Hostname: page-component-586b7cd67f-g8jcs Total loading time: 0 Render date: 2024-11-26T16:58:46.567Z Has data issue: false hasContentIssue false

Uhl’s anomaly: a difficult prenatal diagnosis

Published online by Cambridge University Press:  01 May 2014

Laurence Vaujois
Affiliation:
Department of Pediatrics, Sainte-Justine Hospital Center, Division of Pediatric Cardiology, Montréal, Québec, Canada
Nicolaas van Doesburg
Affiliation:
Department of Pediatrics, Sainte-Justine Hospital Center, Division of Pediatric Cardiology, Montréal, Québec, Canada
Marie-Josée Raboisson*
Affiliation:
Department of Pediatrics, Sainte-Justine Hospital Center, Division of Pediatric Cardiology, Montréal, Québec, Canada
*
Correspondence to: M.-J. Raboisson, MD, Division of Cardiology, CHU Sainte-Justine, Cardiologie, 3175 Chemin de la côte Sainte Catherine, Montreal, Québec, Canada, H3T 1C5. Tel: +001 514 345 4931 ext 5410; Fax: +001 514 345 4896; E-mail: [email protected]

Abstract

Uhl’s anomaly is an evolutive disease leading to terminal right ventricular failure. The most difficult differential diagnosis at presentation is the Ebstein disease. We describe the evolution of a foetus with Uhl’s anomaly from 21 to 30 weeks of gestation, with progressive reduction in the right ventricular anterior myocardium suggestive of apoptosis, leading to foetal demise.

Type
Brief Reports
Copyright
© Cambridge University Press 2014 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Uhl HSM. A previously undescribed congenital formation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp 1952; 91: 197205.Google Scholar
2. Wager, GP, Couser, RJ, Edwards, OP, Gmach, C, Bessinger, B Jr. Antenatal ultrasound findings in a case of Uhl’s anomaly. Am J Perinatol 1988; 5: 164167.Google Scholar
3. Hornung, TS, Heads, A, Wright, C, Hunter, S. Fetal diagnosis of lethal dysfunction of the right heart in three siblings. Cardiol Young 2000; 10: 621624.Google Scholar
4. Benson, CB, Brown, DL, Roberts, DJ. Uhl’s anomaly of the heart mimicking Ebstein’s anomaly in utero. J Ultrasound Med 1995; 14: 781783.CrossRefGoogle ScholarPubMed
5. James, TN, Nichols, MM, Sapire, DW, DiPatre, PL, Lopez, SM. Complete heart block and fatal right ventricular failure in an infant. Circulation 1996; 93: 15881600.Google Scholar
6. Cardaropoli, D, Russo, MG, Paladini, D, et al. Prenatal echocardiography in a case of Uhl’s anomaly. Ultrasound Obstet Gynecol 2006; 27: 713714.Google Scholar
7. Ikari, NM, Azeka, E, Aiello, VD, Atik, E, Barbero-Marcial, M, Ebaid, M. Uhl’s anomaly. Differential diagnosis and indication for cardiac transplantation in an infant. Arq Bras Cardiol 2001; 77: 6976.Google Scholar
8. Hébert, JL, Duthoit, G, Hidden-Lucet, F, et al. Images in cardiovascular medicine. Fortuitous discovery of partial Uhl anomaly in a male adult. Circulation 2010; 121: 426429.Google Scholar
9. Song, BG. A rare case of partial absence of the right ventricular musculature in asymptomatic adult man: partial Uhl’s anomaly. Heart Lung 2013; 42: 215217.Google Scholar
10. Uhl, HS. Uhl’s anomaly revisited. Circulation 1996; 93: 14831484.Google Scholar