Hypoplastic left heart syndrome: feasibility study for patients undergoing completion fontan at or prior to two years of age

Abstract

Background: There is limited data regarding the management of children with HLHS (hypoplastic left heart syndrome) and completion Fontan (CF) at or prior to 2 years of age. A study was undertaken to investigate intermediate outcomes.

Methods: From August 1999 to December 2008, 52 HLHS survivors underwent extracardiac-conduit CF (29 prior to [group A] versus 23 after 25 months of age [group B]). Mean weight and median follow-up was 9.9 +/- 1.3 kg and 79 months (2 to 112) for group A versus 12.1 +/- 2.2 kg and 87 months (1 to 97) for group B, respectively. Polytetrafluoroethylene conduits were used. Perioperative outcome variables were studied.

Results: There was no hospital mortality and 1 late death (group B). In group A, 16 CF had 20-mm conduit (vs 18 mm in 13). Thirteen group B patients received 18-mm conduit (vs 20 mm in 10). No CF was taken down. Eight patients in each group required fenestration (p = not significant [NS]). Cardiopulmonary bypass was 78 +/- 37.3 minutes in group A versus 77 +/- 33.9 minutes in group B (p = NS). Mean pulmonary artery size, McGoon ratio, and transpulmonary gradient in group A versus group B were 7.94 +/- 0.59 mm versus 7.87 +/- 0.58 (p = NS), 1.79 +/- 0.2 versus 1.77 +/- 0.19 (p = NS), and 4.1 +/- 1.1 mm Hg versus 3.5 +/- 0.9 (p = NS), respectively. Aspirin and warfarin were used postoperatively. Mean hospital length of stay, intensive care unit length of stay, and pleural drainage duration between groups A and B were the following: 10.9 +/- 5.8 days versus 12.7 +/- 6.1 (p = NS); 5.6 +/- 2.6 days versus 6.7 +/- 2.9 (p = NS); and 7.6 +/- 3.8 days versus 8.7 +/- 4.1 (p = NS), respectively. Between groups, no difference in ventilatory support time, arrhythmia, sinus-atrioventricular node dysfunction, protein-losing-enteropathy, and thromboembolic events were noted.

Conclusions: In HLHS patients, extracardiac conduit CF can be performed with good intermediate results at or prior to 2 years of age. Earlier unloading of a univentricular heart by means of CF in patients with collateral accessory flow between systemic and pulmonary circulation might be advantageous for future ventricular function preservation. In addition, relief from early cyanosis might alleviate deleterious effects from a prolonged cyanotic state. Fenestration is less often required without effect in pleural drainage duration. Age at CF had no effect in preoperative pulmonary artery growth and conduit size selection.