Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of th
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MATERNAL-FETAL MEDICINE
Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries Andrea Krummholz1 · I. Gottschalk2 · A. Geipel1 · U. Herberg3 · C. Berg1,2 · U. Gembruch1 · A. Hellmund1 Received: 6 June 2020 / Accepted: 5 November 2020 © The Author(s) 2020
Abstract Purpose To analyze anatomic features and associated malformations in 37 prenatally detected cases of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate the prenatal course, neonatal outcome and mid-term follow-up. Methods Retrospective analysis of prenatal ultrasound of 37 patients with ccTGA in two tertiary centers between 1999 and 2019. All fetuses received fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports. Results Isolated ccTGA without associated cardiac anomalies was found in 13.5% (5/37), in all other fetuses additional defects such as VSD (73.0%), pulmonary obstruction (35.1%), tricuspid valve anomalies (18.9%), aortic arch anomalies (13.5%), ventricular hypoplasia (5.4%) or atrioventricular block (5.4%) were present. The rate of extracardiac malformations or chromosomal aberrations was low. There were 91.9% (34/37) live births and postnatal survival rates reached 91.2% in a mean follow-up time of 4.98 years. The prenatal diagnosis of ccTGA was confirmed postnatally in all but one documented live birth and the prenatal counselling regarding the expected treatment after birth (uni- versus biventricular repair) was reassured in the majority of cases. The postnatal intervention rate was high, 64.7% (22/34) received surgery, the intervention-free survival was 36.7%, 35.0% and 25.0% at 1 month, 1 year and 10 years, respectively. Conclusions ccTGA is a rare heart defect often associated with additional heterogeneous cardiac anomalies that can be diagnosed prenatally. The presented study demonstrates a favorable outcome in most cases but the majority of patients require surgical treatment early in life. Keywords Corrected transposition · Atrioventricular discordance · Prenatal diagnosis · Congenital heart disease · Fetus · Fetal echocardiography
Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare form of congenital heart disease, mainly characterized by both atrioventricular and ventriculoarterial discordance [1, 2]. The prevalence is 0.02 per 1000 live * Andrea Krummholz [email protected] 1
Department of Obstetrics and Prenatal Medicine, University of Bonn, Venusberg‑Campus 1, 53127 Bonn, Germany
2
Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University of Cologne, Cologne, Germany
3
Department of Pediatric Cardiology, University of Bonn, Bonn, Germany
births and it accounts for less than 1% of all congenital heart defects [1–5]. In ccTGA, the right atrium drains into a right-sided morphological left ventricle through the mitral valve and the left atrium into a left-side
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