The small left ventricle: How small is too small for biventricular repair?

Left ventricular hypoplasia is associated with a variety of congenital heart defects, including critical aortic stenosis, unbalanced atrioventricular canal, and total anomalous pulmonary venous connection, and is almost uniformly fatal without surgical or catheter-directed intervention. Accurately determining whether the left ventricle can adequately support the systemic circulation can be challenging and may be approached in a variety of ways, depending on the cardiac defect. The decision is more difficult in the present era of pediatric cardiology and cardiothoracic surgery because other options, such as the Norwood procedure and cardiac transplantation, are available to infants with left ventricular hypoplasia with improving survival. This report is a review of the present understanding of left ventricular hypoplasia and gives suggestions about how to stratify these complex patients to single versus two-ventricle repair. Copyright 1999 by W.B. Saunders Company