Uhl's anomaly

Alternative eponyms

Synonyms

Arrhythmogenic right ventricular dysplasia, hypoplastic right ventricle, parchment heart, parchment right ventricle.

Related people

Congenital hypoplasia or aplasia of the myocardium of the right ventricle.

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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