Hypoplastic left heart syndrome

Terminal care is not the only option

Hypoplastic left heart syndrome is not an uncommon condition and accounts for about 10% of critical congenital heart disease in infants. Each year in Britain about 200 children will be born with this condition, which, unlike some other congenital cardiac lesions, usually occurs without serious associated non-cardiac malformations. Hypoplastic left heart syndrome in its most common form consists of an underdeveloped left ventricle, mitral valve, and aortic root. Initially, systemic blood flow is maintained by the patent ductus arteriosus, and death occurs soon after this closes. Most children born with hypoplastic left heart syndrome in Britain will be managed with terminal supportive care, very few will present to cardiac surgeons (M Elliott, personal communication). Newborn infants with other complex congenital cardiac malformations–such as pulmonary atresia with intact ventricular septum–can usually expect corrective or palliative surgery with a reasonable likelihood of a satisfactory outcome. Why then are children with hypoplastic left heart syndrome still singled out for terminal care? Is there any evidence that surgery for these children is a reasonable proposition?

It …