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J Thorac Cardiovasc Surg 2003;126:504-510
© 2003 The American Association for Thoracic Surgery

Surgery for congenital heart disease

Right ventricle–pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome

Shunji Sano, MDa,*, Kozo Ishino, MDa, Masaaki Kawada, MDa, Sadahiko Arai, MDa, Shingo Kasahara, MDa, Tomohiro Asai, MDa, Zen-ichi Masuda, MDa, Mamoru Takeuchi, MDb, Shin-ichi Ohtsuki, MDc

a Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan
b Department of Anesthesiology, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan
c Department of Pediatrics,c Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan

Read at the Eighty-second Annual Meeting of The American Association for Thoracic Surgery, Washington, DC, May 5-8, 2002.

Received for publication June 5, 2002; revisions received September 5, 2002; revisions received November 7, 2002; accepted for publication December 2, 2002.

* Address for reprints: Shunji Sano, MD, Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine and Dentistry, 2-5-1 Shikata-cho, Okayama-City 700-8558, Japan
s_sano{at}cc.okayama-u.ac.jp

OBJECTIVE: Pulmonary overcirculation through a systemic-pulmonary shunt has been one of the major causes of early death after the Norwood procedure. To avoid this lethal complication, we constructed a right ventricle–pulmonary shunt in first-stage palliation of hypoplastic left heart syndrome.

METHODS: Between February 1998 and February 2002, 19 consecutive infants, aged 6 to 57 days (median, 9 days) and weighing 1.6 to 3.9 kg (median, 3.0 kg), underwent a modified Norwood operation with the right ventricle–pulmonary artery shunt. The procedure included aortic reconstruction by direct anastomosis of the proximal main pulmonary artery and a nonvalved polytetrafluoroethylene shunt between a small right ventriculotomy and a distal stump of the main pulmonary artery. The size of the shunt used was 4 mm in 5 patients and 5 mm in 14.

RESULTS: All patients were managed without any particular manipulation to control pulmonary vascular resistance. There were 17 survivors (89%), including 3 patients weighing less than 2 kg. Two late deaths occurred due to obstruction of the right ventricle–pulmonary artery shunt. Thirteen patients underwent a stage II Glenn procedure after a mean interval of 6 months, with 2 hospital deaths. To date, a stage III Fontan procedure has been completed in 4 patients. Overall survival was 62% (13/19). Right ventricular fractional shortening at the last follow-up (3-48 months after stage I) ranged from 26% to 43% (n = 13, mean, 33%).

CONCLUSION: Without delicate postoperative management to control pulmonary vascular resistance, the modified Norwood procedure using the right ventricle–pulmonary shunt provides a stable systemic circulation as well as adequate pulmonary blood flow. This novel operation may be particularly beneficial to low-birth-weight infants with hypoplastic left heart syndrome.




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