This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Greer, M.-L. Articles by Adatia, I. Search for Related Content PubMed PubMed Citation Articles by Greer, M.-L. Articles by Adatia, I. Related Collections Apoptosis Imaging CT and MRI Pediatric and congenital heart disease, including cardiovascular surgery

(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@sickkids.on.ca

	Introduction

 
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 . . . [Full Text of this Article]