Noninvasive detection of myocardial fibrosis in Arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging

Article · February 2005with20 Reads
DOI: 10.1016/j.jacc.2004.09.053 · Source: PubMed
Abstract
We evaluated the role of myocardial delayed-enhancement (MDE) magnetic resonance imaging (MRI) for noninvasive detection of fibrosis in Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Arrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by fibro-fatty replacement of the right ventricle (RV) leading to arrhythmias and RV failure. Endomyocardial biopsy can demonstrate fibro-fatty replacement of the RV myocardium; however, the test is invasive and carries a risk of perforation. Thirty consecutive patients were prospectively evaluated for ARVD/C. Magnetic resonance imaging was performed on a 1.5-T scanner. Ten minutes after intravenous administration of 0.2 mmol/kg of gadodiamide, MDE-MRI was obtained. Diagnosis of ARVD/C was based upon the Task Force criteria and did not include MRI findings. Twelve (40%) of 30 patients met the Task Force criteria for ARVD/C. Eight (67%) of the 12 ARVD/C patients demonstrated increased signal on MDE-MRI in the RV compared with none (0%) of the 18 patients without ARVD/C (p <0.001). Endomyocardial biopsy was performed in 9 of the 12 ARVD/C patients. Of the nine patients, four had fibro-fatty changes consistent with the diagnosis of ARVD/C. Each of these patients had increased RV signal on MDE-MRI. None of the patients without ARVD/C had any abnormalities either on histopathology or on MDE-MRI. Electrophysiologic testing revealed inducible sustained ventricular tachycardia (VT) in six of the eight ARVD/C patients with delayed enhancement, compared with none of the ARVD/C patients without delayed enhancement (p=0.01). Noninvasive detection of RV myocardial fibro-fatty changes in ARVD/C is possible by MDE-MRI. Magnetic resonance imaging findings had an excellent correlation with histopathology and predicted inducible VT on programmed electrical stimulation, suggesting a possible role in evaluation and diagnosis of patients with suspected ARVD/C.
    • Diagnosis of ARVC is currently guided by the Task Force criteria (TFC), revised in 2010 [2]. Cardiovascular Magnetic Resonance (CMR) is commonly used for diagnostic purposes in ARVC patients to identify global and regional structural abnormalities , dysfunction and for identification of fatty/ fibrotic regions [3, 4] . Although regional structural abnormalities at the inflow tract, outflow tract and apex of the RV are known in these patients [5], quantitative measures of structural abnormalities are generally limited to global measures including end-diastolic volume (EDV), end-systolic volume (ESV) or ejection fraction (EF) by CMR, in addition to right ventricular outflow tract (RVOT) diameter from echocardiography.
    [Show abstract] [Hide abstract] ABSTRACT: Background Altered right ventricular structure is an important feature of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), but is challenging to quantify objectively. The aim of this study was to go beyond ventricular volumes and diameters and to explore if the shape of the right and left ventricles could be assessed and related to clinical measures. We used quantifiable computational methods to automatically identify and analyse malformations in ARVC patients from Cardiovascular Magnetic Resonance (CMR) images. Furthermore, we investigated how automatically extracted structural features were related to arrhythmic events. MethodsA retrospective cross-sectional feasibility study was performed on CMR short axis cine images of 27 ARVC patients and 21 ageing asymptomatic control subjects. All images were segmented at the end-diastolic (ED) and end-systolic (ES) phases of the cardiac cycle to create three-dimensional (3D) bi-ventricle shape models for each subject. The most common components to single- and bi-ventricular shape in the ARVC population were identified and compared to those obtained from the control group. The correlations were calculated between identified ARVC shapes and parameters from the 2010 Task Force Criteria, in addition to clinical outcomes such as ventricular arrhythmias. ResultsBi-ventricle shape for the ARVC population showed, as ordered by prevalence with the percent of total variance in the population explained by each shape: global dilation/shrinking of both ventricles (44 %), elongation/shortening at the right ventricle (RV) outflow tract (15 %), tilting at the septum (10 %), shortening/lengthening of both ventricles (7 %), and bulging/shortening at both the RV inflow and outflow (5 %). Bi-ventricle shapes were significantly correlated to several clinical diagnostic parameters and outcomes, including (but not limited to) correlations between global dilation and electrocardiography (ECG) major criteria (p = 0.002), and base-to-apex lengthening and history of arrhythmias (p = 0.003). Classification of ARVC vs. control using shape modes yielded high sensitivity (96 %) and moderate specificity (81 %). Conclusion We presented for the first time an automatic method for quantifying and analysing ventricular shapes in ARVC patients from CMR images. Specific ventricular shape features were highly correlated with diagnostic indices in ARVC patients and yielded high classification sensitivity. Ventricular shape analysis may be a novel approach to classify ARVC disease, and may be used in diagnosis and in risk stratification for ventricular arrhythmias.
