French Observatory of Congenital Ventricular Septal Defect With Pulmonary Overload

Study Overview
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Brief Summary
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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.

Official Title
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French Observatory of Congenital Ventricular Septal Defect With Pulmonary Overload

Conditions
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Congenital Heart Disease

Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 1 Year

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Perimembranous VSD with high pulmonary flow rate<br>It is an observational study, no intervention or examination will be realized for the sole purpose of the study. Patient management will be at the discretion of referral cardiologists according to the practices of the centers. As part of the usual follow-up of these patients, the participating centers collect the clinical and echocardiography data from inclusion and the following year, as well as data from a functional assessment at baseline and at one year. and the collection of cardiovascular events at 5 years and 10 years of follow-up. Data from a possible percutaneous or surgical closure procedure will be collected. The indication of VSD closure will be left to the discretion of participating centers. There will be no recommendation for percutaneous or surgical closure of VSD for the sole purpose of this observatory. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Incidence of Cardiovascular Events at 5 Years of Perimembranous VSD with pulmonary overload | The main criterion cardiovascular event is a composite criterion. At least 1 of the following criteria is required for the primary criterion to be met: endocarditis, aortic stenosis (mean gradient> 20 mmHg) aortic insufficiency left ventricular outflow tract stenosis (mean gradient> 20 mmHg) tricuspid insufficiency ≥2 surgery or cardiac interventional catheterization for an abnormality in relation to the VSD (other than simple closing) persistent supraventricular arrhythmias, sustained ventricular arrhythmia, stroke Complete atrioventricular block (AVB) Pulmonary Arterial Hypertension (PAH) heart failure cardiovascular deaths, severe haemolysis (= requiring transfusion or interventional catheterization or surgical). | 5 years of follow-up | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Anatomical predictive elements of events at 5 years of follow-up. | The event criterion meets the same definition as the primary judgment criterion. The association between anatomical elements (size of the VSD, presence of aneurysm, diameter and depth of the aneurysm, septo-aortic angulation) and cardiovascular events will be studied. | 5 years of follow-up | 
| Evolution of the left ventricular end diastolic diameter z-score one year after VSD closure | Evolution of the left ventricular end diastolic diameter z-score one year after VSD closure | 1 year of follow-up | 
| Incidence of cardiovascular events of high-flow VSDs according to the different therapeutic options at 5 years of follow-up | Incidence of cardiovascular events of high-flow VSDs according to the different therapeutic options (medical - percutaneous closure - surgical closure) at 5 years of follow-up. The event criterion meets the same definition as the primary judgment criterion. | 5 years of follow-up | 
| Incidence of cardiovascular events of high-flow VSDs according to the different therapeutic options at 10 years of follow-up. | Incidence of cardiovascular events of high-flow VSDs according to the different therapeutic options (medical - percutaneous closure - surgical closure) at 10 years of follow-up. The event criterion meets the same definition as the primary judgment criterion. | 10 years of follow-up | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Ventricular septal defect, Congenital heart disease, Outcome