CORRECTED TRANSPOSITION OF THE GREAT-ARTERIES WITH ISOLATED AORTIC COARCTATION - IN-UTERO ECHOCARDIOGRAPHIC DIAGNOSIS

Physiologically corrected transposition of the great arteries (cTGA), defined by discordant atrioventricular and ventriculoarterial connecti ons, is an uncommon congenital cardiac malformation. It rarely exists without associated cardiac anomalies, the most common of which are ven tricular septal defect, pulmonary outflow obstruction, tricuspid valve (systemic) deformity, and rhythm disturbances, Conversely, hypoplasia of the systemic ventricle and systemic inflow or outflow obstructions have seldom been reported, although their recognition may significant ly influence surgical repair and the patient's prognosis. We report a case of cTGA with complete heart block, moderate hypoplasia of the sys temic ventricle, and severe aortic coarctation that was echocardiograp hically diagnosed in utero at 30 weeks' gestation because of fetal gro wth retardation and persistent fetal bradycardia. After delivery the p atient underwent epimyocardial pacemaker implantation and aortic coarc tation repair at 2 weeks of age. Unfortunately, the patient died on th e seventh postoperative day because of systemic ventricular hypertroph y. Although it is well known that fetal echocardiography may reliably diagnose uncommon congenital cardiac malformations, to the best of our knowledge, this paper represents the first reported case of antenatal diagnosis of this complex anomaly.