Outcomes of surgical treatment of infants with hypoplastic left heart syndrome: An institutional experience 1983-2004

Aim: To determine outcomes of surgical treatment of infants with hypoplastic left heart syndrome (HLHS). Methods: Retrospective analysis of medical records of infants with HLHS. Results: 129 of 206 (63%) infants with HLHS were managed surgically over the period 1983-2004. Survival from all stages of surgical repair was 52 (40%) patients with significantly different (P < 0.001) survival according to surgical techniques and post-operative intensive care management recognisable in three eras. During 1983-1995 a classical Norwood stage 1 operation with a systemic-pulmonary shunt was performed for 61 infants with 13 (21%) survivors. From 1996 to 2002, pulmonary vasoconstriction and systemic vasodilatation after stage 1 operation were used to optimise systemic blood flow yielding a survival of 22 of 46 (48%) infants. From 2002 to 2004 a ventricular-pulmonary conduit was used with survival of 17 of 22 (77%) infants. Survival at 1, 6, 12 months and at 5, 10 and 15 years was 65%, 53%, 48%, 38%, 38% and 25%, respectively. The mean +/- SD number of surgical procedures was 4.5 +/- 3.7; duration of hospitalisation 53 +/- 52 days (median 38); number of hospital admissions 3.0 +/- 3.5; duration in intensive care 18 +/- 20 days (median 11); hours of mechanical ventilation 278 +/- 398 (median 151). Conclusion: Short-term survival of HLHS has improved substantially over recent years with a ventricular-pulmonary conduit while long-term survival has been mediocre after arterial systemic-pulmonary shunts. Irrespective of type of primary surgery, infants undergo many operations and spend long periods in hospital and intensive care.