Congenitally Corrected Transposition of the Great Arteries: Fetal Diagnosis, Associations, and Postnatal Outcome: A Fetal Heart Society Research Collaborative Study

Jennifer Cohen, Bhawna Arya, Richard Caplan, Mary T. Donofrio, Dina Ferdman, Jamie K. Harrington, Deborah Y. Ho, Whitnee Hogan, Lisa K. Hornberger, Simone Jhaveri, Stacy A.S. Killen, Christopher L. Lindblade, Erik Michelfelder, Anita J. Moon-Grady, Sheetal Patel, Emilio Quezada, Christina Ronai, Aura A.Sanchez Mejia, David N. Schidlow, Corey StiverVarsha Thakur, Shubhika Srivastava

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

BACKGROUND: Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly re-ported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. METHODS AND RESULTS: Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included. Fetuses with severe ventricular hypoplasia thought to definitively preclude biventricular repair were excluded. We included 205 fetuses diagnosed with ccTGA at a median gestational age of 23 (interquartile range, 21–27) weeks. Genetic abnormalities were found in 5.9% tested, with extracardiac anomalies in 6.3%. Associated cardiac defects were diagnosed in 161 (78.5%), with atrioventricular block in 23 (11.3%). On serial fetal echocardiogram, 39% demonstrated a functional or anatomic change, most commonly increased tricuspid regurgitation (6.7%) or pulmonary outflow obstruction (11.1%). Of 194 fetuses with follow-up, 26 were terminated, 3 experienced fetal death (2 with atrioventricular block), and 165 were live-born. Of 158 with postnatal data (median follow-up 3.7 years), 10 (6.6%) had death/transplant before 1 year. On univariable analysis, fetal factors associated with fetal death or death/transplant by 1 year included ≥ mild tricuspid regurgitation, pulmonary atre-sia, aortic obstruction, fetal arrhythmia, and worsening hemodynamics on serial fetal echocardiogram (defined as worse right ventricular function, tricuspid regurgitation, or effusion). CONCLUSIONS: Associated cardiac lesions and arrhythmias are common in fetal ccTGA, and functional changes commonly occur through gestation. Worse outcomes are associated with fetal tricuspid regurgitation (≥mild), any arrhythmia, pulmonary atresia, aortic obstruction, and worsening hemodynamics on serial echocardiograms. These findings can inform prenatal counseling and perinatal management planning.

Original languageEnglish
Article numbere029706
JournalJournal of the American Heart Association
Volume12
Issue number11
DOIs
StatePublished - 6 Jun 2023

Keywords

  • atrioventricular block
  • congenitally corrected transposition of the great arteries
  • fetal echocardiography
  • situs inversus

Fingerprint

Dive into the research topics of 'Congenitally Corrected Transposition of the Great Arteries: Fetal Diagnosis, Associations, and Postnatal Outcome: A Fetal Heart Society Research Collaborative Study'. Together they form a unique fingerprint.

Cite this