Prenatal Diagnosis of Uhl Anomaly with Autopsy Correlation

Saji Philip; Sarasa Bharati; Kottureth Mammen Cherian; Saroja Bharati

doi:10.1055/s-0035-1566296

American Journal of Perinatology Reports, Table of Contents

AJP Rep 2016; 06(01): e91-e95
DOI: 10.1055/s-0035-1566296

Case Report

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Prenatal Diagnosis of Uhl Anomaly with Autopsy Correlation

Saji Philip

¹Fetal Cardiac Services, Division of Pediatric Cardiology, St. Gregorios Cardio-Vascular Centre, Parumala, Kerala, India

,

Sarasa Bharati

²Division of Cardiac Pathology, Frontier Mediville, Frontier Life Line Hospital, Chennai, India

,

Kottureth Mammen Cherian

³Dr. K M. Cherian Heart Foundation, Frontier Life Line hospital, Chennai, India

,

Saroja Bharati

⁴Department of Pathology, Rush University Medical Center, Chicago, University of Illinois at Chicago, Rosalind Franklin University of Sciences, North Chicago, Illinois

⁵The Advocate Children's Hospital, Oak Lawn, Illinois

› Author Affiliations

Abstract

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.

Keywords

dilated right ventricle - fetal autopsy - right ventricular dysplasia - Uhl anomaly

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References

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