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

被引：2

作者：

Cohen, Jennifer ^{[1
]}

Arya, Bhawna ^{[2
]}

Caplan, Richard ^{[3
]}

Donofrio, Mary T. ^{[4
]}

Ferdman, Dina ^{[5
]}

Harrington, Jamie K. ^{[6
]}

Ho, Deborah Y. ^{[7
]}

Hogan, Whitnee ^{[8
]}

Hornberger, Lisa K. ^{[9
]}

Jhaveri, Simone ^{[10
]}

Killen, Stacy A. S. ^{[11
]}

Lindblade, Christopher L. ^{[12
]}

Michelfelder, Erik ^{[13
]}

Moon-Grady, Anita J. ^{[14
]}

Patel, Sheetal ^{[15
]}

Quezada, Emilio ^{[14
]}

Ronai, Christina ^{[16
]}

Mejia, Aura A. Sanchez ^{[17
]}

Schidlow, David N. ^{[18
]}

Stiver, Corey ^{[19
]}

Thakur, Varsha ^{[20
]}

Srivastava, Shubhika ^{[21
]}

机构：

[1] Icahn Sch Med Mt Sinai, New York, NY USA

[2] Seattle Childrens Hosp, Seattle, WA USA

[3] Christiana Care Hlth Syst, Wilmington, DE USA

[4] Childrens Natl Hosp, Washington, DC USA

[5] Yale Sch Med, New Haven, CT USA

[6] Columbia Univ, Med Ctr, New York, NY USA

[7] Lucile Packard Childrens Hosp, Stanford Sch Med, Palo Alto, CA USA

[8] Univ Utah, Salt Lake City, UT USA

[9] Univ Alberta, Edmonton, AB, Canada

[10] Northwell Hlth Physician Partners, New Hyde Pk, NY USA

[11] Vanderbuilt Univ, Med Ctr, Nashville, TN USA

[12] Phoenix Childrens Hosp, Phoenix, AZ USA

[13] Emory Univ, Childrens Healthcare Atlanta, Atlanta, GA USA

[14] Univ Calif San Francisco, San Francisco, CA USA

[15] Northwestern Univ, Ann & Robert H Lurie Childrens Hosp Chicago, Feinberg Sch Med, Chicago, IL USA

[16] Oregon Hlth & Sci Univ, Portland, OR USA

[17] Texas Childrens Hosp, Houston, TX USA

[18] Boston Childrens Hosp, Boston, MA USA

[19] Nationwide Childrens Hosp, Columbus, OH USA

[20] Hosp Sick Children, Toronto, ON, Canada

[21] Nemours Childrens Hlth Syst, Wilmington, DE USA

来源：

JOURNAL OF THE AMERICAN HEART ASSOCIATION | 2023年 / 12卷 / 11期

基金：

美国国家卫生研究院;

关键词：

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

D O I：

10.1161/JAHA.122.029706

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

BackgroundFetal 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 ResultsFetuses 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). ConclusionsAssociated 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.

引用

页数：21

共 34 条

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