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Uhl's anomaly
Also known as:
Uhl's disease
Uhl's syndrome
Synonyms:
Arrhythmogenic right ventricular dysplasia, hypoplastic right ventricle, parchment heart, parchment right ventricle.
Associated persons:
Henry Stephen Magraw Uhl
Description:
Congenital hypoplasia or aplasia of the myocardium of the right ventricle, resulting in decreased output of the right side of the heart. Symptoms are dyspnoea, fatigue, chest pain and syncope on exertion, non-specific systolic murmur. It is a cause of cardiac failure and sudden death in neonates and young adults.
We thank Martin Lehn, Germany, for information submitted.
Bibliography:
- H. S. M. Uhl:
A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle.
Bulletin of the Johns Hopkins Hospital, 1952, 91: 197-205.
- H. S. M. Uhl:
Uhl's anomaly revisited. Circulation, 1996, 93: 1483-1484.
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