Original article
Pediatric cardiac
Hypoplastic Left Heart Syndrome: Feasibility Study for Patients Undergoing Completion Fontan at or Prior to Two Years of Age

Presented at the Fifty-sixth Annual Meeting of the Southern Thoracic Surgical Association, Marco Island, FL, Nov 4–7, 2009.
https://doi.org/10.1016/j.athoracsur.2010.04.079Get rights and content

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.

Section snippets

Study Design

The Heart Institute for Children's database was retrospectively reviewed and analyzed. All patients who had undergone CF after Norwood operation and bidirectional Glenn for HLHS from August 1999 to December 2008 were included. Patients classified as single ventricle anatomy other than HLHS were excluded (including unbalanced atrioventricular septal defect, double outlet right ventricle with mitral and (or) aortic atresia, or heterotaxy syndrome). Only patients with a hypoplastic left ventricle

Results

The pre-CF catheter interventions performed included coil occlusion of systemic-pulmonary collaterals or pulmonary arteriovenous malformation and balloon dilatation of pulmonary arteries (Table 2). Pre-CF main pulmonary artery diameter, MGr, and TPG were 7.94 ± 0.59 mm, 1.79 ± 0.2, and 4.1 ± 1.1 mm Hg for group A compared with 7.87 ± 0.58 mm, 1.77 ± 0.19, and 3.5 ± 0.9 mm Hg for group B (p = NS), respectively (Table 2). The pulmonary resistance to systemic resistance ratio and pulmonary

Comment

Better understanding of the natural history and anatomic morphology of HLHS, advances in surgical techniques and intraoperative myocardial protection, and comprehensive postoperative management have contributed to a remarkable improvement in early and late mortality, before and after CF [5, 6, 7, 8, 9, 10]. However, elimination of cyanosis and right ventricle unloading from systemic and pulmonary circulation by CF at an earlier age remains a controversial topic. Volume unloading can be evident

References (35)

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