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Uhl’s anomaly: a difficult prenatal diagnosis

Published online by Cambridge University Press:  01 May 2014

Laurence Vaujois ,
Nicolaas van Doesburg  and
Marie-Josée Raboisson
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: mjraboisson@gmail.com
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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.

Keywords

Uhl’s anomalyparchment-like ventricleright ventricular dilatationapoptosisfoetal echocardiography
Type
Brief Reports
Information
Cardiology in the Young , Volume 25 , Issue 3 , March 2015 , pp. 580 - 583
Copyright
© Cambridge University Press 2014 

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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: 197–205.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: 164–167.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: 621–624.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: 781–783.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: 1588–1600.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: 713–714.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: 69–76.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: 426–429.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: 215–217.Google Scholar
10. Uhl, HS. Uhl’s anomaly revisited. Circulation 1996; 93: 1483–1484.Google Scholar