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Right aortic arch with mirror image branching accompanied by absent pulmonary valve syndrome and tricuspid stenosis: Prenatal echocardiographic diagnosis of an unusual congenital heart defect
Author(s) -
Yang Zexuan,
Zhou Liuying
Publication year - 2019
Publication title -
echocardiography
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.404
H-Index - 62
eISSN - 1540-8175
pISSN - 0742-2822
DOI - 10.1111/echo.14466
Subject(s) - medicine , cardiology , tricuspid valve , ventricle , stenosis , autopsy , tricuspid stenosis , aortic arch , hypoplastic left heart syndrome , radiology , aorta , heart disease
Right aortic arch (RAA) with mirror image branching (RAMI) accompanied by absent pulmonary valve syndrome (APVS), tricuspid stenosis, and hypoplastic right ventricle is an extremely rare combination of congenital heart anomalies. This combination might result in severe cardiac failure and respiratory compromise, indicating a poor prognosis. Here, we describe a detailed prenatal echocardiographic diagnosis of RAMI accompanied by APVS and tricuspid stenosis. These anomalies were further confirmed by autopsy. This case could be helpful in improving our understanding of this abnormal combination and the development of an early therapeutic strategy.

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