Uhl anomaly
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-in-Chief: Keri Shafer, M.D. [2]
Synonyms and keywords: parchment right ventricle. See also: arrhythmogenic right ventricular dysplasia
Overview
Overview
Uhl anomaly is a very rare congenital heart disease with a partial or total loss of the myocardial muscle in the right ventricle.
Pathophysiology
Pathophysiology
There is a total loss of myocardial muscle in the right ventricle. It represents a severe from of arrhythmogenic right ventricular dysplasia.
Epidemiology and Demographics
Epidemiology and Demographics
Less than 100 cases have been reported between 1900–1993.
Diagnosis
Diagnosis
Fetal echocardiographic findings
Three findings are enlarged right ventricular cavity without apical trabeculation with a thin hypokinetic ventricular wall.[2]
References
References
- ↑ Uhl HSM. A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bulletin of Johns Hopkins Hospital 1952; 91:197–205.
- ↑ D. Cardaropoli, M. G. Russo, D. Paladini, C. Pisacane, S. Caputo, P. Giliberti, R. Calabrò Prenatal echocardiography in a case of Uhl’s anomaly Ultrasound in Obstetrics and Gynecology Volume 27, Issue 6, Date: June 2006, Pages: 713-714
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