Uhl anomaly

Uhl anomaly
Classification and external resources
ICD-9-CM	746.8 (CDC/BPA 746.882)
OMIM	107970
DiseasesDB	33469

Uhl's anomaly was first described in 1952 by Dr. Henry Uhl (1921–2009) upon examining one of his patients. ^[1] It 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.

Fetal echocardiographic findings

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

References

↑ Uhl, HS (1952). "A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle". Bulletin of the Johns Hopkins Hospital. 91 (3): 197–209. PMID 12978573.
↑ Cardaropoli, D; Russo, MG; Paladini, D; Pisacane, C; Caputo, S; Giliberti, P; Calabrò, R (2006). "Prenatal echocardiography in a case of Uhl's anomaly". Ultrasound in Obstetrics & Gynecology. 27 (6): 713–4. doi:10.1002/uog.2798. PMID 16710881.

Aortopulmonary septal defect	R→L: Double outlet right ventricle Taussig–Bing syndrome Transposition of the great vessels dextro levo Persistent truncus arteriosus Aortopulmonary window

Atrial septal defect	L→R: Sinus venosus atrial septal defect Lutembacher's syndrome

Ventricular septal defect	L→R and R→L: Eisenmenger's syndrome R→L, with other conditions: Tetralogy of Fallot

Atrioventricular septal defect	L→R: Ostium primum

Valvular heart disease/
heart chambers

Right	pulmonary valves stenosis insufficiency tricuspid valves stenosis atresia Ebstein's anomaly Hypoplastic right heart syndrome Uhl anomaly

Left	aortic valves stenosis insufficiency bicuspid mitral valves stenosis regurgitation Hypoplastic left heart syndrome

Other

This article is issued from Wikipedia - version of the 10/7/2016. The text is available under the Creative Commons Attribution/Share Alike but additional terms may apply for the media files.

Uhl anomaly

Fetal echocardiographic findings

References

Further reading