Hypoplastic Left Heart Syndrome - Surgical Management

Surgical Management

Surgical operations to assist with hypo-plastic left heart are complex and need to be individualized for each patient, a cardiologist must assess all medical and surgical options on a case-by-case basis.

Currently, infants who undergo either the staged reconstructive surgery (Norwood procedure almost immediately after birth, Glenn procedure at 3 to 6 months of age, and the Fontan procedure at 3 to 5 years of age) or who undergo cardiac transplantation have a 5-year survival of approximately 50-60% as a group. However, it is becoming clear that there are higher risk subsets where 5 year survival is lower (including those with lower birth weight, additional congenital anomalies, a genetic syndrome or those with a highly restrictive atrial septum.) For patients without these additional risk factors, 5 year survival now approaches 80%. Further, studies show that about 50% of those children who survived surgery in the early development of staged reconstruction have developmental delay or need special education; about 25% of these surgical survivors have severe disabilities. There is growing evidence that while the incidence of developmental and behavioral disabilities continues to be higher than that in the general population, children operated upon in the more current era have shown significantly better neurological outcomes. An alternative to the traditional Norwood is the Hybrid procedure.

Some parents choose an option referred to as compassionate care, and these infants receive treatment aimed at relieving suffering without extending life. Compassionate care is overseen by a physician, and may be carried out either in the hospital or at home. Studies show that when presented with all options, about half of parents choose life-prolonging interventions and half choose compassionate care. A study in 2003 concluded that a selection of physicians who are experts in the care of children with HLHS were evenly split when asked what they would do if their own children were born with HLHS, with 1/3 stating that they would choose surgery, 1/3 stating that they would choose palliative (compassionate) treatment without surgery, and 1/3 stating that they are uncertain which choice they would make.

The three-stage procedure is a palliative procedure (not a cure), as the child's circulation is made to work with only two of the heart's four chambers.