Asian Cardiovasc Thorac Ann 2006;14:489-494
© 2006 Asia Publishing EXchange Ltd
Surgical Outcome of Heterotaxy Syndrome in a Single Institution
Koh Takeuchi, MD,
Arata Murakami, MD,
Yasutaka Hirata, MD,
Kazuo Kitahori, MD,
Yoshiroh Doi, MD,
Shunichi Takamoto, MD
Department of Cardiac Surgery, University of Tokyo Graduate School of Medicine, Tokyo, Japan
For reprint information contact: Koh Takeuchi, MD Tel: 81 33 416 0181 Fax: 81 33 416 2222 email: koutakeuchi-circ{at}umin.ac.jp, Department of Cardiovascular Surgery, National Children’s Hospital and National Center for Child Health and Development, 2-10-1, Ohkura, Setagaya-ku, Tokyo 157-8535, Japan.
Heterotaxy syndrome encompasses a wide range of anatomical variants including poor pulmonary vascular development. Surgical outcome remains poor in this difficult subgroup. Between April 1996 and November 2004, 27 patients with visceral heterotaxy were enrolled in this study. The median age at presentation was 25 days. There were 11 patients with asplenia and 16 with polysplenia. There were 5 deaths (4 in patients with asplenia, 1 in a patient with polysplenia). All patients with asplenia were scheduled for single-ventricle repair, and 5 completed a modified Fontan procedure. Of the 16 patients with polysplenia, 8 completed 2-ventricle repair and 6 had a modified Fontan. In polysplenia, actuarial survival was 93.8% at 4 months, and remained constant thereafter. In asplenia, survival was 81.8% at 1 month, and 53.0% at 1 year. A significant correlation was found between nonconfluent pulmonary arteries and mortality. The medium-term result in polysplenia was satisfactory, but not in asplenia. Nonconfluent pulmonary arteries carry a high risk of mortality, and a strategy to create pulmonary vascular confluence and satisfactory pulmonary blood flow is mandatory.
Copyright © 2006 by the Asia Publishing EXchange Ltd.
