Hypoplastic left heart syndrome outcomes

Without intervention, hypoplastic left heart syndrome (HLHS) is almost universally fatal in infancy. Staged surgical palliation, such as a Norwood procedure, improves survival, but concerns remain about outcomes. A retrospective single-centre study from Evelina London Children's Hospital studied outcomes in 58 HLHS patients born between 1995 and 2003 who received palliative surgery.1 Outcomes were reported to outcomes in 44 non-HLHS patients with single ventricle physiology. Educational concerns were reported in 57% of HLHS versus 28% of non-HLHS patients; 9% of HLHS (vs. 5%) had developmental delay, and 7% (vs. 0%) attended special needs school. Behavioural concerns were also significantly more common in HLHS: 12% had a diagnosed behavioural disorder, 7% had autism spectrum disorder and 7% had attention deficit hyperactivity disorder compared with no non-HLHS children. Reduced exercise tolerance was reported by 72% of HLHS children versus 45% non-HLHS children (P = 0.01). The authors conclude that children with HLHS have worse physical, psychological and educational outcomes than other children with complex congenital heart disease. This is important information for parents in whom this diagnosis is made antenatally.

Reviewer: David Isaacs, [email protected], Children's Hospital at Westmead, Sydney