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A Case of Prenatally Diagnosed Uhl’s Anomaly with Absent Pulmonary Valve Leaflets and Dysplastic Tricuspid Valve
- Source :
- Children, Vol 8, Iss 3, p 190 (2021)
- Publication Year :
- 2021
- Publisher :
- MDPI AG, 2021.
-
Abstract
- Uhl’s anomaly is a very rare malformation of unknown cause, characterized by complete or partial absence of the right ventricular myocardium. The cardiac malformation causes progressive right heart failure, increased right-sided cardiac pressure, massive peripheral edema, and ascites. Patients usually present in infancy and rarely survive to adulthood. Previously, diagnosis was made at post-mortem evaluation, but advances in cardiac imaging now permit diagnosis during fetal life. We report a case of Uhl’s anomaly in a newborn baby imaged at 23 + 3 weeks of gestation by fetal echocardiography. There was an aneurysmally dilated thin-walled right ventricle with hypertrophy of the right ventricular apical muscles, the tricuspid valve was dysplastic, and the pulmonary valve leaflets were absent.
- Subjects :
- Uhl’s anomaly
fetal echocardiography
pulmonary valve leaflets
Pediatrics
RJ1-570
Subjects
Details
- Language :
- English
- ISSN :
- 22279067 and 39341054
- Volume :
- 8
- Issue :
- 3
- Database :
- Directory of Open Access Journals
- Journal :
- Children
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.6ac80d95085c4ed39341054b46788eb4
- Document Type :
- article
- Full Text :
- https://doi.org/10.3390/children8030190