Hypoplastic left heart syndrome

The outlook for newborns with hypoplastic left heart syndrome has been dramatically altered in the past decade with the successful application of staged reconstructive techniques. Once considered a uniformly fatal condition, refinements in operative technique and perioperative care have been largely responsible for this improved survival. The first stage in the reconstructive process, the Norwood procedure, continues to carry the most significant risk. The Norwood procedure must provide: unobstructed systemic and coronary blood flow from the right ventricle; unobstructed pulmonary venous return across the atrial septum; and sufficient pulmonary blood flow without significant volume overload. Although the postoperative management of these patients must achieve the proper ratio of systemic and pulmonary vascular resistance, a properly performed reconstruction should result in a largely uncomplicated recovery. The second stage procedure, the hemi-Fontan operation, is performed between 4 and 6 months of age. This step results in removal of the ventricular volume overload imposed by the systemic shunt and the connection of the superior vena cava to the pulmonary arteries. Augmenting the central pulmonary arteries, avoiding conduction disturbances, and constructing a potential connection for the inferior vena cava to the pulmonary arteries are essential components of this procedure. The Fontan procedure is performed at approximately 1.5-2 years of age. Inferior vena cava return is channeled to the pulmonary arteries through the previously constructed atriocaval connection to complete the separation of the pulmonary and systemic circulations. Although the current techniques have resulted in substantial improvements in the quantity and quality of survival, efforts to refine each stage of the process continue to evolve with increasing follow-up and evaluation.