Pediatric Cardiology and Cardiac Surgery
Vol.27 No.4 2011 (163-167)

Toshikatsu Yagihara

National Cerebral and Cardiovascular Center, Osaka, Japan


Heterotaxy syndrome, especially a right atrial isomerism, is generally associated with complex cardiac malformations, including a total anomalous pulmonary venous connection and regurgitation of the common atrioventricular valve, which had been the major surgical risk during early infancy. During latest three decades, better surgical outcomes from palliative surgery for heterotaxy syndrome were obtained due to various morphological knowledge, a developed open heart surgery system, and the development of procedures. As a result, mortality involving the modified Fontan operation dropped since the 1990s. In limited patients with well-balanced ventricles, the reasonable long-term results of biventricular repair have gradually been elucidated. Nevertheless, further improvement is needed because surgical treatment of heterotaxy is the most difficult among the congenital heart diseases.