Key result
Fetal arrhythmias, outflow obstruction, and tricuspid regurgitation link to ~7% death or transplant by one year.
Why the study?
Fetal diagnosis of congenitally corrected transposition of the great arteries has been increasingly reported, but predictors of clinical outcomes remain underexplored.
Cohort (n=205)
Yes
Fetal diagnosis of ccTGA is frequently associated with other cardiac defects and arrhythmias, and specific echocardiographic features like tricuspid regurgitation and worsening hemodynamics predict poor early outcomes.
Supports intensified prenatal monitoring for identified risk factors in fetal ccTGA; leaves open whether interventions improve 1-year survival.
Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; 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 atresia, 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.
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Cohen et al. (2023) conducted a cohort in Congenitally corrected transposition of the great arteries (ccTGA) (n=205). Fetal echocardiographic and clinical risk factors vs. Absence of risk factors was evaluated on Fetal death or death/transplant by 1 year. Fetal risk factors including ≥mild tricuspid regurgitation, arrhythmias, and outflow obstruction were associated with fetal death or death/transplant by 1 year, which occurred in 6.6% of cases.
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