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Masahiro Koh
Toshikatsu Yagihara
Hideki Uemura
Ikuo Hagino
Toru Ishizaka
Soichiro Kitamura
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Ann Thorac Surg 2006;81:1808-1816
© 2006 The Society of Thoracic Surgeons

Original article: Cardiovascular

Biventricular Repair for Right Atrial Isomerism

Masahiro Koh, MD a , Toshikatsu Yagihara, MD a , * , Hideki Uemura, MD b , Koji Kagisaki, MD a , Ikuo Hagino, MD a , Toru Ishizaka, MD a , Soichiro Kitamura, MD a

a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
b Department of Cardiothoracic Surgery, Royal Brompton Hospital, London, United Kingdom

Accepted for publication December 1, 2005.

* Address correspondence to Dr Yagihara, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan (Email: yagihara{at}hsp.ncvc.go.jp).

Presented at the Forty-first Annual Meeting of The Society of Thoracic Surgeons, Tampa, FL, Jan 24–26, 2005.

BACKGROUND: Biventricular repair is often difficult to accomplish in patients with right atrial isomerism because of complex anomalous structures.

METHODS: Ten patients with right atrial isomerism underwent biventricular repair. Their ages ranged from 15 months to 21 years. The follow-up period ranged from 1 month to 21 years. The Fontan procedure was unsuitable in 7 patients. The atrioventricular valves were separated in 5 patients and common in 5. One patient who had severe right-sided atrioventricular valvular regurgitation required concomitant prosthetic valve replacement. Another patient with a hypoplastic intraventricular septum underwent ventricular septation. Nine patients had two balanced ventricles. A ventricular septal defect was enlarged for rerouting in 3 patients. All patients had anomalous venoatrial connections and required intra-atrial baffle rerouting. One with major aortopulmonary collateral arteries underwent staged unifocalization. Three had extracardiac conduit repair. The outcomes were compared with 97 patients who underwent the Fontan procedure.

RESULTS: There were 3 early deaths and 1 late death. Six survivors are in New York Heart Association functional class I or II. Two are free from medications. Two required reoperation owing to infection or prosthetic valve failure. At 1 year, cardiac index and systemic venous pressure were 3.2 ± 0.9 L · min–1 · m–2 and 6.6 ± 1.6 mm Hg, respectively. There were no significant differences in survival, freedom from arrhythmia, freedom from reoperation, or exercise tolerance between biventricular repair and the Fontan procedure.

CONCLUSIONS: Biventricular repair provided good long-term outcomes in patients with right atrial isomerism. In selected circumstances, biventricular repair is an acceptable alternative to the Fontan procedure.






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