Key result
Fetal cardiac ultrasound accurately identifies Uhl anomaly with severe right ventricular dilatation at 20 weeks.
Why the study?
Prenatal diagnosis of Uhl anomaly is rare and important for prognosis and management, but few cases with fetal autopsy correlation have been reported.
Case Report (n=1)
May enable mid-gestation ultrasound detection of Uhl anomaly; leaves open validation in larger prospective series.
Uhl anomaly is a rare form of congenital hypoplasia of the right ventricular myocardium. Here, we report, a rare finding in fetal cardiac ultrasound in a 33-year-old woman who presented at 20 weeks' of gestation. A diagnosis of Uhl anomaly was made. An autopsy was performed at 23weeks gestation after obtaining permission for medicolegal termination of pregnancy. Histopathological examination confirmed the diagnosis. Diagnosing Uhl anomaly in fetal life is essential since mortality and survival mainly depend on the severity of right ventricle dysfunction related to, the either partial or complete absence of the myocardium. Hence, surviving cases need to be followed up carefully and counselled accordingly.
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Bharati et al. (2015) conducted a case report in Fetal Uhl anomaly (n=1). Prenatal echocardiography and fetal autopsy was evaluated on Correlation of prenatal ultrasound diagnosis with autopsy findings. Fetal cardiac ultrasound at 20 weeks' gestation successfully diagnosed Uhl anomaly, characterized by severe right ventricular dilatation, which was confirmed by subsequent fetal autopsy.
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