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Ann Thorac Surg 2001;71:49-53
© 2001 The Society of Thoracic Surgeons

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Original article: cardiovascular

Surgical outcome of double-outlet right ventricle with subpulmonary VSD

^a Department of Cardiac Surgery, The Children’s Hospital, Boston, Massachusetts, USA
^b Department of Anesthesiology, The Children’s Hospital, Boston, Massachusetts, USA
^c Department of Cardiology, The Children’s Hospital, Boston, Massachusetts, USA
^d Department of Biostatistics, The Children’s Hospital, Boston, Massachusetts, USA

Address reprint requests to Dr del Nido, Department of Cardiac Surgery, The Children’s Hospital, 300 Longwood Ave, Bader 279, Boston, MA 02115
e-mail: delnido{at}al.tch.harvard.edu

Presented at the Thirty-sixth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 31–Feb 2, 2000.

Background. Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial. We reviewed our 7-year experience with patients who had this anatomic configuration.

Methods. Between January 1992 and January 1999, 20 patients underwent an arterial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan). Mean follow-up was 23 ± 18 months.

Results. An initial palliative operation was done in 19 patients (9 in the ASO group, 10 in the Glenn/Fontan group). There were no deaths in the Glenn/Fontan group. Four patients in the ASO group died within 33 days postoperatively. Two of them had a single coronary artery, 1 had a straddling mitral valve, 1 had a hypoplastic aortic arch, and 1 had multiple ventricular septal defects. Three patients had reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO. Four patients (3 in the ASO group, 1 in the Glenn/Fontan) required a pacemaker for postoperative complete atrioventricular block. Actuarial survival at 5 years for the entire group was 87% (70% confidence interval, 81% to 93%).

Conclusions. The ASO remains our preferred treatment for infants with double-outlet right ventricle and subpulmonary ventricular septal defect. However, associated anatomic defects are important risk factors.

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