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

Shunji Sano, Kozo Ishino, Masaaki Kawada, Sadahiko Arai, Shingo Kasahara, Tomohiro Asai, Zen-ichi Masuda, Mamoru Takeuchi, Shin Ichi Ohtsuki, S. Bert Litwin, Constantine Mavroudis, Marco Pozzi, Christian Pizarro, Francois Lacour-Gayet

Research output: Contribution to journalArticlepeer-review

379 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)504-509
Number of pages6
JournalJournal of Thoracic and Cardiovascular Surgery
Issue number2
Publication statusPublished - Aug 1 2003

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine


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