(Circulation. 2000;101:e230.)
© 2000 American Heart Association, Inc.
Circulation Electronic Pages |
MRI of Uhl’s Anomaly
From the Division of Cardiology (I.A.) and Departments of Diagnostic Imaging (M.-L.G., C.M.) and Critical Care Medicine (I.A.), Toronto Hospital for Sick Children and University of Toronto, Canada.
Correspondence to Ian Adatia, MBChB, FRCP(C), MRCP(UK), Department of Critical Care Medicine, Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada. E-mail ian.adatia{at}sickkids.on.ca
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Introduction |
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 Top Introduction References |
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Uhl’s anomaly of the right ventricle is an unusual cardiac disorder with almost complete absence of right ventricular myocardium, normal tricuspid valve, and preserved septal and left ventricular myocardium.1
We report MRI of the heart from a 17-year-old boy with Uhl’s anomaly. He presented as a neonate with severe hypoxemia (arterial PO2 of 29 mm Hg) and functional pulmonary atresia. He was treated with prostaglandins until the pulmonary vascular resistance decreased and forward flow across the pulmonary valve was established. His cyanosis resolved with closure of the foramen ovale and ductus arteriosus. His follow-up examinations have shown persistent right ventricular dilation and restrictive right ventricular physiology but normal left ventricular function. Despite right atrial enlargement, he remains free of arrhythmias.
The MR images depict an extremely thin-walled right ventricle with
almost complete absence of right ventricular free wall
myocardium (Figure 1A
), with
a paucity of apical trabeculations (Figures 1A and 3A) with normal left ventricular myocardium
(Figures 1A, 2A, and 3A). There is an absence of fibrofatty
infiltration of the right ventricular free wall (Figure 2A), which may serve to differentiate
Uhl’s disease from arrhythmogenic right ventricular
dysplasia (Figure 2B).2 The tricuspid valve hinges
normally, is not dysplastic, and serves to exclude Ebstein’s anomaly
of the tricuspid valve as the cause of a dilated and thin-walled right
ventricle (Figure 3A). The right atrium
is dilated and hypertrophied as a consequence of the right
ventricular restrictive
cardiomyopathy3 and dependence on
atrial contraction to augment pulmonary artery forward flow
(Figure 3A).
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). B, Right ventricle in
normal 17-year-old man for comparison.
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Selective but unrestrained apoptosis of right ventricular myocytes after complete cardiac development has been postulated to explain Uhl’s anomaly.4 5
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Footnotes |
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The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
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References |
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TopIntroductionReferences |
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1. Uhl HSM. A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp. 1952;91:197–205.[Medline] [Order article via Infotrieve]
2.
Gerlis L, Schmidt-Ott SC, Ho S, et al. Dysplastic
conditions of the right ventricular myocardium:
Uhl’s anomaly v arrhythmogenic right
ventricular dysplasia. Br Heart J. 1993;69:142–150.
3. Tumbarello R, Adatia I, Yetman A, et al. From functional pulmonary atresia to right ventricular restriction: long term follow up of Uhl’s anomaly. Int J Cardiol. 1998;67:161–164.[Medline] [Order article via Infotrieve]
4.
Uhl HSM. Uhl’s anomaly revisited.
Circulation. 1996;93:1483–1484.
5.
James T, Nichols M, Sapire D, et al. Complete heart
block and fatal right ventricular failure in an infant.
Circulation. 1996;93:1588–1600.
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