Uhl anomaly

	WikiDoc Resources for Uhl anomaly
Articles
Most recent articles on Uhl anomaly Most cited articles on Uhl anomaly Review articles on Uhl anomaly Articles on Uhl anomaly in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Uhl anomaly Images of Uhl anomaly Photos of Uhl anomaly Podcasts & MP3s on Uhl anomaly Videos on Uhl anomaly
Evidence Based Medicine
Cochrane Collaboration on Uhl anomaly Bandolier on Uhl anomaly TRIP on Uhl anomaly
Clinical Trials
Ongoing Trials on Uhl anomaly at Clinical Trials.gov Trial results on Uhl anomaly Clinical Trials on Uhl anomaly at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Uhl anomaly NICE Guidance on Uhl anomaly NHS PRODIGY Guidance FDA on Uhl anomaly CDC on Uhl anomaly
Books
Books on Uhl anomaly
News
Uhl anomaly in the news Be alerted to news on Uhl anomaly News trends on Uhl anomaly
Commentary
Blogs on Uhl anomaly
Definitions
Definitions of Uhl anomaly
Patient Resources / Community
Patient resources on Uhl anomaly Discussion groups on Uhl anomaly Patient Handouts on Uhl anomaly Directions to Hospitals Treating Uhl anomaly Risk calculators and risk factors for Uhl anomaly
Healthcare Provider Resources
Symptoms of Uhl anomaly Causes & Risk Factors for Uhl anomaly Diagnostic studies for Uhl anomaly Treatment of Uhl anomaly
Continuing Medical Education (CME)
CME Programs on Uhl anomaly
International
Uhl anomaly en Espanol Uhl anomaly en Francais
Business
Uhl anomaly in the Marketplace Patents on Uhl anomaly
Experimental / Informatics
List of terms related to Uhl anomaly

	Uhl anomaly;
OMIM	107970
DiseasesDB	33469

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

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]

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)