacement to clinical and pathologic consequences.
This study was performed to test the hypothesis that measurements of jet area by Doppler color flow imaging can predict the angiographic severity and hemodynamic consequences of mitral regurgitation. Doppler color flow imaging was performed in 47 patients undergoing cardiac catheterization and left ventriculography. The jet area was measured as the largest clearly definable flow disturbance in the parasternal and apical views, and expressed as the maximal jet area, the mean of the largest jet area (average jet area) in two views or as the ratio of these measures to left atrial area. Correlation of all Doppler color flow measurements with angiographic grades of mitral regurgitation were comparable, maximal jet area being closest at r = 0.76. A maximal jet area greater than 8 cm2 predicted severe mitral regurgitation with a sensitivity of 82% and specificity of 94%, whereas a maximal jet area less than 4 cm2 predicted mild mitral regurgitation with a sensitivity and specificity of 85% and 75%, respectively. All patients with an average jet area greater than 8 cm2 manifested severe mitral regurgitation. However, jet area measurements showed limited correlation with regurgitant volume and fraction (r = 0.55 and 0.62, respectively) for maximal jet area, and were not predictive of hemodynamic abnormalities, including those of pulmonary wedge pressure, stroke volume or ventricular volumes. Thus, in patients with mitral regurgitation, maximal jet area from Doppler color flow imaging provides a simple measurement that predicts angiographic grade, but manifests a weak correlation with regurgitant volume and does not predict hemodynamic dysfunction.
At the request of the Councils of The Society of Thoracic Surgeons (STS) and The American Association for Thoracic Surgery (AATS) the Ad Hoc Liaison Committee for Standardizing Definitions of Prosthetic Heart Valve Morbidity 'revisited' the 'Guidelines' published in September 1988. The purpose of the review was to update and clarify definitions within the guidelines and to consider recommendations made by others. The variety of cardiac valvular procedures has expanded since 1988; therefore, in this document the term 'operated valve' indicates prosthetic and bioprosthetic heart valves of all types: operated or repaired native valves and allograft and autograft valves. The term 'operated valve' includes any cardiac valve altered by a surgeon during an operation. Much morbidity and mortality is a direct consequence of the interaction between the patient and operated valve(s), although patient variables (eg, age, degree of coronary arterial disease, follow-up care) may be more responsible for outcomes than an operated valve. However, no set of guidelines can identify all possible patient factors that may affect morbidity and mortality. General agreement regarding the following definitions of terms and suggestions for reporting data do not preclude more detailed analyses or constructive recommendations and investigators are encouraged to identify relevant patient factors in addition to factors related to operated valves.
The geometric or anatomic diagnosis of mitral valve prolapse, as opposed to the pathologic diagnosis of myxomatous valve disease, is based on the relationship of the mitral leaflets to the surrounding anulus. Current echocardiographic criteria for this diagnosis include leaflet displacement above the annular hinge points in any two-dimensional view; implicit in this equivalent use of intersecting views is the assumption that the mitral anulus is a euclidean plane. Prolapse by these criteria is found in a surprisingly large proportion of the general population. In most of these individuals, however, prolapse is present in the apical four-chamber view and absent in roughly orthogonal long-axis views of the left ventricle. This frequently observed discrepancy between leaflet-annular relationships in intersecting views suggests an underlying geometric property of the mitral apparatus that would produce the appearance of prolapse in one view without actual leaflet distortion. To address this possibility, a model of the mitral valve and anulus was constructed. When the model anulus was given a nonplanar, saddle-shaped configuration, the clinical observations were reproduced: the leaflets appeared to lie above the low points of the anulus in one plane, and below its high points in a perpendicular plane. Therefore, the appearance of mitral valve prolapse can occur without actual leaflet displacement above the most superior points of the mitral anulus if the anulus is nonplanar. To determine whether this pattern is reflected in the human mitral anulus, two-dimensional echocardiographic views of the mitral apparatus were obtained by rotation about the cardiac apex in 20 patients without evident annular or rheumatic valvular disease. In all cases the mitral anulus, as reconstructed from these views, had a nonplanar systolic configuration, with high points located anteriorly and posteriorly. This is consistent with the findings of other groups in animals, and would favor the appearance of prolapse in the four-chamber view and its absence in long-axis views that are oriented anteroposteriorly. This model can therefore explain the frequently observed discrepancy between leaflet-annular relationships in roughly orthogonal views. It challenges the assumption that the mitral anulus is planar as well as the diagnosis of prolapse in many otherwise normal individuals based on that assumption.
