Clinical Outcome, Valve Dysfunction, and Progressive Aortic Dilation in a Pediatric Population With Isolated Bicuspid Aortic Valve

Abstract

The aim of this study was to explore the medium-term clinical outcome and the risk of progression of aortic valve disease and aortic dilation in pediatric patients with isolated bicuspid aortic valve (BAV). 179 pediatric patients with isolated BAV were prospectively followed from January 1995 to December 2010. Patients with severe valve dysfunction at baseline were excluded. Clinical outcome included cardiac death, infective endocarditis, aortic complications, cardiac surgery and percutaneous valvuloplasty. Echocardiographic endpoints were: progression of aortic stenosis (AS) or regurgitation (AR) and progressive aortic enlargement at different levels of the aortic root, evaluated as z-score. The median age at diagnosis was 7.8 [2.7-12.0] years. After a median followup of 5.4 [2.3-9.2] years, all patients were alive. The clinical endpoint occurred in 4 (2.2 %) patients (0.41 events per 100 patient-years). A progression of AS and AR was observed in 9 (5.0 %) and 29 (16.2 %) patients, respectively. The z-scores at the end of follow-up were not significantly different from baseline at the annulus, Valsalva sinuses and sinotubular junction, whereas a slight increase was observed at the level of the ascending aorta (1.9 vs 1.5, p = 0.046). Significant progressive aortic dilation occurred in a minority of patients (10.6, 5.6, 9.5, and 19.0 % respectively). The clinical outcome in pediatric patients with isolated BAV is favourable and the progression of aortic valve dysfunction and aortic dilation is relatively slow. These findings may be taken into account to better guide risk assessment and clinical follow-up in these patients.

Thoracic aorta 3D hemodynamics in pediatric and young adult patients with bicuspid aortic valve

“…Of note, our limited analysis of aortic growth rates in this cohort does not suggest a correlation between hemodynamics and ascending aorta growth rates. However, the number of patients with repeat studies in our cohort is relatively small, and as our data suggests and a recent study by Spaziani et al 35 reports, aortic dilation in children is a very slow process and would require longer follow-up in a larger cohort of patients to adequately assess the impact of altered hemodynamics on aortic growth rates.…”

4-D flow MRI aortic 3-D hemodynamics and wall shear stress remain stable over short-term follow-up in pediatric and young adult patients with bicuspid aortic valve

“…A study by Holmes et al [12] found that the pediatric BAV population underwent greatly increased AAo growth rates compared to normal children. Another study found that pediatric patients with BAV with left–right coronary commissure fusion morphology are at increased risk for aortic root dilation and that baseline aortic annular hypoplasia predicts progressive aortic stenosis [21]. However these studies used echocardiographic data and lacked the anatomical measurements and advanced hemodynamic parameters (wall shear stress, 3-D blood flow visualization) available with 4-D flow MRI, and these measurements might provide additional insight into BAV disease progression.…”

A Novel Method for Screening Children with Isolated Bicuspid Aortic Valve

“…Bicuspid aortic valve (BAV) has been reported as the most common congenital cardiac anomaly, affecting 0.5-2% of children population. 22,24,31,33 It is predominantly seen in males and likely that its presence has a genetic basis. 19,31 Children with BAV have the risk of the lethal cardiovascular problems i.e., aortic aneurysm.…”

“…2,19,35 Progressive dilatation of the ascending aorta or aortic root, is one of the most common complications of BAV, seen even when the valve is functionally normal. 1,15,33 Association of this complication has been validated over a large data population, 22 and the American College of Cardiology and American Heart Association recommended guidelines for managing BAV with dilated ascending aorta. 14 A number of the studies reported aortic aneurysm as a serious complication of BAV with vital importance.…”

“…Many studies showed that timely detection of BAV can substantially increase life time of the patients and prevent further complications. 3, 19,31,33 This paper presents a novel method for screening children with isolated BAV, even in the early asymptomatic phase, based on the PCG signal processing. The main focus of the paper is on the processing algorithm for automatic analyzes of PCG signal, in light of the BAV screening.…”