Abstract

Background. Models that predict survival in neonates with left ventricular hypoplasia and critical aortic stenosis may not be applicable to neonates with left ventricular hypoplasia and coarctation. Methods and Results. We report 8 infants with severe aortic coarctation and left ventricular hypoplasia. Mean age was 18 days (range 1–48 days), and mean weight was 3.5 kg (range 2.7–4.3 kg). Associated diagnoses included mild aortic stenosis (4), ventricular septal defect (2), and venous anomalies (2). All had coarctation repair as a primary procedure (3 of these had concomitant intracardiac procedures); 7 had subsequent operations. All are alive and well 1.1–6.7 years (mean 3.1 years) after the first surgery. Progressive increases were observed in aortic and mitral diameters, and in left ventricular dimensions, areas, and volumes when the preoperative, earliest postoperative, and most recent echocardiograms were compared. Conclusions. Despite severe left ventricular hypoplasia, a two-ventricle repair is possible in selected cases. The prognostic criteria for left ventricular hypoplasia in critical aortic stenosis may not be applicable to infant coarctation. Relief of coarctation may result in the growth of the very small left ventricle, especially when the aortic root and mitral diameters are satisfactory.

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