Floppy mitral valve is usually attributed to connective-tissue degeneration. However, we have observed several instances in which both a floppy mitral valve and an abnormal mitral annulus fibrosus were present at autopsy. To study this association, we examined 900 hearts (after postmortem arteriography and fixation in distention) from autopsies of adults at The Johns Hopkins Hospital. Twenty-five (3 percent) of the hearts had a morphologically typical floppy mitral valve; in 23 of them (92 percent), the mitral annulus fibrosus showed disjunction--i.e., a separation between the atrial wall-mitral valve junction and the left ventricular attachment. In 42 other hearts (5 percent), which were from significantly younger patients (mean age [+/- SE], 60 +/- 2 years vs. 68 +/- 3; P less than 0.05), there was mitral annulus disjunction but no floppy mitral valve. Two hearts had a floppy mitral valve but no disjunction of the annulus; both of them had old infarcts of the papillary muscle. Our results show that floppy mitral valve is significantly associated with disjunction of the mitral annulus fibrosus (P less than 0.001). We suggest that floppy mitral valve develops from hypermobility of the valve apparatus, and that it is usually secondary to disjunction of the mitral annulus fibrosus, an anatomic variation in the morphology of the annulus.
Fifty normal mitral valves from adults were studied. Commissures, identified by commissural chordae tendineae and the tips of papillary muscles, partition the mitral valvular tissue into anterior and posterior leaflets. This definition incorporates into the posterior leaflet the structures formerly regarded as accessory leaflets. The posterior leaflet is further divided into scallops by clefts in its tissue. Cleft chordae provide a guide to these interscallop indentations or clefts. Partitioned this way, the posterior leaflet was tri-scalloped in 46 hearts. In 42, a large middle scallop was present with two smaller scallops on either side. Rough and clear zones can be defined on the anterior leaflet and rough, clear, and basal zones on the posterior leaflet.
Cardiac surgery has achieved remarkable progress in the past 10 years. Safer techniques of anesthesia and postoperative care, improved extracorporeal circulation and myocardial protection, and sophisticated surgical techniques are new tools which have been instrumental in reducing hospital mortality and increasing the efficiency of our operations. New surgical tools impose new surgical goals. It's not enough to save patients' lives; we must also take into consideration the quality of life given to the patient and the socioeconomic impact of our surgical actions. There already have been some trends in this direction, such as operating for congenital malformations at an earlier stage and the development of reconstructive operations to replace palliative techniques. Reconstructive valve surgery can very well be considered another example of this nouvelle chirurgie.
Changes in mitral annular areas and cyclic area changes were measured in 17 patients with mitral valve prolapse (MVP) and compared with those in 12 normal subjects. Using wide-angle, phased-array, two-dimensional echocardiography, mitral valve annular attachments were recorded in a view close to the apical four-chamber view, then at 30° rotational intervals around the circumference of the annulus. Diameters and chords from planes at 30° rotational intervals were used to reconstruct the annulus at selected times during the cardiac cycle. Annular areas were measured by planimetry, corrected for body surface area and expressed as area index. In normal subjects, the maximal mitral annular area index was 3.9 ± 0.7 cm2/m2 (mean ±SD). Of 11 MVP patients with minimal mitral regurgitation (MR), five had a dilated annulus (5.8 ± 0.3 cm2/m2, p <0.001) and six had a normal-sized annulus (4.2 ± 0.5 cm2/m2). In six MVP patients with at least moderate MR, annular dilatation was marked (area index 8.5 ± 1.4 cm2/m2, p <0.001) and systolic annular area reduction (16 ± 5%) was less than in normal subjects (27 ± 3%, p < 0.001). Cyclic annular area changes in MVP patients with minimal MR were similar to those in normal subjects. Mitral annular size in patients with MVP ranges from normal to markedly dilated. The occurrence of mitral annular dilatation in MVP patients with minimal MR indicates primary or intrinsic annular dilatation rather than annular dilatation secondary to left atrial or left ventricular enlargement. Patients with MVP and significant MR had marked annular dilatation and less-than-normal systolic annular area reduction.
