Single Ventricle Repair — Procedure Guide, Recovery & Risks | MyMedicPlus

Single ventricle repair encompasses three staged palliative cardiac surgeries for hearts with only one functional ventricle (hypoplastic left heart syndrome, tricuspid atresia, double-inlet left ventricle). The goal is to direct venous blood passively to the lungs without relying on the single ventricle to pump it.

Indicated for hypoplastic left heart syndrome (HLHS), tricuspid atresia, double-inlet left ventricle (DILV), unbalanced atrioventricular septal defect, and heterotaxy syndromes where two-ventricle repair is not anatomically feasible. Diagnosis is often made prenatally by fetal echocardiography.

Stage 1 (Norwood, birth-2 weeks): reconstructs neoaorta from native aorta and pulmonary artery; adds a Blalock-Taussig shunt or Sano shunt. Stage 2 (bidirectional Glenn, 4-6 months): connects superior vena cava to pulmonary artery. Stage 3 (Fontan, 2-4 years): lateral tunnel or extracardiac conduit connects inferior vena cava to pulmonary artery.

Each stage requires paediatric cardiac ICU admission; hospital stay is 2-4 weeks per stage. Inter-stage home monitoring programs detect early deterioration between Stages 1 and 2. After Fontan completion, lifelong anticoagulation with aspirin or warfarin, annual cardiology review, and exercise-restricted lifestyle are required.

Inter-stage mortality between Stages 1 and 2 is 5-15%, the most vulnerable period. Fontan failure, protein-losing enteropathy (5-10%), plastic bronchitis, atrial arrhythmias, systemic ventricular failure, and eventual heart transplantation in the third or fourth decade of life are recognized long-term complications.

10-year survival after Fontan completion is approximately 85-90% at experienced specialist centres. Exercise tolerance is moderately reduced (VO2 max 50-70% of predicted). Most patients attend mainstream schooling and lead productive lives. Transplant-free survival beyond age 40 remains an active area of research.