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Abstract
This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse. Large muscular VSDs may be closed by percutaneous techniques. A large number of devices have been used in the past for VSD occlusion, but currently Amplatzer Muscular VSD Occluder is the only device approved by the US Food and Drug Administration for clinical use. A hybrid approach may be used for large muscular VSDs in small babies. Timely intervention to prevent pulmonary vascular obstructive disease (PVOD) is germane in the management of these babies. There are several types of AVSDs: partial, transitional, intermediate, and complete. Complete AVSDs are also classified as balanced and unbalanced. All intermediate and complete balanced AVSDs require surgical correction, and early repair is needed to prevent the onset of PVOD. Surgical correction with closure of atrial septal defect and VSD, along with repair and reconstruction of atrioventricular valves, is recommended. Palliative pulmonary artery banding may be considered in babies weighing less than 5 kg and those with significant co-morbidities. The management of unbalanced AVSDs is more complex, and staged single-ventricle palliation is the common management strategy. However, recent data suggest that achieving two-ventricle repair may be a better option in patients with suitable anatomy, particularly in patients in whom outcomes of single-ventricle palliation are less than optimal. The majority of treatment modes in the management of VSDs and AVSDs are safe and effective and prevent the development of PVOD and cardiac dysfunction.
Highlights
Septal defects are the most common types of congenital heart defects (CHDs) with the exception of bicuspid aortic valve
Discussion of ventricular septal defects (VSDs) seen in association with tetralogy of Fallot, pulmonary atresia/stenosis, transposition of the great arteries, tricuspid and mitral atresia, and double-outlet right ventricle (RV) and heterotaxy syndromes will not be included in this article
The pediatricians and the general pediatric cardiologists should serve as gatekeepers to prevent intervention by pediatric cardiac surgeons and interventional pediatric cardiologists for “small” VSDs that do not strictly fit established criteria for closure, irrespective of the type of intervention, whether it is conventional surgical, percutaneous, or hybrid
Summary
Septal defects are the most common types of congenital heart defects (CHDs) with the exception of bicuspid aortic valve. Whereas transcatheter (percutaneous) approaches are the mainstay in the management of secundum ASDs, the VSDs and AVSDs are at present managed largely by surgical methodology since a limited number of lesions are amenable for transcatheter and hybrid approaches. We will present a classification of the VSDs and AVSDs, indications for repair, and a discussion of surgical, transcatheter, and hybrid methodologies with a particular focus on advances in the management of these defects. The membranous defects are most common among the VSDs (80% prevalence), and supracristal (5 to 7%), AV septal (8%), and muscular (5 to 20%) defects are much less common. These defects may be large, medium, or small in size. Most of the defects are single; multiple defects may be present in the muscular septum, described as the “Swiss cheese” type of VSDs
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