2DE is increasingly being used to determine LVEF. There remain, however, important questions about the 2DE determination of EF: (1) which 2DE formulae correlate best with contrast ventriculography; (2) how often can a particular formula be applied to a large group of patients; and (3) what effect does 2DE quality or the presence of segmental wall motion abnormalities have on the accuracy of echo determined EF? To answer these questions, we prospectively determined the 2DE EF utilizing 10 differen formulae in 65 consecutive patients undergoing contrast ventriculogram within the following 24 hours. We also sought to examine the ability of trained observers to estimate EF from 2DE. The 2DE EF formulae that utilized biplane areas were generally more accurate than single-plane area or diameter only formulae, but were obtainable in fewer patients. The biplane Simpson's rule yielded a correlation with ventriculogram of r = 0.89, but was available in only 34 patients. While the single-plane formulae were slightly less accurate, they were measured in more patients; ellipsoid single-plane apical four-chamber r = 0.80, N = 56, and short ellipse r = 0.86, N = 47. The measured EF in patients with akinetic segments yielded a greater standard error of the mean, although correlations remained adequate when compared to the normal patient population. The EF from patients with poor quality as compared to good quality echo studies had a slightly greater standard error, but correlations were little affected. Thus biplane formulae for calculating EF yield better correlations, but are available from fewer patients than single-plane formulae. An estimate of EF was sufficiently accurate for most clinical situations and was available in 98% of the patients. The presence of abnormal wall motion or a poor quality 2DE study increased the standard error slightly, but had little effect on correlation with contrast ventriculogram.
Using wide-angle, phased-array, two-dimensional echocardiography, mitral leaflets and their annular attachments were recorded from a view close to the standard apical four-chamber view. The transducer was rotated and recordings were made at 30 degrees rotational intervals around the circumference of the mitral valve annulus. To reconstruct the annulus, the diameters (chords) from each rotational interval were arranged around a reference point. This was done for 12 times during the cardiac cycle. Annular areas were planimetered and circumferences measured. Correlation was good for areas reconstructed and measured by the same observer on separate occasions (r = 0.963) and by two different observers (r = 0.987). In 11 normal subjects the annular area index (area divided by body surface area) increased during diastole to a maximum of 3.8 +/- 0.7 cm2/m2 (mean +/- SD) in late diastole. There was presystolic followed by systolic narrowing to a minimum in midsystole. The mean reduction in area was 26 +/- 3%. The maximal annular circumference was 9.3 +/- 0.9 cm and the mean reduction in circumference was 13 +/- 3%. The overall motion pattern was similar to that reported in experimental studies in the dog. Mitral annular reconstruction may provide new information about normal and abnormal function of the mitral valve apparatus.
