Uhl anomaly: Difference between revisions

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	Uhl anomaly;
OMIM	107970
DiseasesDB	33469

VisualWikitext

Revision as of 20:11, 22 September 2012

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 alos: arrhytmogenic 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

Template:WikiDoc Sources

[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

[1]

[2]

@@ Line 17: / Line 17: @@
 {{SK}} parchment right ventricle
+See alos: [[arrhytmogenic right ventricular dysplasia]].
 ==Overview==

v t e Congenital malformations and deformations of circulatory system (Q20-Q28, 745-747)
Cardiac chambers and connections	Persistent truncus arteriosus - Double outlet right ventricle (Taussig-Bing syndrome) - Transposition of the great vessels (dextro, levo)
Cardiac septa	Ventricular septal defect - Atrial septal defect (Lutembacher's syndrome) - Atrioventricular septal defect (Ostium primum) - Tetralogy of Fallot - Eisenmenger's syndrome
Right: pulmonary and tricuspid valves	pulmonary valves (stenosis, insufficiency) - tricuspid valves (stenosis, atresia) - Ebstein's anomaly
Left: aortic and mitral valves	aortic valves (stenosis, insufficiency, bicuspid) - mitral valves (stenosis, regurgitation) - Hypoplastic left heart syndrome
Other congenital malformations of heart	Dextrocardia - Levocardia - Cor triatriatum
Great arteries	aorta (Patent ductus arteriosus, Aortic coarctation, Interrupted aortic arch, Overriding aorta, Aneurysm of sinus of Valsalva, Vascular ring) - Pulmonary atresia
Great veins	Persistent left superior vena cava - Total anomalous pulmonary venous connection - Scimitar syndrome
Other	Arteriovenous malformation (Cerebral arteriovenous malformation)
See also non-congenital conditions (I, 390-459)