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 journalArticle

331 Citations (Scopus)

Abstract

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)504-510
Number of pages7
JournalJournal of Thoracic and Cardiovascular Surgery
Volume126
Issue number2
DOIs
Publication statusPublished - Aug 1 2003

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Hypoplastic Left Heart Syndrome
Pulmonary Artery
Norwood Procedures
Heart Ventricles
Lung
Vascular Resistance
Fontan Procedure
Polytetrafluoroethylene
Low Birth Weight Infant
Survivors
Cause of Death
Survival

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

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Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. / Sano, Shunji; Ishino, Kozo; Kawada, Masaaki; Arai, Sadahiko; Kasahara, Shingo; Asai, Tomohiro; Masuda, Zen Ichi; Takeuchi, Mamoru; Ohtsuki, Shin-ichi; Litwin, S. Bert; Mavroudis, Constantine; Pozzi, Marco; Pizarro, Christian; Lacour-Gayet, Francois.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 126, No. 2, 01.08.2003, p. 504-510.

Research output: Contribution to journalArticle

Sano, S, Ishino, K, Kawada, M, Arai, S, Kasahara, S, Asai, T, Masuda, ZI, Takeuchi, M, Ohtsuki, S, Litwin, SB, Mavroudis, C, Pozzi, M, Pizarro, C & Lacour-Gayet, F 2003, 'Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome', Journal of Thoracic and Cardiovascular Surgery, vol. 126, no. 2, pp. 504-510. https://doi.org/10.1016/S0022-5223(02)73575-7
Sano, Shunji ; Ishino, Kozo ; Kawada, Masaaki ; Arai, Sadahiko ; Kasahara, Shingo ; Asai, Tomohiro ; Masuda, Zen Ichi ; Takeuchi, Mamoru ; Ohtsuki, Shin-ichi ; Litwin, S. Bert ; Mavroudis, Constantine ; Pozzi, Marco ; Pizarro, Christian ; Lacour-Gayet, Francois. / Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. In: Journal of Thoracic and Cardiovascular Surgery. 2003 ; Vol. 126, No. 2. pp. 504-510.
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abstract = "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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T1 - Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome

AU - Sano, Shunji

AU - Ishino, Kozo

AU - Kawada, Masaaki

AU - Arai, Sadahiko

AU - Kasahara, Shingo

AU - Asai, Tomohiro

AU - Masuda, Zen Ichi

AU - Takeuchi, Mamoru

AU - Ohtsuki, Shin-ichi

AU - Litwin, S. Bert

AU - Mavroudis, Constantine

AU - Pozzi, Marco

AU - Pizarro, Christian

AU - Lacour-Gayet, Francois

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N2 - 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.

AB - 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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