Prolapse of Aortic Cusp in Ventricular Septal Defect in an Elderly Male Laubry- Pezzi Syndrome: A Blessing in Disguise

Abstract

Ventricular septal defect (VSD) is one of the most common congenital cardiac anomalies seen in children. Prolapse of aortic cusp and secondary aortic regurgitation(AR) is considered to be a risk factor and warrants early surgery even in small VSD. The Association of congenital ventricular septal defect with acquired aortic cusp prolapse responsible for AR is also known as Laubry–Pezzi syndrome. Untreated large VSD may lead to Eisenmenger syndrome in older children or adolescent. Here we are presenting rare case of an elderly patient with large perimembranous VSD (pmVSD) restricted by prolapse of aortic valve and severe AR. The prolapse of aortic valve into VSD restricted the effective left to right shunt, prevented eisenmengerisation and prolonged the longevity of patient which was treated successfully by surgery. Early management should be done in such cases to prevent progression of aortic regurgitation.