Uhl anomaly: Difference between revisions
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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]] 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.<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 === | |||
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 |
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| 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
- Leon Gerlis, Uhl's anomaly, Orphanet Encyclopedia, January 2003.
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
External links