We developed a surgical technique for mitral valve reconstruction without a prosthetic ring. This procedure may have two advantages. One is avoidance of the potential thrombogenicity of the prosthetic ring, and the other is that this procedure may maintain the normal function of mitral annulus. To clarify the latter advantage, we defined a method for 3D assessment of the heart, especially for the dynamic changes of the mitral annulus. 3D images of the heart, including both mitral and tricuspid annuli in eight phases during the cardiac cycle, were reconstructed from magnetic resonance images of seven normal subjects, and used for this study. To depict the changes in the annular shape, we determined the following parameters of the annular function: (a) annular excursion, (b) direction of motion (direction cosine) and (c) orientation of the annulus (direction cosine) for the annular motion, (d) annular area and (e) displacement of the anterior portion from the approximated plane of the annulus. The data for the systolic annular motion indicate that the mitral annulus moves towards the apex with slight caudal deviation, with the excursion of 12.1 mm. The change in annular orientation indicates that the mitral annulus shows translational motion during systole. The mitral area was reduced by 25.6% (n = 5) from mid-diastole to mid-or late systole. Displacement at the anterior portion of the annulus did not change markedly during systole. The results demonstrate the physiologic function of the mitral annulus in normal subjects. This method will be applied to the clinical study of mitral valve reconstruction surgery. Based on the differences in annular length in intact and excised states, we describe the intact state of the posterior leaflet as "natural redundancy." Restoration of this natural redundancy has been a hallmark of successful mitral repair for over 20 years.
The myxomatous, degenerated, prolapsed or "floppy" mitral valve is the most common cause of mitral regurgitation in North America. Mitral valve reconstruction for mitral regurgitation was carried out in 219 consecutive patients with a myxomatous mitral valve from 1984 to 1993. Of the 139 men and 80 women, 23 to 84 years of age (mean 63 years), 36% of patients were 70 years of age or older, 77% were in New York Heart Association functional class III or IV, and 29% had coronary artery disease necessitating coronary bypass. The most common operation was posterior leaflet resection (161 patients [73%]). The anterior leaflet was resected in 14 patients, and both the anterior and posterior leaflets were resected in 15 patients. A variety of other techniques were used, including commissuroplasty and use of annuloplasty rings. A flexible Duran ring was used in 111 patients (51%), a Carpentier-Edwards ring in 44 patients (20%), and no ring was used in 64 patients (29%). Five operative deaths occurred (2.3%); four of the five deaths occurred in patients 70 years of age or older (5.1%); and one in 141 patients (0.7%) was younger than 70 years of age. In the late postoperative period (mean follow-up 2 years), 90% of patients had no symptoms, two had endocarditis, and seven patients had thromboemboli (transient in four, permanent in three). Structural valve degeneration requiring reoperation occurred late in 12 patients; eight were in posterior leaflet resection and two in anterior or anterior and posterior; six of 12 had no annuloplasty ring. The incidence of structural valve degeneration was less than 5% from 1990 to 1993. No systolic anterior motion of the mitral valve was seen with postoperative echocardiography before discharge. Actuarial analysis at 5 years for overall survival was 86% +/- 5%, freedom from infectious valve degeneration 97% +/- 2%, and freedom from thromboembolism 94% +/- 3%. Freedom from structural valve degeneration overall was 83% +/- 4%, with a flexible ring it was 89% +/- 6%, with a rigid ring it was 88% +/- 6%, and with no ring it was 67% +/- 12% (p = 0.03). Mitral valve reconstruction for complicated myxomatous disease of the mitral valve, regardless of leaflet involvement, is feasible and offers excellent early and late results.
A new annuloplasty ring has been developed with the aim of adding flexibility to the remodeling annuloplasty concept. Here we report its clinical use with special emphasis on segmental valve analysis and valve sizing.
From October 1992 through June 1994, 137 patients aged 4 to 76 years (mean age, 49.1 years) were operated on. The main causes of mitral valve insufficiency were degenerative, 90; bacterial endocarditis, 15; and rheumatic, 13. The indication for operation was based on the severity of the mitral valve insufficiency (90 patients were in grade III or IV) rather than on functional class (60 patients were in class III or IV). At echocardiography 6 patients had normal leaflet motion (type I), 119 leaflet prolapse (type II), and 12 restricted leaflet motion (type III). Surgical repair was carried out using Carpentier techniques of valve reconstruction. In 3 patients, inadequate ring sizing was responsible for systolic anterior motion of the anterior leaflet diagnosed by intraoperative echo. The valve was replaced in 2 patients. There were three hospital deaths, no late deaths, one reoperation for recurrent mitral valve insufficiency due to chordal rupture 1 month after repair, one reoperation for atrial thrombus formation 5 months after repair, one anticoagulant-related hemorrhage, and one thromboembolic episode.
