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J Thorac Cardiovasc Surg 2008;135:339-346
© 2008 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Surgical outcome for patients with the mitral stenosis–aortic atresia variant of hypoplastic left heart syndrome

^a Department of Cardiac Surgery, Children’s Hospital Boston, Boston, Mass
^b Department of Cardiology, Children’s Hospital Boston, Boston, Mass.

Received for publication May 9, 2007; revisions received August 30, 2007; accepted for publication September 14, 2007.

^_* Address for reprints: Francis Fynn-Thompson, MD, Department of Cardiac Surgery, Children’s Hospital Boston, Harvard Medical School, 300 Longwood Ave, Bader 273, Boston, MA 02115. (Email: francis.fynnthompson{at}cardio.chboston.org).

Objective: We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure.

Methods: From January 2001 through December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children’s Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis–aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan–Meier method was used to assess survival.

Results: Thirty-eight (23%) of 165 patients had mitral stenosis–aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis–aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle–subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis–aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time (P = .02) and the need for postoperative extracorporeal membrane oxygenation support (P < .001) were associated with a higher mortality rate.

Conclusions: With improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis–aortic atresia variant and left ventricle–subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.

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Discussion
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