Characterization and risk factors for aortic dilatation in pediatric patients with bicuspid aortic valve

Anna Sabaté-Rotés1, Laura Sabidó Sanchez1, Ferran Gran Ipiña1

  • 1Cardiología Pediátrica, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, España.

Medicina Clinica
|June 21, 2017
PubMed

Insights

Pediatric patients with bicuspid aortic valve often experience aortic dilatation, particularly of the ascending aorta. Early medical follow-up is crucial for managing this common valve impairment.

Area of Science:

  • Cardiovascular Medicine
  • Pediatric Cardiology
  • Medical Genetics

Background:

  • Bicuspid aortic valve (BAV) is a common congenital heart defect associated with aortic dilatation, a significant cause of morbidity and mortality.
  • Ascending aortic dilatation in adults with BAV is well-documented, but pediatric involvement requires further characterization.

Purpose of the Study:

  • To identify the prevalence and characteristics of aortic involvement in pediatric patients with BAV.
  • To determine risk factors associated with aortic dilatation in this population.

Main Methods:

  • Retrospective analysis of aortic measurements in pediatric patients with BAV from 1997-2015.
  • Exclusion of patients with syndromic aortic dilatation.

Main Results:

  • Aortic dilatation (z-score>2) was observed in over one-third of 206 pediatric BAV patients.
  • Ascending aorta was primarily affected (70/80 patients), with the aortic root often spared.
  • Absence of coarctation and a vertical valve opening pattern were associated with ascending aorta dilatation.

Conclusions:

  • Pediatric patients with BAV frequently exhibit aortic dilatation, particularly of the ascending aorta.
  • Regular medical follow-up is essential for early detection and management of aortic abnormalities in these children.
  • Identifying risk factors like valve opening pattern can aid in risk stratification.
Abstract

Related Concept Videos

Aortic Regurgitation II: Clinical Features and Diagnostic Tests01:22

Aortic Regurgitation II: Clinical Features and Diagnostic Tests

Aortic valve regurgitation (AR) occurs when the aortic valve fails to close properly, allowing blood to flow backward from the aorta into the left ventricle. This backflow can result in two distinct clinical presentations: acute and chronic AR, each characterized by its own set of symptoms and physical findings.Acute Aortic RegurgitationAcute AR presents with a sudden onset of severe symptoms. Patients typically experience profound dyspnea (shortness of breath), chest pain, and signs of left...
694
Aneurysm I: Introduction01:30

Aneurysm I: Introduction

An aortic aneurysm is a localized outpouching or dilation at a weak point in the artery wall. It may involve different parts of the aorta, such as the abdominal aorta, aortic arch, or thoracic aorta.Etiological factorsSeveral disorders are associated with aortic aneurysms.Congenital causes, such as primary connective tissue disorders like Marfan syndrome, impact the integrity and strength of connective tissues, notably affecting the aorta. Marfan syndrome is a genetic disorder that specifically...
556
Aortic Regurgitation I: Introduction01:15

Aortic Regurgitation I: Introduction

IntroductionAortic regurgitation is characterized by the backward flow of blood from the aorta into the left ventricle during diastole and arises from the improper closure of the aortic valve. This condition results in left ventricular volume overload and can stem from both acute and chronic etiologies, each contributing uniquely to the disease's progression and symptomatology.Acute and Chronic CausesAcute aortic regurgitation often results from events that suddenly impair the integrity of the...
898
Aortic Regurgitation III: Medical Management01:25

Aortic Regurgitation III: Medical Management

Aortic regurgitation (AR) is when the aortic valve does not close or seal properly, leading to backward blood circulation from the aorta into the left ventricle during diastole. Common causes of AR include rheumatic heart disease, congenital valve defects, and aortic root dilation. Managing AR requires a multifaceted approach to alleviate symptoms, preserve left ventricular function, and address the underlying cause of the regurgitation. Patients with symptomatic AR or significant left...
518
Aneurysm II: Clinical Manifestations and Diagnostic Studies01:21

Aneurysm II: Clinical Manifestations and Diagnostic Studies

Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
441
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
956