Staged surgical palliation in hypoplastic left heart syndrome and its variants

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease Management

Background:

• Hypoplastic left heart syndrome (HLHS) presents limited surgical options: cardiac transplantation or staged palliation.
• Staged palliation involves the Norwood operation followed by a two-staged Fontan procedure.

Purpose of the Study:

• To describe a 17-year experience with staged palliation for HLHS and its variants.
• To evaluate outcomes and identify factors influencing mortality in HLHS treatment.

Main Methods:

• A cohort of 64 infants with HLHS underwent the Norwood procedure between 1989 and 2006.
• Follow-up data included 143.2 patient-years, with subsequent stages of palliation performed as indicated.

Main Results:

• Early mortality after the Norwood procedure significantly decreased from 39.06% (1989-1999) to 0% (2004-2006).
• Mortality causes included sepsis, capillary leak, and heart failure. Survivors experienced minimal long-term complications.

Conclusions:

• Outcomes in staged palliation for HLHS have improved due to accumulated experience and expertise.
• Lower operative weight, ascending aortic size, and cardiopulmonary bypass duration significantly impact early Norwood procedure mortality.