Congenital Cardiac Malformations Associated with Dextrocardia: Analysis of 75 Patients in a Tertiary Center

Dextrocardia is a rare congenital anomaly. Careful examination is essential because of the coexistence of additional cardiac malformations that may require early treatment. The aim of this study is to evaluate the types and frequency of congenital cardiac malformations associated with dextrocardia. Patients who were diagnosed with dextrocardia were retrospectively examined in 51.045 children who had admitted to pediatric cardiology department. Clinical and echocardiographic findings as well as interventional and surgical procedures were analyzed. Of the total 75 cases, 60% were male. Median age at diagnosis was 2.95 months, (0 days-15.6 years). Situs inversus dextrocardia (SID) was found in 70.7% of cases, while situs solitus (SSD) and situs ambiguous dextrocardia (SAD) was found in 22.7% and 6.6%, respectively. Thirty-five (46.7%) patients had additional congenital heart disease (CHD). SAD was the most frequent subtype associated with additional CHD (100%) followed by SSD (76.5%) and SID (32.1%). Complex cardiac malformations including atrioventricular (AV) and ventriculoarterial discordance, malposition of great arteries, univentricular heart, AV valve and pulmonary atresia were detected. A total of 27 patients (36%) had undergone surgical or interventional procedures. Dextrocardia is closely associated with multiple complex CHDs that require surgical procedures. SID is more likely to have a structurally normal heart. SAD is definitely associated with additional CHDs, which are almost serious and complex defects. SSD usually has CHDs. Accurate identification of cardiac morphology with segmental analysis at early period of life is an important first step in improving the clinical course of patients. Thus, careful examination of heart structures via segmental analysis is essential in patients with dextrocardia