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Sitaram M. Emani
Emile A. Bacha
Doff B. McElhinney
Frank A. Pigula
Pedro J. del Nido
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J Thorac Cardiovasc Surg 2009;138:1276-1282
© 2009 The American Association for Thoracic Surgery

Congenital Heart Disease

Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart

Sitaram M. Emani, MD, Emile A. Bacha, MD, Doff B. McElhinney, MD, MPH, Gerald R. Marx, MD, Wayne Tworetzky, MD, Frank A. Pigula, MD, Pedro J. del Nido, MD*

Children's Hospital Boston, Boston, Mass

Received for publication May 1, 2009; revisions received July 26, 2009; accepted for publication August 9, 2009.

* Address for reprints: Pedro J. del Nido, MD, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA 02115. (Email: pedro.delnido{at}cardio.chboston.org).

Objective: Borderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation—endocardial fibroelastosis resection with mitral or aortic valvuloplasty—on left ventricular function and clinical outcomes.

Methods: All patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test.

Results: Between 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1–38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively.

Conclusion: In patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group.

Abbreviations and Acronyms ASD = atrial septal defect; EFE = endocardial fibroelastosis; LV = left ventricular; LVOT = left ventricular outflow tract; MRI = magnetic resonance imaging; UVR-SA = Univentricular Survival Advantage






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