Corrected Transposition of the Great Arteries with Isolated Aortic Coarctation: In Utero Echocardiographic Diagnosis

Abstract

Physiologically corrected transposition of the great arteries (cTGA), defined by discordant atrioventricular and ventriculoarterial connections, is an uncommon congenital cardiac malformation. It rarely exists without associated cardiac anomalies, the most common of which are ventricular 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 significantly influence surgical repair and the patient's prognosis. We report a case of cTGA with complete heart block, moderate hypoplasia of the systemic ventricle, and severe aortic coarctation that was echocardiographically diagnosed in utero at 30 weeks' gestation because of fetal growth retardation and persistent fetal bradycardia. After delivery the patient underwent epimyocardial pacemaker implantation and aortic coarctation repair at 2 weeks of age. Unfortunately, the patient died on the seventh postoperative day because of systemic ventricular hypertrophy. 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.

Congenitally Corrected Transposition of the Great Arteries With Ebstein Malformation and Hypoplasia of the Aortic Arch in a Fetus

“…Fetal diagnosis of corrected transposition has been described [7,8]. However, the fetal echocardiographic features of this particular combination have not been detailed.…”

Echocardiographic Diagnosis of Transposition of the Great Arteries in a Neonatal Foal

“…Because complete transposition of the great vessels results in two separate parallel circulations (pulmonary and systemic), rather than two circulations in a series, these shunts must be present for the animal to survive. Therefore, complete transposition of the great vessels usually occurs in combination with other congenital cardiac defects 2–7 such as VSD, patent foramen ovale, and/or PDA. Additional intercirculatory shunting may be present in the form of extensive bronchopulmonary anastomotic channels, which are often present in children 6 and have been recognized in an equine case of complete transposition of the great vessels as well 5 .…”

“…Transposition of the great arteries is a common congenital heart defect in children 2,6 and has been sporadically reported in calves, 3,8 cats, 9 and horses 4,5 . It is thought to form during cardiogenesis as a result of abnormal truncus arteriosus development 2,8 . A genetic or teratogenic etiology is unconfirmed, but the defect has been associated with thalidomide teratogenicity in cats 10 .…”

Malformations cardiaques

“…Une fuite déjà présente in utero est encore plus péjorative ; • un obstacle sur la voie d'éjection aortique, rare mais possible : hypoplasie aortique, coarctation de l'aorte [338], interruption de la crosse aortique [339]. Même en l'absence d'insuffisance tricuspide foetale, on peut craindre que cette valve devienne incontinente à la naissance avec l'élévation des résistances systémiques.…”