First-Degree Relatives Screening of Patients with Bicuspid Aortic Valve: Effectiveness and Feasibility in Pediatric Card
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ORIGINAL ARTICLE
First‑Degree Relatives Screening of Patients with Bicuspid Aortic Valve: Effectiveness and Feasibility in Pediatric Cardiology Daily Practice Claire Massardier1 · Florence Desroches2 · Narcisse Singbo3 · Jean‑Marc Côté1 · Christian Drolet1 · Christine Houde1 · Laurence Vaujois1 · Philippe Chetaille1 Received: 26 March 2020 / Accepted: 21 July 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Bicuspid aortic valve (BAV) is the most common congenital heart disease. Since heritability is suspected, actual guidelines recommend to perform an echocardiographic assessment for first-degree relatives (FDR) of patient with BAV. This study aimed to assess the effectiveness and the feasibility of the current guidelines for the screening of FDR of patient with BAV in a pediatric cardiology daily practice. Consecutive patients with BAV and their FDR were prospectively included from January 2015 to March 2018 at Centre Hospitalier Universitaire de Laval, Quebec City (Canada). Data were retrospectively collected and analyzed. A total of 713 FDR of 213 consecutives index cases [median age: 11 (6–20) years] were studied. Up to 32 (6.6%) FDR had a BAV and 26 (5.4%) had an aortic valve dysfunction. A total of 14 (2.9%) FDR had an ascending aorta dilatation according to Z-score including 6 (1.2%) patients with an ascending aorta ≥ 45 mm. No statistically significant differences regarding BAV, aortic valve dysfunction and ascending aorta dilatation prevalence were identified between generations. Screening was done in 482 (67.6%), prescribed but not done in 134 (19%), not prescribed in 92 (13%) and declined in 5 (1%) FDR. The prevalence of BAV in FDR was similar to prospective adult studies and supports actual guidelines in pediatric cardiology practice. Ascending aorta dilatation was rare in our young population. Exhaustiveness and additional burden to implement current guidelines remain a challenge in daily practice. Keywords Bicuspid aortic valve · Ascending aorta dilatation · Familial screening · Echocardiography
Introduction Bicuspid aortic valve (BAV) is the most common congenital heart disease. Prevalence of BAV in the general population is estimated to be 1–2% with a male predominance (sex ratio 3:1) [1]. Complications related to aortic valve dysfunction occur in 16–68% [1] of adults with BAV and 20–84% Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00246-020-02423-x) contains supplementary material, which is available to authorized users. * Philippe Chetaille [email protected] 1
Pediatric Cardiology Unit, Department of Pediatrics, Centre Hospitalier Universitaire Laval, 2705 Boulevard Laurier, Québec City, QC G1V 4G2, Canada
2
Laval University, Québec City, Canada
3
CHU de Québec Research Center, Laval University, Québec City, Canada
of them have an associated ascending aorta dilatation [2]. Patients are mainly asymptomatic until significant valve dysfunction or aortic dissection occur. Althou
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