All issues > Volume 36(4); 1993

Original Article

J Korean Pediatr Soc. 1993;36(4):543-549. Published online April 15, 1993.

Morphlolgical Analysis of the Congenital Heart Disease Associated with Infrahepatic Interruption of IVC with Azygos Contnuation

Jun Hee JH Sul¹, Jong Kyun JK Lee¹, Sung Kyu SK Lee¹

¹Division of Pediatric Cardiology, Cardiovascular Center, Yonsei University, College of Medicine, Seoul, Korea

Abstract

Infrahepatic interruption of the inferior vena cava with azygos continuation is elatively infrequent cardiovascular developmental anomaly occurring both in association with congenital heart disease and as an isolated anomaly of no hemodynamic importance. During the past 5years, we observed 38cases of infrahepatic interruption of IVC with azygos continuation out of 2,397 cases of congenital heart disease catheterized at Yonsei Cardiovascular Center. We conducted the study with a view point of position of the heart and abdominal organs and segmental analysis of the underlying congenital heart disease. We also analysed the associated extracardiac vascular anomalies. The following results were abtained: 1) The incidence of this anomaly among congenital heart disease was 1.7% and the sex ratio 1.4:1. Twenty four cases (63.1%) was below 5years of age and 30 cases(79.0%) had cyanosis. 2) We observed 21 cases (55.2%) with the malposition of the heart and 17 cases(44.8%) with malposition of the abdominal organs. The ventricular loops revealed D-loop in 25 cases, L-loop in 5 cases and in the remaining 7 cases, it was uncertain. Eighteen cases showed normally related great arteries and malposition was present in another 18 cases. 3) Associated cardiovascular anomalies were as follows: right-sided arch;12 cases, patent ductus arteriosus; 10 cases, pulmonic stenosis?19 cases and bilateral superior vena cava;9 cases. In conclusion, the presence of this anomaly should alert one to seek the severe intracardiac anomalies which are usually associated with it. Also, ith is essential that, if any patient with this anomaly should come to thoracotomy, the azygos vein be not sacrificed.

Keywords :Interruption of IVC, Azygos continuation and Malposition