Background and Aims-Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options. We report outcome from a centre where most cases are antenatally diagnosed and delivered on site. The development of staged reconstructive surgery for hypoplastic left heart syndrome (HLHS) by W I Norwood and colleagues in the early 1980s has radically altered the prognosis of this condition, 1 which was previously universally fatal. This approach provides a viable treatment alternative to heart transplantation, the use of which has been limited by a shortage of suitable donor organs.
Methods-Information2-4 The important anatomical features of HLHS are severe aortic and mitral valve hypoplasia or atresia, with underdevelopment of the left ventricle, and varying degrees of hypoplasia of the aortic arch. Both pulmonary and systemic circulations are, therefore, dependent on the right ventricle, with flow to the descending aorta being provided through a patent arterial duct. Patency of the duct can be maintained with an infusion of prostaglandin E 1 or E 2 while the infant is stabilised prior to surgery, but if the duct is allowed to close the systemic circulation cannot be supported and the infant will die if untreated.
5Reconstructive surgery for this condition is performed in three stages. The first stage, which has become known as the Norwood operation, is performed within the first few days of life. This consists of reconstruction of the hypoplastic aorta using the proximal main pulmonary artery, removal of the atrial septum, and insertion of a systemic to pulmonary artery shunt.The second stage, the hemi-Fontan operation, is carried out between 4 and 12 months, and consists of an anastomosis between the superior vena cava (SVC) and the right pulmonary artery. The third stage, the Fontan operation, is performed at 3-4 years of age, and consists of an anastomosis between the inferior vena cava (IVC) and the right pulmonary artery. This series of operations allows for complete separation of the pulmonary and systemic circulations, with systemic venous drainage passing directly to the lungs, and the right ventricle acting solely as the systemic pump.The improved prognosis of HLHS makes antenatal diagnosis all the more important, to allow the parents time to consider the treatment options, and to arrange for elective delivery in a specialist centre, if desired. It also allows for a coordinated service to be provided between fetal cardiology, neonatology, paediatric cardiology, and cardiac surgery, such that the parents can meet with the relevant specialists prior to delivery of their baby, and be shown around the unit. In order to counsel parents accurately antenatally, it is essential to understand the natural history of this condition, 6 and to be able to describe survival after surgery. [7][8][9][10][11] We report the outcome of staged reconstructive surgery for HLHS in a centre wher...