Mid-term follow-up between 6 and 18 months was available in 94 patients. Echocardiography showed trivial or no regurgitation in 93.2% of the patients and minimal regurgitation in 6.8%. The average transmitral diastolic gradient was 3.55 +/- 1.93 mm Hg. Left ventricular end-systolic diameter and volume decreased postoperatively, demonstrating an improved left ventricular function.
This preliminary experience has provided promising results and allowed us to define the indications of the Physio-Ring versus the classic ring. It has also shown that valve sizing and proper ring selection are of primary importance.
Mitral annulus anatomy and dynamics were evaluated in 12 subjects using a three-dimensional transesophageal echocardiographic technique. The mitral annular area, diameters and distance from the left ventricular apex were measured in end-diastole, mid-systole, end-systole and mid-diastole. The mitral annulus had its largest area in end-systole and the smallest area in end-diastole. The shape of the annulus changed during the cardiac cycle with the maximal change occurring in the diameters passing close to the middle of the mitral leaflets. In the vertical plane, the annulus had a shallow ski-slope shape, with the attachment of the anterior leaflet being farthest from the apex. In other words, the highest point of the annulus was situated anteromedially and was visualized in the long axis imaging plane.
At the request of the Councils of the Society of Thoracic Surgeons (STS) and the American Association of Thoracic Surgery (AATS) the Ad Hoc Liaison Committee for Standardizing Definitions of Prosthetic Heart Valve Morbidity reviewed the "Guidelines" published in September 1988 [3, 7, 8]. The purpose of the review was to update and clarify definitions within the guidelines and to consider recommendations made by other [2, 11]. The variety of cardiac valvular procedures has expanded since 1988; therefore, in this document the term "operated valve" indicates prosthetic and bioprosthetic heart valves of all types, operated or repaired native valves and allograft and autograft valves. The term "operated valve" includes any cardiac valve altered by a surgeon during an operation. Much morbidity and mortality is a direct consequence of the interaction between the patient and operated valve(s), although patient variables (e.g., age, degree of coronary arterial disease, follow-up care, etc.) may be more responsible for outcomes than an operated valve. However, no set of guidelines can identify all possible patient factors that may affect morbidity and mortality. General agreement regarding the following definitions of terms and suggestions for reporting data do not preclude more detailed analyses or constructive recommendations and investigators are encouraged to identify relevant patient factors in addition to factors related to operated valves. Purpose The purpose of these guidelines is to facilitate the analysis and reporting of results of operations on diseased cardiac valves. The definitions and recommendations that follow are guidelines, not standards, and are designed to facilitate comparisons between the experiences of different surgeons who treat different cohorts of patients at different times with different techniques and materials.
Appropriate patient selection for surgical repair of the mitral valve depends on the specific location and mechanism of regurgitation, which, in turn, has necessitated a more detailed method to accurately describe mitral pathology. This study tests a strategy of using multiplane transesophageal echocardiography to systematically localize mitral regurgitant defects and compares these results with the surgical findings.
Fifty patients with mitral regurgitation underwent intraoperative transesophageal echocardiography for the evaluation of mitral pathology and potential repair. Mitral regurgitant defects were localized using a systematic strategy and a simple nomenclature that divides each mitral valve into six sections (three sections per leaflet) and each prosthetic sewing ring into six sections (60 radial degrees = one section).
