Risk of late aortic events after an isolated aortic valve replacement for bicuspid aortic valve stenosis with concomitant ascending aortic dilation

Department of Cardiac Surgery, Central Clinic Bad Berka, Bad Berka, Germany.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery (Impact Factor: 3.3). 03/2012; 42(5):832-8. DOI: 10.1093/ejcts/ezs137
Source: PubMed


The optimal surgical treatment of patients with bicuspid aortic valve (BAV) disease and ascending aortic aneurysm is controversial. The aim of this study was to evaluate the risk of late aortic events after an isolated aortic valve replacement (AVR) for BAV stenosis with concomitant mild-to-moderate proximal aortic dilation.
A review of our institutional BAV database identified a subgroup of 153 consecutive BAV patients (mean age 54.2 ± 10.5 years, 73% men) with BAV stenosis and concomitant ascending aortic dilation of 40-50 mm who underwent an isolated AVR from 1995 to 2000. All cases of simultaneous aortic surgery (i.e. ascending aorta with a diameter of >50 mm) were excluded. The follow-up (1759 patient-years) was 100% complete. The mean follow-up was 11.5 ± 3.2 years. Adverse aortic events were defined as the need for proximal aortic surgery, the occurrence of aortic dissection/rupture or sudden death during the follow-up.
Actuarial survival rates of our study population were 86 and 78% at 10 and 15 years, respectively. Ascending aortic surgery was required in five patients (3%) for progressive ascending aortic aneurysm. Freedom from aortic interventions at 10 and 15 years was 97 and 94%, respectively. No documented aortic dissection or rupture occurred. Freedom from adverse aortic events was 95% at 10 years and 93% at 15 years postoperatively. In a separate group of patients presenting with aortic insufficiency (i.e. root phenotype), freedom from adverse aortic events was significantly lower (88 and 70% at 10 and 15 years, P = 0.009).
BAV patients with aortic valve stenosis and concomitant mild-to-moderate ascending aortic dilation are at a considerably low risk of adverse aortic events at 15 years after an isolated AVR. The BAV phenotype should be considered when determining the risk of subsequent adverse aortic events and the need for concomitant aortic replacement.

