TY - JOUR
T1 - Congenitally Corrected Transposition of the Great Arteries
T2 - Fetal Diagnosis, Associations, and Postnatal Outcome: A Fetal Heart Society Research Collaborative Study
AU - Cohen, Jennifer
AU - Arya, Bhawna
AU - Caplan, Richard
AU - Donofrio, Mary T.
AU - Ferdman, Dina
AU - Harrington, Jamie K.
AU - Ho, Deborah Y.
AU - Hogan, Whitnee
AU - Hornberger, Lisa K.
AU - Jhaveri, Simone
AU - Killen, Stacy A.S.
AU - Lindblade, Christopher L.
AU - Michelfelder, Erik
AU - Moon-Grady, Anita J.
AU - Patel, Sheetal
AU - Quezada, Emilio
AU - Ronai, Christina
AU - Mejia, Aura A.Sanchez
AU - Schidlow, David N.
AU - Stiver, Corey
AU - Thakur, Varsha
AU - Srivastava, Shubhika
N1 - Funding Information:
This work was supported by an Institutional Development Award (IDeA) from the National Institute of General Medical Sciences of the National Institutes of Health under grant number U54-GM104941 (PI: Hicks). Funding for this project also includes the Matthews Hearts of Hope Grant (2019) and the Mount Sinai Early Career Pediatric Scholar Award (2020). This publication was developed with the support of the Fetal Heart Society sponsoring institutions, includ-ing the Children’s National Hospital, University of California, San Francisco Benioff, Children’s Hospital, The Hospital for Sick Children, The University of Utah School of Medicine/Intermountain Primary Children’s Hospital, Lucile Salter Packard Children’s Hospital at Stanford University, Texas Children’s Hospital, Columbia University Irving Medical, Center, Phoenix Children’s Hospital, Children’s Hospital of Minnesota, University Hospitals Rainbow Babies & Children’s Hospital, Children’s Healthcare of Atlanta, Mount Sinai Hospital, Children’s Health System of Texas (Dallas), Ann & Robert H Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Nemours Children’s Hospital & Foundation, Cincinnati Children’s Hospital Medical Center/UK Health Care Kentucky Children’s Hospital, Arkansas Children’s Hospital, The Children’s Mercy Hospital (Kansas), The Heart Institute at UPMC Children’s Hospital of Pittsburgh, Inova Health Care Services, Seattle Children’s Hospital, Nationwide Children’s Hospital, University of Virginia Children’s Hospital, University of California Davis Medical Center, Duke University, Monroe Carell Jr. Children’s Hospital at Vanderbilt, OSF Healthcare–Children’s Hospital of Illinois, Johns Hopkins All Children’s, Fairview Health Services, Children’s Hospital & Medical Center Omaha, Washington University School of Medicine in St. Louis, and Cleveland Clinic Children’s Hospital. Without their contributions, the evolution of the Fetal Heart Society and this and ongoing research would not be possible.
Funding Information:
This work was supported by an Institutional Development Award (IDeA) from the National Institute of General Medical Sciences of the National Institutes of Health under grant number U54-GM104941 (PI: Hicks). Funding for this project also includes the Matthews Hearts of Hope Grant (2019) and the Mount Sinai Early Career Pediatric Scholar Award (2020). This publication was developed with the support of the Fetal Heart Society sponsoring institutions, including the Children’s National Hospital, University of California, San Francisco Benioff, Children’s Hospital, The Hospital for Sick Children, The University of Utah School of Medicine/Intermountain Primary Children’s Hospital, Lucile Salter Packard Children’s Hospital at Stanford University, Texas Children’s Hospital, Columbia University Irving Medical, Center, Phoenix Children’s Hospital, Children’s Hospital of Minnesota, University Hospitals Rainbow Babies & Children’s Hospital, Children’s Healthcare of Atlanta, Mount Sinai Hospital, Children’s Health System of Texas (Dallas), Ann & Robert H Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Nemours Children’s Hospital & Foundation, Cincinnati Children’s Hospital Medical Center/UK Health Care Kentucky Children’s Hospital, Arkansas Children’s Hospital, The Children’s Mercy Hospital (Kansas), The Heart Institute at UPMC Children’s Hospital of Pittsburgh, Inova Health Care Services, Seattle Children’s Hospital, Nationwide Children’s Hospital, University of Virginia Children’s Hospital, University of California Davis Medical Center, Duke University, Monroe Carell Jr. Children’s Hospital at Vanderbilt, OSF Healthcare–Children’s Hospital of Illinois, Johns Hopkins All Children’s, Fairview Health Services, Children’s Hospital & Medical Center Omaha, Washington University School of Medicine in St. Louis, and Cleveland Clinic Children’s Hospital. Without their contributions, the evolution of the Fetal Heart Society and this and ongoing research would not be possible.
Publisher Copyright:
© 2023 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.
PY - 2023/6/6
Y1 - 2023/6/6
N2 - 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.
AB - 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.
KW - atrioventricular block
KW - congenitally corrected transposition of the great arteries
KW - fetal echocardiography
KW - situs inversus
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U2 - 10.1161/JAHA.122.029706
DO - 10.1161/JAHA.122.029706
M3 - Article
C2 - 37259984
AN - SCOPUS:85162061658
SN - 2047-9980
VL - 12
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
IS - 11
M1 - e029706
ER -