Hypoplastic left heart

Hypoplastic Left Heart Syndrome comprises a spectrum of congenital heart abnormalities where there is underdevelopment of the left ventricle, in association with aortic and mitral stenosis or atresia together with a small ascending aorta. The babies’ systemic circulation is dependant on the right ventricle perfusing the body through a patent arterial duct. When the duct closes after birth acidosis and death occur through lack of systemic circulation.The introduction of Prostaglandin and intensive resuscitation techniques enabled these babies to survive. The development by Norwood of a three-stage complex palliative repair programme over the first few years of life has improved the survival rate at 1 year up to 75%. The Fontan procedure with a right ventricular dependant systemic circulation is the third and final operation in the Norwood pathway. Patients can have a good quality of life in the medium term, but the long-term outlook for the Fontan circulation is guarded by virtue of early development of myocardial failure, protein loosing enteropathy and arrhythmias. Cardiac transplantation options are limited because of the few donors.