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    • "This particular phenotype is related to faster tubular ascending aorta dilatation [20] and the aortic regurgitation is in turn related to faster root dilatation [19]. Furthermore , the root phenotype with aortic regurgitation has been recently associated with higher dissection risk in a small BAV subgroup following isolated AVR [42]. In addition, 2 separate recent analyses of consecutive patients presenting with acute type A dissection have revealed a distinctive dissection pattern with entry tear located in the aortic root in BAV patients versus tricuspid aortic valve patients [43] [44]. "
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    ABSTRACT: Bicuspid aortic valve is the most common congenital heart defect and is associated with an aortopathy manifested by dilatation of the ascending thoracic aorta. The clinical consequences of this aortopathy are the need for periodic monitoring of aortic diameters, elective prophylactic surgical aortic repair, and the occurrence of aortic dissection or rupture. This review describes the current knowledge of BAV aortopathy in adults, including incidence, pathophysiologic insights into its etiology, contemporary hypothesis-generating observations into its complications, and recommendations for monitoring and intervention. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.
    International journal of cardiology 08/2015; 201:400-407. DOI:10.1016/j.ijcard.2015.08.106 · 4.04 Impact Factor
    • "The definition of aortic valve morphology (i.e. BAV versus TAV) was described in our previous study [5]. Aortic valve insufficiency was quantified using the uniform and validated Doppler-based echocardiographic measurements [7]. "
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    ABSTRACT: Various forms of bicuspid aortic valve (BAV) aortopathy exist, and the optimal treatment for the different subgroups is insufficiently defined. We aimed to analyse the risk of adverse aortic events after aortic valve replacement (AVR) for BAV insufficiency and concomitant mild-to-moderate dilatation of the aortic root (i.e. BAV root phenotype). A total of 56 consecutive patients (mean age 47 ± 11 years, 95% men) with BAV insufficiency and a root diameter of 40-50 mm underwent AVR surgery from 1995 to 2008. All patients, as identified from our institutional BAV database, had a dilated aortic annulus (i.e. defined as valve prosthesis size ≥27 mm) without aortic stenosis (i.e. mean gradient ≤20 mmHg). Patients who underwent concomitant aortic surgery were excluded. Follow-up (622 patient-years) including echocardiography data was available for all patients. Aortic events were defined as the need for proximal aortic surgery, the occurrence of aortic dissection/rupture, echocardiographic evidence of increasing aortic root diameter/occurrence of late paravalvular leakage or sudden death during follow-up. Actuarial survival was 90% at 10 years and 78% at 15 years. Adverse aortic events occurred in 19 (34%) study patients. Redo aortic surgery was performed in 6 patients (11%), 2 of which were for aortic dissection. Four patients (7%) suffered sudden cardiac death. Moreover, follow-up echocardiography revealed a significant, progressive enlargement of the aortic root diameter in 7 (13%) patients and occurrence of late de novo paravalvular leakage in 2 (3%) patients. The resultant freedom from aortic events was 81% at 10 years and 51% at 15 years. Patients with a BAV root phenotype are at significant risk of aortic events after isolated AVR. Simultaneous root/ascending aortic surgery should be strongly considered in such patients. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
    European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 07/2015; DOI:10.1093/ejcts/ezv259 · 3.30 Impact Factor
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    • "Besides its epidemiological relevance (1–2% of all live births, responsible for 50% of the aortic valve stenosis cases requiring surgery [1]), other characteristics of the BAV aortopathy that can account for the increasing interest in it include the relative unpredictability of its natural history, the persisting unknowns on the causative mechanisms and the heterogeneity of the anatomoclinical forms that it can assume [1] [2] [3] [4]. In particular, the variability in valve morphology and in phenotypic expression of the aortopathy is believed to likely account for the inconsistent findings both in studies of clinical outcomes and among investigations on the pathogenetic processes underlying dilatation [5] [6]. This calls for a standardization of the language used to indicate the variable phenotypes of BAV-related disease, which would allow more precise selection of study populations and comparability between analysis reports by different Authors. "
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    ABSTRACT: Different methods to classify the anatomical configurations of the aorta with bicuspid aortic valve (BAV) have been proposed. We aimed to test them in terms of descriptive power (i.e. capability to identify different clusters of patients with unique associations of anatomo-clinical features) and possible prognostic significance. A consecutive echocardiographic series of 696 BAV patients (mean age 48 ± 16 years, male:female ratio 3:1) was analysed. Three possible schemes for classification of the patterns of aortic dimensions were compared. One defined the aortic shape as 'N' (ascending < sinuses > sinotubular junction (STJ)), 'A' (ascending > sinuses > STJ) or 'E' (sinotubular ≥ sinuses), the second as 'non-dilated', 'ascending phenotype' (dilated, with ascending > sinuses) or 'root phenotype' (dilated, with sinuses > ascending) and the third as normal, 'type I' (dilated only at the ascending tract), 'type II' (dilated at both ascending and sinus levels) or 'type III' (dilated only at the sinuses). We evaluated the correlation with valve morphotypes (right-left fusion, right-non-coronary fusion) and patient characteristics. In a smaller longitudinal study (n = 150), the occurrence of fast growth of the aorta (fifth quintile: ≥1 mm/year) during follow-up (5 ± 3 years) in the various phenotypes was assessed. The three classification methods proved meaningful in terms of association with valve morphotypes: significant associations were found between right-left-coronary BAV and the root phenotype (P < 0.001) and between the right-non-coronary BAV and the shapes A and E (P<0.001) as well as type I aortic configuration (P < 0.001). The aortic shape showed significant association with five of the other tested clinical variables, the phenotype and the type of dilatation with eight. In the longitudinal study, the root phenotype showed the most significant association with fast growth (>1 mm/year) of the ascending diameter (50% root phenotype patients; P = 0.005). The association with the N type was weaker (P = 0.055); no association was found with types from the other classification scheme (P = 0.42). When tested on a large population, three previously suggested phenotypic classifications of the BAV aorta proved to categorize patients into significantly different clusters, but only the classification system distinguishing between ascending phenotype and root phenotype showed a potential prognostic value. Phenotypic class of the aorta could be a factor to integrate in future comprehensive models for risk stratification of BAV aortopathy.
    European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 01/2014; 46(2). DOI:10.1093/ejcts/ezt621 · 3.30 Impact Factor
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