Abstract
With definitive repair for the univentricular heart now available, review of the management of infants with this defect is important. Twenty-eight infants were seen at less than ly and followed for 8m-17y (mean=73m). All had cardiac cath. Eight who died had autopsy; 18 had single left ventricle with an outlet chamber, and 10 had other types; 13 had pulmonary stenosis (PS) and 7 pulmonary atresia (PAtr.); 8 had no PS; 4 had coarctation; and 1 subaortic stenosis. Nine patients (pts.) with severe cyanosis had 13 systemic-pulmonary artery (SA-PA) shunts (8 Blalock-Taussig, 5 Ao-PA with Impra graft); the initial shunt was done at ld-6m, and the second at 3m-14y; there was no operative death, but 4 died later. Two pts. with PAtr. and 1 with coarctation died without surgery. Of 6 pts. with pulmonary hypertension (PH) and PA banding (PAB) at 2-20m, 4 survived, 2 died. Three pts. had no surgery and are doing well. Of the total group of 28 pts., 20 survived beyond ly of age. Ten pts. subsequently had more surgery (7 Glenn shunt, 2 Fontan, and 1 ventricular septation & Rastelli procedure) and 3 pts. died. The overall mortality was 39.3% (11/28); 8 of them had PS. The prognosis is better if pts. survive beyond infancy. Most surviving pts. had clinical improvement. Pts. with severe PS need SA-PA shunts, and pts. with PH need PAB at an early age. If palliative procedures are successful, the pt. could undergo Glenn procedure and Fontan procedures at an older age.
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Chen, S., Pennington, P., Nouri, S. et al. 121 MANAGEMENT OF INFANTS WITH UNIVENTRICULAR HEART. Pediatr Res 15 (Suppl 4), 460 (1981). https://doi.org/10.1203/00006450-198104001-00130
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DOI: https://doi.org/10.1203/00006450-198104001-00130
