Dextrocardia in children: Review of 15 cases

Abstract

Dextrocardia is a rare cardiac anomaly, which is divided into 3 different types. Dextrocardia with complete situs inversus occurs in approximately 2 per 10.000 live births. The incidence of congenital heart disease is relatively low among these individuals and is estimated to be about 3 %. Dextrocardia with situs solitus or situs ambiguous is considerably less common and occurs in perhaps 1 per 20.000 live births. The incidence of congenital heart disease is extremely high in this situation and lies probably in the range of 90% or greater. The frequency of different types of Dextrocardia is as follows: Situs solitus 48.5%, situs ambiguus 33.8%, and situs inversus 17.7% polysplenia or asplenia is found in about one-third of patients with Dextrocardia and almost invariably with situs ambiguous. The aim of this retrospective investigation was to find the incidence of different types of Dextrocardia. During a period of 5 years from 1988 to 1993 15 patients with Dextrocardia were admitted to pediatric cardiology departments in 4 university hospitals in Tehran. 9 (60%) were male and 6 (40%) female. The frequency of different types of Dextrocardia in study group was as follows: situs inversus 8 (57.2%), situs solitus 4 (28.6%), situs ambiguus 2 (14.2%) and dextroversion due to right lung sequestration 1 (6.6%). The incidence of congenital heart disease among these individuals was as follows: situs inversus 4 (50%), situs solitus 3 (75%), and situs ambiguus 2 (100%). different types of congenital heart disease were as follows: VSD 4 (28.6%), TGA 4 (28.6%) and fallot's tetralogy 1 (7.1%). 8 patients (53.3%) had also extra-cardiac malformation and in 1/3 of them this was a musculoskeletal anomaly. 1 patient suffered from karthagener syndrome. In this study the order to frequency of different types of Dextrocardia and the incidence of congenital heart disease in situs inversus differed from that published in the literature