    Full-text · Article · Oct 2016
    • This phenomenon may be related to the intensity of exercise performed over the years inducing RV diameter increases without modifying systolic function or increasing biomarkers of myocardial damage, all of which point to a non-pathological nature of RV remodeling . Moreover, such remodeling was independent of RV fibrosis observed on cMRI, though the limitation of this method for discerning LGE in the free wall of the RV owing to the thin walls of this ventricle must be considered [27]. Further, because the pattern of cardiac fibrosis found in athletes is usually mild and diffuse, it may not always be detected by currently available cMRI techniques [22].
    [Show abstract] [Hide abstract] ABSTRACT: Background The impact of high exercise loads on a previously healthy heart remains controversial. We examined the consequences of decades of strenuous endurance exercise at the highest competition level on heart dimensions and volumes as well as on serum biomarkers of cardiac fibrosis/remodeling. Methods and Results We compared echocardiographic measurements and serum biomarkers of cardiac fibrosis/remodeling [troponin I, galectin-3, matrix metallopeptidase-2 and 9, N-terminal pro-brain natriuretic peptide, carboxy-terminal propeptide of type I procollagen, and soluble suppressor of tumorigenicity-2 (sST-2)/interleukin(IL)-1R4] in 53 male athletes [11 former professional (‘elite’) and 42 amateur-level (‘sub-elite’) cyclists or runners, aged 40–70 years] and 18 aged-matched controls. A subset of 15 subjects (5 controls, 3 sub-elite and 7 elite athletes) also underwent cardiac magnetic resonance imaging (cMRI). Elite and sub-elite athletes had greater echocardiography-determined left ventricular myocardial mass indexed to body surface area than controls (113 ± 22, 115.2 ± 23.1 and 94.8 ± 21 g/m2, respectively, p = 0.008 for group effect), with similar results for left (50.5 ± 4.4, 48.2 ± 4.3 and 46.4 ± 5.2 mm, p = 0.008) and right (38.6 ± 3.8, 41.1 ± 5.5 and 34.7 ± 4.3 mm, p < 0.001) ventricular end-diastolic diameter, and cMRI-determined left atrial volume indexed to body surface area (62.7 ± 8.1, 56.4 ± 16.0 and 39.0 ± 14.1 ml/m2, p = 0.026). Two athletes showed a non-coronary pattern of small, fibrotic left ventricular patches detected by late gadolinium enhancement. No group effect was noted for biomarkers. Conclusions Regardless of their competition level at a younger age, veteran endurance athletes showed an overall healthy, non-pathological pattern of cardiac remodeling. Nonetheless, the physiopathology of the ventricular fibrotic patches detected warrants further investigation.
    Full-text · Article · Jul 2016
    • Therefore, as LGE is a well validated technique for assessment of myocardial fibrosis, this is the preferred approach for RV tissue characterization and identification of fibro-adipose replacement [11,12]. In addition there is a high degree of correlation between LGE and RV biopsy, RV function and inducible ventricular arrhythmias on electrophysiologic study, making LGE an important tool in the management of these patients, including risk stratification [16]. In a previous study with ARVD/C associated mutation carriers without prior sustained ventricular arrhythmias, te Riele et al. [17] demonstrated that a combined strategy using electrocardiography, Holter and CMR identified patients at high risk for arrhythmias.
