Ventricular septal defect complicated by infective endocarditis of the aortic valve causing severe aortic regurgitation: The role of aortic valve repair

Background and aims of the study: Children with ventricular septal defect (VSD) who develop severe aortic regurgitation following infective endocarditis (IE) require aortic valve replacement in most cases. Few reports exist of repair of the aortic valve under such circumstances. Here, we report details from two patients in whom aortic valve repair resulted in a good outcome. Methods: Two children (aged 4 and 3 years) with either perimembranous or doubly committed subarterial VSD presented with congestive cardiac failure (NYHA class IV) following IE. The aortic valve in both children, and the pulmonary valve in one child, were severely damaged with vegetation, leading to severe (grade IV) aortic regurgitation. The second patient had bilateral bronchiectasis with respiratory infection and severe anemia. In addition to VSD closure, partial cuspal replacement using glutaraldehyde-tanned pericardium was performed successfully in both cases to restore geometry. Results: Both children showed a dramatic improvement following surgery and during follow up of 36 and 11 months, respectively. Both remained asymptomatic, with good growth, good left ventricular function and the presence of only trivial aortic regurgitation. Conclusion: In selected patients with suitable anatomy, repair of the aortic valve with pericardial cusp replacement may be the procedure of choice. An additional benefit is the avoidance of long-term anticoagulation.