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Meena Nathan
Pedro J. del Nido
John E. Mayer
Emile A. Bacha
Frank A. Pigula
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J Thorac Cardiovasc Surg 2007;133:1329-1335
© 2007 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Early repair of hemitruncus: Excellent early and late outcomes

Meena Nathan, MD, David Rimmer, MS, Gary Piercey, BS, Pedro J. del Nido, MD, John E. Mayer, MD, Emile A. Bacha, MD, Frank A. Pigula, MD*

Children’s Hospital, Boston, Harvard Medical School, Boston, Mass.

Poster Presentation at the Western Thoracic Surgical Association meeting, Sun Valley Idaho, June 21-24, 2006.

Received for publication June 19, 2006; revisions received November 10, 2006; accepted for publication December 26, 2006.

* Address for reprints: Frank A. Pigula, MD, Department of Cardiac Surgery, Children’s Hospital, 300, Longwood Ave, Boston, MA 02115. (Email: frank.pigula{at}childrens.harvard.edu).

Objective: Anomalous origin of 1 of the branch pulmonary arteries from the aorta with 2 normal semilunar valves (hemitruncus) is a rare entity. There have been several small case series reported. We report here our single-institution surgical experience with hemitruncus from 1982 to 2006.

Methods: A retrospective case review of all cases of conotruncal anomalies at Children’s Hospital Boston revealed 16 patients with hemitruncus. Ten patients had surgery in the neonatal period (<30 days), 4 at 1 to 6 months, 1 at 8 months, and 1 at 2 years. Diagnosis of hemitruncus was the indication for operation in all but 1. Fourteen of the 16 had anomalous right pulmonary artery from aorta, and 2 had left pulmonary artery from aorta. Common associated anomalies included patent foramen ovale in 14, patent ductus arteriosus in 11, and ventricular septal defect in 4. All patients had elevated right ventricular pressures with systemic pressures in 5 and suprasystemic pressures in 9.

Results: There was 1 operative death in this series in an infant who died from sepsis following ligation of a tracheoesophageal fistula. One patient required reoperation for supravalvar aortic stenosis and right pulmonary artery stenosis 1 year postoperatively. Three patients required 4 catheter-based interventions postoperatively. At 20 years, survival by Kaplan–Meier was 93%; freedom from reoperation, 93%; and freedom from catheter reintervention, 79%.

Conclusions: Early repair of hemitruncus results in excellent hemodynamic and anatomic results. Survival is excellent, with a low incidence of reoperation or reintervention.

Abbreviations and Acronyms LPA = left pulmonary artery; MPA = main pulmonary artery; PDA = patent ductus arteriosus; PFO = patent foramen ovale; PVOD = pulmonary vascular obstructive disease; RPA = right pulmonary artery; TEF = tracheoesophageal fistula; VSD = ventricular septal defects






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