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.
"This particular phenotype is related to faster tubular ascending aorta dilatation  and the aortic regurgitation is in turn related to faster root dilatation . Furthermore , the root phenotype with aortic regurgitation has been recently associated with higher dissection risk in a small BAV subgroup following isolated AVR . 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  . "
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 . Aortic valve insufficiency was quantified using the uniform and validated Doppler-based echocardiographic measurements . "
"Besides its epidemiological relevance (1–2% of all live births, responsible for 50% of the aortic valve stenosis cases requiring surgery ), 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    . 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  . 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. "
[Show abstract][Hide abstract] 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
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.