Transplantation after first-stage reconstruction for hypoplastic left heart syndrome

The surgical treatment of infants born with hypoplastic left heart syndrome has received considerable attention in recent years. Although this lesion was previously considered uniformly fatal, dramatic successes have been achieved with the use of staged reconstructive and replacement therapies. However, both surgical options have benefits and limitations, and neither has demonstrated clear superiority over the other. As survival for first-stage reconstruction by the Norwood procedure has improved, a greater number of patients are potential candidates for the Fontan operation, but not all will be suitable at an acceptable risk. These patients may be treated by cardiac transplantation. The results of a treatment protocol for 90 patients with classic hypoplastic left heart syndrome or its variants at The University of Michigan are reported, with particular attention given to those patients surviving initial palliation but judged to be unsuitable for a subsequent Fontan procedure.