French Observatory of Congenital Ventricular Septal Defect With Pulmonary Overload

French Observatory of Congenital Ventricular Septal Defect With Pulmonary Overload

Ventricular septal defects (VSD) are the most common cardiac congenital heart defect (about 1/3 of patients with congenital heart disease). VSD management is related to hemodynamics and anatomical localization and the occurrence of complications. Small perimembranous VSD without pulmonary hypertension and without significant left to right shunting are tolerated, whereas large VSD with pulmonary hypertension require early surgical management in the first months of life. The management uncertainties concern the medium-sized perimembranous VSD causing a significant left-right shunt but without pulmonary hypertension, which are of variable treatment (surgical correction, percutaneous treatment, medical or abstention). There are no recommendations or consensus on the preferred indication of a therapeutic attitude.

The Pediatric and Congenital Cardiology Subsidiary, within the French Society of Cardiology, set up an observatory of perimembranous VSD with significant shunting, without pulmonary hypertension the objectives of this study are:

• To study the incidence of cardiovascular events in perimembranous VSD and search for predictive anatomical markers of events.
• To study the evolution of echocardiographic and functional data of patients having percutaneous or surgical closure compared to patient managed medically.

This observatory will provide a better understanding of the therapeutic algorithm in the management of VSD with pulmonary overload without pulmonary hypertension.

Inclusion Criteria:

• Patient at least 1 year old
• Having a perimembranous VSD with pulmonary overload defined by "a left-right shunt and a z-score of the left ventricular end-diastolic diameter> = 2".
• Consent for inclusion in the study was signed by the parents or legal guardian for minors, by the patient for the adults.

Exclusion Criteria:

• Congenital heart disease associated with membranous VSD
• Stenosis of the left ventricular outflow tract (average gradient ≥20 mmHg)
• Aortic insufficiency
• sub-pulmonary stenosis (mean gradient ≥20 mmHg)
• Tricuspid insufficiency ≥ 2/4
• History of cardiac surgery or cardiac interventional catheterization
• Shunt right-left through the VSD
• Pulmonary Arterial Hypertension defined on the data of a catheterization by PAPM> = 25 mmHg and pulmonary vascular resistance> = 3 UW.m²
• Active infectious endocarditis
• Cardiac insufficiency according to the "ESC 2016" criteria, other than a symptomatology of pulmonary hyper flow during the first year of life. Heart failure is defined by the presence of clinical signs of heart failure associated with a structural or cardiac functional abnormality resulting in a decrease in cardiac output and / or an increase in filling pressures.
• History of persistent or chronic atrial arrhythmia (atrial flutter, atrial tachycardia or chronic atrial fibrillation or requiring electrical cardioversion, drug therapy or endocavitary ablation)
• History of sustained ventricular arrhythmia (duration> = 30 seconds)
• Complete BAV
• Refusal of the patient or guardian to participate in the study