Thirty-nine patients with native mitral valves were studied, for a total of 234 sections evaluated. Eighty-seven of these sections contained regurgitant defects by transesophageal echocardiography (mean number of regurgitant defects per valve, 2.2; range, 1 through 6). There was agreement between the transesophageal echocardiographic and surgical localizations in 96% (224/234; p < 0.0001) of the sections. Eleven patients with prosthetic mitral valves were studied, for a total of 66 sections evaluated. Twenty-three of these sections contained paravalvular leaks by transesophageal echocardiography (mean number of leaks per prosthesis, 2.1; range, 1 through 6). There was agreement between the transesophageal echocardiographic and surgical localizations in 88% (58/66; p < 0.001) of the sections.
This transesophageal echocardiographic strategy provides a systematic method to accurately localize mitral regurgitant lesions and has the potential to improve the preoperative assessment of patients with significant mitral regurgitation.
This study was carried out to evaluate the long-term results of mitral valve repair for mitral regurgitation caused by myxomatous disease of the mitral valve and the late effects of chordal replacement with expanded polytetrafluoroethylene sutures in this operation.
A total of 324 patients with mitral regurgitation caused by myxomatous disease underwent mitral valve repair from 1981 to 1995; the group comprised 241 men and 83 women whose mean age was 58 +/- 14 years. Chordal replacement with expanded polytetrafluoroethylene sutures has been performed in 165 patients since 1985. Most of the patients who had chordal replacement with expanded polytetrafluoroethylene sutures had prolapse of the anterior leaflet or prolapse of both leaflets, whereas most patients who had mitral valve repair without chordal replacement had prolapse of the posterior leaflet. Patients were followed up at annual intervals and had a Doppler echocardiographic study. The follow-up was complete and extended from 6 to 156 months (mean 36 +/- 30 months).
Two operative and 21 late deaths occurred (14 cardiac and 7 noncardiac). At 10 years the actuarial survival was 75% +/- 5%, the freedom from stroke was 94% +/- 2%, the freedom from transient ischemic attacks was 92% +/- 4%, the freedom from endocarditis was 99% +/- 1%, the freedom from mitral valve reoperation was 96% +/- 1%, and the freedom from severe mitral regurgitation was 93% +/- 3%. Chordal replacement with expanded polytetrafluoroethylene sutures had no effect on any of these end points.
Mitral valve repair was feasible in most patients with mitral regurgitation caused by myxomatous disease and it was associated with low rates of valve-related complications. Chordal replacement with expanded polytetrafluoroethylene had no adverse effect on the late outcome and was believed to have increased the probability of mitral valve repair.
Degenerative mitral valve disease is the most common cause of mitral regurgitation in the United States. Mitral valve repair is applicable in the majority of these patients and has become the procedure of choice.
This study was undertaken to identify factors influencing the durability of mitral valve repair.
Between 1985 and 1997, 1072 patients underwent primary isolated mitral valve repair for valvular regurgitation caused by degenerative disease. Repair durability was assessed by multivariable risk factor analysis of reoperation. It was supplemented by a search for valve-related risk factors for death before reoperation. Three hospital deaths occurred (0.3%); complete follow-up (4152 patient-years) was available in 1062 of 1069 hospital survivors (99.3%).
At 10 years, freedom from reoperation was 93%. Among 30 patients who required reoperation for late mitral valve dysfunction, the repair failed in 16 (53%) as a result of progressive degenerative disease. Durability of repair was adversely affected by pathologic conditions other than posterior leaflet prolapse, use of chordal shortening, annuloplasty alone, and posterior leaflet resection without annuloplasty. Durability was greatest after quadrangular resection and annuloplasty for posterior leaflet prolapse and was enhanced by the use of intraoperative echocardiography. Death before reoperation was increased in patients having isolated anterior leaflet prolapse or valvular calcification and by use of chordal shortening or annuloplasty alone.
Repair durability is greatest in patients with isolated posterior leaflet prolapse who have posterior leaflet resection and annuloplasty. Chordal shortening, annuloplasty alone, and leaflet resection without annuloplasty jeopardize late results.
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Mitral regurgitation (MR) is a major determinant of outcome in cardiac surgery. The location and mechanism of mitral lesions determine the approach to various repairs and their feasibility. Because of incomplete evaluations or change in patient condition, detailed in