Prenatal Diagnosis of Uhl Anomaly with Autopsy Correlation

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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.

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Most cited references 6

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A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle.

H Uhl (1952)

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Dysplastic conditions of the right ventricular myocardium: Uhl's anomaly vs arrhythmogenic right ventricular dysplasia.

L M Gerlis, Daniel S Y Ho, C. Schmidt … (1993)

Since 1905 there have been many reports of cases in which the right ventricle was deficient in myocardium. Several terms have been used to describe this condition. Of these, "Uhl's anomaly" and "arrhythmogenic right ventricular dysplasia" are most often used. Our study investigates the relation between these entities. Five cases with a primary deficiency of the right ventricular musculature were examined. The findings were compared with those published reports to evaluate the similarities and differences between Uhl's anomaly and arrhythmogenic dysplasia. The five cases showed two patterns of myocardial deficiency in the right ventricle. On the one hand, the parietal wall was paper thin with complete absence of musculature and apposition of the endocardial and epicardial layers. On the other hand, patchy, localised fibrofatty tissue replacement was found within the parietal musculature. Evidence from our cases, combined with analysis of other publications, showed different modes and timing of clinical presentation of the patients with these two anatomical conditions, congestive heart failure or arrhythmia. The conditions variously described as Uhl's anomaly and arrhythmogenic dysplasia are separate and distinct morphological entities.

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Uhl's anomaly. Differential diagnosis and indication for cardiac transplantation in an infant

Nana Miura Ikari, Estela Azeka, Vera Demarchi Aiello … (2001)

We report the case of an 8-month-old female infant with Uhl's anomaly, who underwent successful cardiac transplantation. The clinical findings, complementary laboratory tests, anatomic findings, and differential diagnosis of the anomaly are discussed.

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Author and article information

Journal

Journal ID (nlm-ta): AJP Rep

Journal ID (iso-abbrev): AJP Rep

Journal ID (doi): 10.1055/s-00000169

Title: AJP Reports

Publisher: Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )

ISSN (Print): 2157-6998

ISSN (Electronic): 2157-7005

Publication date (Electronic): 02 November 2015

Publication date (Print): March 2016

Volume: 6

Issue: 1

Pages: e91-e95

Affiliations

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

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

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

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

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

Author notes

Address for correspondence Dr. Saji Philip, PhD, FAHA St. Gregorios Cardio-Vascular Centre, Dr. K M. Cherian Heart Foundation Parumala, Pathanamthitta District, KeralaIndia 689626 tfcsaji@ 123456yahoo.co.in tfcsaji@ 123456hotmail.com

Article

Publisher ID: 150059

DOI: 10.1055/s-0035-1566296

PMC ID: 4737623

PubMed ID: 26929879

SO-VID: d5689bb0-d5cd-4bcf-a36b-a739852a785d

History

Date received : 27 September 2015

Date accepted : 01 October 2015

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