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

Abstract

To determine outcomes of surgical treatment of infants with hypoplastic left heart syndrome (HLHS). Retrospective analysis of medical records of infants with HLHS. 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). 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.