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Original Articles: Pediatric Cardiac

Ventricular Function Deteriorates With Recurrent Coarctation in Hypoplastic Left Heart Syndrome

^a Department of Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
^b Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts

Accepted for publication April 21, 2008.

^_* Address correspondence to Dr Bacha, Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115 (Email: emile.bacha{at}cardio.chboston.org).

Presented at the Forty-fourth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2008.

Background: Recurrent coarctation (re-CoA) after stage I palliation in hypoplastic left heart syndrome (HLHS) is deleterious. We studied whether re-CoA had an effect on ventricular systolic function.

Methods: Retrospectively reviewed were HLHS patients surviving stage I Norwood palliation (stage I) and cavopulmonary shunt (CPS) between January 2004 and February 2007. Echocardiographic right ventricular fractional area change (RV-FAC) was used to evaluate ventricular systolic function after stage I, before CPS, and before Fontan procedures. Cardiac catheterization and magnetic resonance imaging data before CPS were reviewed to assess re-CoA, using a coarctation index (CI = isthmus diameter/descending aortic diameter).

Results: Fifty-one patients were included, and 21 had a CI of less than 0.75 (mean, 0.82 ± 0.19; 21). Twelve patients required arch balloon dilation between CPS and Fontan. The change of RV-FAC for all patients between stage I and CPS was –2.2% ± 9.6%. Pearson correlation coefficient demonstrated a significant correlation between lower CI values and lower RV-FAC at the pre-CPS echocardiogram (r = .35, p = 0.03); and lower CI values and greater decrease in RV-FAC between stage I and pre-CPS evaluation (r = 0.40, p = 0.018). At follow-up pre-Fontan, RV-FAC for patients who underwent balloon dilation for re-CoA recovered to a level that was inferior but not significantly different from that of patients who did not need balloon dilation.

Conclusions: Recurrent aortic arch obstruction after stage I for HLHS is associated with worse RV systolic function at the time of stage II operation. Timely intervention on the re-CoA results in recovery of RV function.