Hypoplastic Left Heart Syndrome: "Which is the Best Operative Strategy?"

Therapy for the hypoplastic left heart syndrome (HLHS) is still under debate. We report about our experience in the treatment of this cardiac malformation using heart transplantation and Norwood palliation. From 1988 to 1997 a total number of 30 infants with hypoplastic left heart underwent heart transplantation. Mean age at transplantation was 66 days. Mean waiting time was 53 days. Donor-recipient weight ratio ranged from 0.6 to 3.9. There were 6 early hospital deaths and one late death until now. Causes of death were rejection (3), right heart failure (2), infection (1) and multi system organ failure (1). Overall survival was 77%. Increasing experience improved results substantially. From 1988 to 1993 (n=12) survival rate was 58%, whereas from 1994 to 1997 (n=18) survival rate increased to 88%. Quality of life is excellent in the 23 surviving infants. Only one infant shows severe morbidity (neurological defect). The Norwood procedure has been performed in 18 patients since 1993. Five patients died after the first stage. Up until now 10 infants have undergone the second stage and one infant the third stage procedure. None of them have died. Survival rate is 72% for the whole group. Taking into account that 12 infants died on the waiting list for transplanatation (28%), we have comparable results in terms of the survival rate for transplantation and the Norwood procedure in infants with HLHS since 1994. However quality of life and physical development are far better in infants after transplantation according to our experience. Therefore we prefer, whenever possible heart transplantation in the treatment of hypoplastic left heart syndrome. Certainly not all infants with this malformation can undergo transplantation because of the lack of donor organs. Criteria for the decision regarding the operative strategy in our opinion are function of the right ventricle and tricuspid valve, size of the interaterial communication and parental request.