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Hypoplastic left heart syndrome: feasibility study for patients undergoing completion fontan at or prior to two years
Shyam Sathanandam1, Anastasios C Polimenakos, Christopher Blair
1Division of Pediatric Cardiovascular Surgery, Section of Cardiac Surgery, Department of Surgery, The Heart Institute for Children at Advocate Hope Children's Hospital, Oak Lawn, Illinois, USA.
Insights
Performing extracardiac conduit completion Fontan (CF) in children with hypoplastic left heart syndrome (HLHS) before age two yields good intermediate outcomes. Early CF may preserve ventricular function and alleviate cyanosis effects.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Fontan Procedure Management
Background:
- Limited data exists on managing hypoplastic left heart syndrome (HLHS) with completion Fontan (CF) in children under two years old.
- Investigating intermediate outcomes of early versus later extracardiac conduit CF in HLHS survivors is crucial for clinical practice.
Purpose of the Study:
- To evaluate intermediate outcomes of extracardiac conduit completion Fontan (CF) in hypoplastic left heart syndrome (HLHS) survivors.
- To compare outcomes between HLHS patients undergoing CF before 25 months versus after 25 months of age.
Main Methods:
- A cohort of 52 HLHS survivors underwent extracardiac-conduit CF between August 1999 and December 2008.
- Patients were divided into two groups: Group A (CF before 25 months) and Group B (CF after 25 months).
- Perioperative outcomes, including conduit size, fenestration rates, and cardiopulmonary bypass times, were analyzed.
Main Results:
- No in-hospital mortality and only one late death (in Group B) were observed.
- No significant differences were found in fenestration rates, cardiopulmonary bypass times, pulmonary artery size, McGoon ratio, or transpulmonary gradient between groups.
- Hospital length of stay, ICU stay, and pleural drainage duration were similar between the groups, with no significant differences in ventilatory support, arrhythmias, or thromboembolic events.
Conclusions:
- Extracardiac conduit CF can be performed with favorable intermediate results in HLHS patients at or before two years of age.
- Early CF may offer advantages in preserving future ventricular function and mitigating the effects of prolonged cyanosis.
- Age at CF did not impact preoperative pulmonary artery growth or conduit size selection, and fenestration was less frequently required.
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.