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J Thorac Cardiovasc Surg 2008;136:749-756
© 2008 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Treatment of right ventricle to coronary artery connections in infants with pulmonary atresia and intact ventricular septum

^a Division of Cardiothoracic Surgery, University of Minnesota, Minneapolis, Minnesota
^c Department of Cardiothoracic Surgery, The Ochsner Clinic, New Orleans, Louisiana
^b Division of Pediatric Cardiology, University of Minnesota, Minneapolis, Minnesota
^d Department of Anatomy and Embryology, Leiden University Medical Center, Leiden, The Netherlands

Received for publication July 2, 2007; revisions received February 25, 2008; accepted for publication March 30, 2008.

^_* Address for reprints: John E. Foker, MD, PhD, Robert and Sharon Kaster Professor of Surgery, Division of Cardiac Surgery, University of Minnesota, 420 Delaware Street SE, MMC 495, Minneapolis, MN 55455. (Email: foker001{at}umn.edu).

Objective: At the severe end of the spectrum of infants with pulmonary atresia and intact ventricular septum, the likelihood of significant right ventricle to coronary artery connections increases. Our purpose is to present the first series of right ventricle to coronary artery connections ligated off bypass before right ventricular decompression and to evaluate the consequences of this approach.

Methods: From 1988 to 2007, 19 patients with pulmonary atresia and intact ventricular septum had a total of 69 right ventricle to coronary artery connections identified preoperatively, and 10 more were located intraoperatively. Of these, 71 were judged large enough to warrant off-pump direct ligation. Preoperative diagnosis was by transthoracic echocardiography and angiography. Transesophageal and surface echocardiography were used for intraoperative location. Direct visualization and echocardiographic assessment for regional wall motion abnormalities determined the effects of ligation. Right ventricular decompression was done in all patients.

Results: After ligation, coronary flow converted from moderately or largely retrograde to antegrade pefusion. Ligation produced no visual myocardial consequences or immediate local wall motion abnormalities. For 3 patients, however, apical-septal wall motion abnormalities appeared from 2 hours to 3 days postoperatively. Serial studies were done to assess the later effects in the 16 of 19 30-day survivors. No evidence for myocardial injury was found, and all continued on a 2-ventricle repair course.

Conclusion: The location and ligation of right ventricle to coronary artery connections can be reliably accomplished off bypass. Coronary flow became antegrade, improving myocardial oxygenation. No myocardial damage was observed. Inapparent right ventricle to coronary artery connections occasionally enlarge secondarily after right ventricular decompression, making early follow-up evaluation necessary after ligation. Despite the initial presence of significant right ventricle to coronary artery connections, 2-ventricle repairs are possible with long-term benefits.