Hypoplastic Left Heart Syndrome: Can We Change the Rules of the Game?
Hypoplastic left heart syndrome (HLHS) is one of the most challenging forms of congenital heart disease to treat. Management of this condition consumes a substantial amount of energy, time, and bed capacity in many congenital heart centers in the western world today. Simply defined, HLHS is a congenital malformation in which the left ventricle is inadequate and non-viable in its capacity to perform the function of systemic perfusion. The current conventional strategy of staged reconstruction involves the principles of right ventricular recruitment for systemic perfusion, aortic arch reconstruction and a two-step process of channeling systemic venous return directly to the pulmonary vasculature absent the use of a ventricular pump (bidirectional Glenn and Fontan operations). Initially promoted by Norwood in the early 1980's, the management protocol continues to evolve, leading to the survival of thousands of children who would otherwise succumb to this lethal condition.
- fetal intervention
- fetal aortic valvuloplasty
- hypoplastic left heart syndrome
- fetal echocardiography
- Fontan procedure
- Received July 14, 2014.
- Accepted July 15, 2014.