Circulation. 2000;101:e230-e232
(Circulation. 2000;101:e230.)
© 2000 American Heart Association, Inc.
Circulation Electronic Pages |
MRI of Uhl’s Anomaly
Mary-Louise Greer, MBBS;
Cathy MacDonald, MD;
Ian Adatia, MBChB
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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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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Figure 1. A, Sagittal T1-weighted spin-echo sequence in a
17-year-old man with Uhl’s anomaly demonstrates almost total absence
of right ventricular (RV) free-wall myocardium
and decreased trabeculation ( ). B, Right ventricle in
normal 17-year-old man for comparison.
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Figure 3. A, Axial T1-weighted spin-echo sequence through
level of tricuspid valve in Uhl’s anomaly demonstrates normal hinging
of tricuspid valve (*), which is not displaced from annulus; paucity of
apical trabeculature (); and thin-walled right ventricle
(RV). There is enlargement and hypertrophy of right atrium
(RA). B, Example of similar MRI in normal 17-year-old.
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Figure 2. A, Coronal T1-weighted spin-echo sequence in same
patient shows marked dilatation and thinning of right
ventricular (RV) myocardium without fibrofatty
replacement. Normal left ventricular (LV)
myocardium is noted. B, In contrast to A, example of
arrhythmogenic right ventricular dysplasia in 17-year-old
patient demonstrates high signal intensity () in right
ventricular free wall, indicative of fibrofatty
replacement.
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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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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]
-
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.[Abstract/Free Full Text]
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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]
-
Uhl HSM. Uhl’s anomaly revisited.
Circulation. 1996;93:1483–1484.[Free Full Text]
-
James T, Nichols M, Sapire D, et al. Complete heart
block and fatal right ventricular failure in an infant.
Circulation. 1996;93:1588–1600.[Free Full Text]
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Introduction
References