    Article · Jan 2016 · Korean journal of radiology: official journal of the Korean Radiological Society
    • When the clinical suspicion is high, CMRI imaging is commonly employed during our workup looking for pathognomonic changes of the myocardium. These may be best visualized by using T1-weighted imaging and fast spinecho MRI combined with fat suppression (Sen-Chowdhry et al., 2006; Tandri et al., 2005). The mechanism underlying VT in these patients is usually reentry and ablation can be effective therapy, although it may require more than one attempt to adequately target the circuits fromthe endocardium and epicardium.
    Full-text · Article · Nov 2015 · Korean journal of radiology: official journal of the Korean Radiological Society
    • At long term these irritative and pro inflammatory stimuli may become pro fibrotic leading to structural fibrotic alterations of the sinoatrial node and throughout the conduction system, the atrioventricular node, atrioventricular bundle and left bundle branch [29]. Added to these fibrotic alterations other structural abnormalities such as localized wall bulging, wall thinning, fatty infiltration, and fibrosis exist in the RVOT, not only in patients with Arrhythmogenic Right Ventricle Cardiomyopathy (ARVC), [30,31] but also in patients with RVOT tachycardia [32]. If these subclinical myocardial structural alterations may be an important factor limiting the efficacy of CA outcome in general population [33], in MS patients with different morphology of PVCs on surface ECG (different precordial transition and q wave in lead I) and PVCs origin focus have shown not different outcomes, observing all PVCs population and separately RVOT, LVOT and CUSPs PVCs.
    [Show abstract] [Hide abstract] ABSTRACT: The purpose of this study was to investigate the impact of metabolic syndrome (MS) on outcome of catheter ablation (CA) for treatment of frequent premature ventricular contraction beats (PVCs) originating from right ventricular outflow tract (RVOT), left ventricular outflow tract (LVOT) or coronary cusps (CUSPs), in patients with normal ventricular systolic function and absence of cardiac structural disease. In this multicentre prospective study we evaluated 90 patients with frequent PVCs originating from RVOT (n = 68), LVOT (n = 19) or CUSPs (n = 3), treated with CA. According to baseline diagnosis they were divided in patients with MS (n = 24) or without MS (n = 66). The study endpoint was a composite of recurrence of acute or delayed outflow tract ventricular arrhythmia: acute spontaneous or inducible outflow tract ventricular arrhythmia recurrence or recurrence of outflow tract PVCs in holter monitoring at follow up. Patients with MS compared to patients without MS showed a higher acute post-procedural recurrence of outflow tract PVCs (n = 8, 66.6%, vs. n = 6, 9.0%, p = 0.005). At a mean follow up of 35 (17-43) months survival free of recurrence of outflow tract PVCs was lower in patients with baseline MS compared to patients without MS diagnosis (log-rank test, p < 0.001). In cox regression analysis, only MS was independently associated with study endpoint (HR = 9.655 , 95% CI 3.000-31.0.68 , p < 0.001). MS is associated with a higher recurrence rate of outflow tract PVCs after CA in patients without structural heart disease.
    Full-text · Article · Dec 2014
    • CMR can allow for early detection of patients with genotype (+), but with phenotype (-) (189). In addition to its diagnostic role, CMR including LGE imaging can play a prognostic role in ARVD/C patients (190, 191). However, given that the normal variants of the RV are usually greater than those found in the LV, great caution should be exercised in the interpretation of RV findings found in CMR.
    [Show abstract] [Hide abstract] ABSTRACT: Cardiac magnetic resonance (CMR) imaging is now widely used in several fields of cardiovascular disease assessment due to recent technical developments. CMR can give physicians information that cannot be found with other imaging modalities. However, there is no guideline which is suitable for Korean people for the use of CMR. Therefore, we have prepared a Korean guideline for the appropriate utilization of CMR to guide Korean physicians, imaging specialists, medical associates and patients to improve the overall medical system performances. By addressing CMR usage and creating these guidelines we hope to contribute towards the promotion of public health. This guideline is a joint report of the Korean Society of Cardiology and the Korean Society of Radiology.
    Full-text · Article · Nov 2014
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