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(Circulation. 2007;115:e11-e12.)
© 2007 American Heart Association, Inc.

Images in Cardiovascular Medicine

Electrocardiogram, Echocardiography, and Magnetic Resonance Imaging Characteristics in Uhl’s Disease

Akli Otmani, MD; Laurent Leborgne, MD; Cédric Renard, MD; Houssam Bakkour, MD; Serge Quenum, MD; Christophe Tribouilloy, MD; Jean-Luc Rey, MD

From the Cardio Vascular Center, University of Picardie, Amiens, France.

Correspondence to Dr Akli Otmani, CHU Amiens SUD, Service de Cardiologie A, 80054 Amiens Cedex 01, France. E-mail otmani.akli{at}chu-amiens.fr

Uhl’s disease is a very rare affection that was originally described in 1952¹; few cases have been reported in the literature. It is caused by isolated right ventricular enlargement and failure with partial or total absence of right ventricular myocardium. It was first considered to be a congenital development failure in the human embryo in its early stages. However, more recent publications incriminate apoptotic anomalies for right ventricular muscle agenesy.^2–3 Surgical correction has been attempted, without success.⁴ Cardiac transplantation may be proposed when patients become refractory to medical treatment.

We report the case of a 22-year-old woman without any medical history who was referred to us for dyspnea and syncope. She did not complain of palpitation and had no manifestation of right heart failure. Twelve-lead ECG (Figure 1) showed right atrial hypertrophy and an exaggerated, and not anteriorly described, fragmented ventricular depolarization, which corresponded to very slow conduction in the right ventricle. Chest x-ray revealed enlargement of the cardiac silhouette with a prominent right-ventricle contour (Figure 2). Neither supraventricular nor ventricular arrhythmia was observed during long-term ambulatory ECG monitoring. Transthoracic (Movie I) and transesophageal echocardiography (Movie II) depicted a huge hypokinetic right ventricle with severe tricuspid regurgitation. There was no right obstacle and no argument for Ebstein disease. The right atrium was also dilated as a consequence of the right cardiomyopathy and severe tricuspid regurgitation.

View larger version (32K): [in this window] [in a new window]   Figure 1. Twelve-lead ECG (A) and V2 lead (B) showing right atrial hypertrophy and fragmented QRS. Although the left ventricle is activated normally, right ventricular depolarization is delayed and prolonged, with the right ventricle being the last to be activated. Total QRS duration is 180 ms.

View larger version (154K): [in this window] [in a new window]   Figure 2. Chest x-ray showing enlargement of the cardiac silhouette with a prominent right-ventricle contour (arrow).

Transverse T1-weighted conventional spin-echo sequences (Figure 3) showed an extremely dilated thin-wall right ventricle and absence of trabeculation. The right atrium was dilated and hypertrophied. There was no fat signal in the right ventricular wall as seen in arrhythmogenic ventricular dysplasia. The septal and left ventricular myocardium were preserved. Cine magnetic resonance imaging (Figure 4, Movie III) demonstrated an absence of right ventricular wall motion.

View larger version (145K): [in this window] [in a new window]   Figure 3. Transverse T1-weighted spin-echo sequences showing very dilated thin-wall right ventricle and no trabeculation. The right atrium is dilated and hypertrophied. The left ventricle is normal.

View larger version (85K): [in this window] [in a new window]   Figure 4. Cine magnetic resonance imaging showing no right ventricular wall motion in short-axis sequences.

The patient was treated with ß-blocker and diuretics and is free of symptoms at 2 years. In this case, the ECG showed right ventricular electrical activity, indicating persistent myocardial tissue.

	Disclosures

Top Disclosures References

 
None.

	Footnotes

 
The online-only Data Supplement, which contains 3 movies, can be found at http://circ.ahajournals.org/cgi/content/full/115/2/e11/DC1.

	References

Top Disclosures 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.[Medline] [Order article via Infotrieve]

2. Uhl HSM. Uhl’s anomaly revisited. Circulation. 1996; 93: 1483–1484.[Free Full Text]

3. 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.[Free Full Text]

4. Abe T, Kuribayashi R, Sato M, Nioch S, Abe S. Congenital hypoplasia of the right ventricular myocardium (Uhl’s anomaly): a case report and review of the literature. J Cardiovasc Surg. 1973; 14: 431–437.[Medline] [Order article via Infotrieve]

This Article Extract Full Text (PDF) Data Supplement 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 Otmani, A. Articles by Rey, J.-L. Search for Related Content PubMed PubMed Citation Articles by Otmani, A. Articles by Rey, J.-L. Related Collections Other heart failure Myocardial cardiomyopathy disease Electrocardiology Echocardiography