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One-stage repair in early infancy of tetralogy of Fallot with ‘absent pulmonary valve’ syndrome

Published online by Cambridge University Press:  19 August 2008

Toshikatsu Yagihara*
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
Hidefumi Kishimoto
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
Fumio Yamamoto
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
Kyoichi Nishigaki
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
Osamu Matsuki
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
Hideki Uemura
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
Osahiro Takahashi
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
Tetsuroh Kamiya
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
Yasunaru Kawashima
Affiliation:
From the Departments of Cardiovascular Surgery and Pediatric Cardiology, National Cardiovascular Center, Suita
*
Dr. Toshikatsu Yagihara, Department of Cardiovascular SurgeryNational Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita-city, 565Japan. Tel. 6-833-5012; Fax. 6-872-7486.

Abstract

Five infants under three months of age with tetralogy of Fallot and absence of the leaflets of the pulmonary valve were successfully corrected using a one-stage repair. All patients suffered from respiratory failure preoperatively and surgery was performed somewhat urgently. The age at surgery ranged from 26 to 72 days, with an average of 47±17 days. The surgical procedures involved four steps. First, we plicated the aneurysmally dilated central pulmonary arteries. Second, we closed the ventricular septal defect. We then inserted a handmade heterograft pericardial valve at the site of the pulmonary valve, and completed the reconstruction of the right ventricular outflow tract with a patch. All patients survived. Two of them were successfully weaned from ventilation at early (two and 11 days, respectively) periods after surgery, but the remaining three patients required artificial ventilation for one to two months. We conclude that one-stage repair is possible for this anomaly, but that plication of the pulmonary trunk is an essential technique todeliver patients in early infancy from respiratory failure.

Type
Original Articles
Copyright
Copyright © Cambridge University Press 1994

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