Catheter interventions in the staged management of hypoplastic left heart syndrome

AimTo analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome.MethodsThis study is a retrospective case note review of 527 patients undergoing staged Norwood/Fontan palliation at a single centre between 1993 and 2010. Indications and type of catheter interventions were reviewed over a median follow-up period of 7.5 years.ResultsA staged Norwood/Fontan palliation for hypoplastic left heart syndrome was performed in 527 patients. The 30-day survival rate after individual stages was 76.5% at Stage I, 96.3% at Stage II, and 99.4% at Stage III. A total of 348 interventions were performed in 189 out of 527 patients. Freedom from catheter intervention in survivors was 58.2% before Stage II and 46.7% before Stage III. Kaplan–Meier freedom from intervention post Fontan completion was 55% at 10.8 years of follow-up. Post-stage I interventions were mostly directed to relieve aortic arch obstruction – 84 balloon angioplasties – and augment pulmonary blood flow – 15 right ventricle-to-pulmonary conduit interventions; post-Stage II interventions centred on augmenting size of the left pulmonary artery – 73 procedures and abolishing systemic venous collaterals – 32 procedures. After Stage III, the focus was on manipulating the size of the fenestration – 42 interventions – and the left pulmonary artery −31 procedures.ConclusionInterventional cardiac catheterisation constitutes an integral part in the staged palliative management of patients with hypoplastic left heart syndrome. Over one-third (37%) of patients undergoing staged palliation required catheter intervention over the follow-up period.