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{{SI}}
{{SI}}
{{CMG}}; '''Associate Editor-in-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]
{{CMG}}; '''Associate Editor-in-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]
{{SK}} parchment right ventricle. See also: [[arrhythmogenic right ventricular dysplasia]]


==Overview==
==Overview==
'''Uhl anomaly''' is a very rare [[congenital heart disease]] (less than 100 cases 1900–1993) with a partial or total loss of the myocardial muscle in the [[right ventricle]].  
Uhl anomaly is a very rare [[congenital heart disease]] with a partial or total loss of the myocardial muscle in the [[right ventricle]].


==Etymology==
==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==
Less than 100 cases have been reported between 1900–1993.
==Historical Perspective==
'''Uhl anomaly''' was first described in 1952.<ref>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. </ref>  
'''Uhl anomaly''' was first described in 1952.<ref>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. </ref>  


==Diagnosis==
==Diagnosis==


==== Fetal echocardiographic findings ====
=== Fetal echocardiographic findings ===


Three findings are  enlarged [[right ventricle|right ventricular]] cavity without apical trabeculation with a thin hypokinetic ventricular wall.<ref>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</ref>
Three findings are  enlarged [[right ventricle|right ventricular]] cavity without apical trabeculation with a thin hypokinetic ventricular wall.<ref>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</ref>
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{{Congenital malformations and deformations of circulatory system}}
{{Congenital malformations and deformations of circulatory system}}


[[Category:Congenital heart disease]]
[[Category:Congenital heart disease]]

Latest revision as of 20:12, 22 September 2012

Uhl anomaly
OMIM 107970
DiseasesDB 33469

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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

Uhl anomaly is a very rare congenital heart disease with a partial or total loss of the myocardial muscle in the right ventricle.

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

Less than 100 cases have been reported between 1900–1993.

Historical Perspective

Uhl anomaly was first described in 1952.[1]

Diagnosis

Fetal echocardiographic findings

Three findings are enlarged right ventricular cavity without apical trabeculation with a thin hypokinetic ventricular wall.[2]

Sources

References

  1. 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.
  2. 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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