Author + information
- Received September 8, 2015
- Revision received October 7, 2015
- Accepted October 15, 2015
- Published online June 1, 2016.
- aCardiovascular Division, Electrophysiology Section, Temple University Hospital Philadelphia, Pennsylvania
- bDepartment of Cardiac Electrophysiology, San Antonio Military Medical Center, San Antonio, Texas
- cHeart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
- ↵∗Reprint requests and correspondence:
Dr. Joshua M. Cooper, Temple University Hospital, 3401 N. Broad Street, 9th Floor Parkinson Pavilion, Philadelphia, Pennsylvania 19140.
- congenital heart disease
- epsilon waves
- implantable cardioverter defibrillator (ICD)
- right ventricle
- Uhl's anomaly
Uhl’s anomaly is a rare myocardial condition first described by Henry Uhl in 1952 (1). Also known as “parchment heart,” it is characterized by partial or complete absence of the right ventricular (RV) myocardium, with severe RV systolic and diastolic impairment. Patients with Uhl’s anomaly, if they survive to adulthood, often present with right heart failure or arrhythmias (2).
A 30-year-old woman, who received a diagnosis of Uhl’s anomaly at age 7 years, was referred for implantable cardioverter-defibrillator (ICD) implantation. An echocardiogram showed massive RV dilation, and a Holter monitor showed frequent ventricular ectopy and asymptomatic nonsustained ventricular tachycardia. She was managed for years with mexiletine and beta-blockade. A 12-lead electrocardiogram showed prominent fractionated epsilon waves in all QRS complexes. Cardiac magnetic resonance imaging showed severe dilation of the right ventricle and right atrium, with absence of the RV myocardial layer. The RV ejection fraction was 15% and the left ventricular (LV) ejection fraction was 55%. The RV end-diastolic volume was 670 ml, more than 7-fold greater than the LV end-diastolic volume of 87 ml.
A single-chamber ICD was implanted using a passive fixation ICD lead. A dual-coil lead was selected in anticipation of the abnormal leftward location of the RV coil, with a desire to incorporate a right-sided component to the shock vector. Lead positioning was challenging (Figure 1, Online Videos 1, 2, 3, and 4). R waves were 10.2 mV, and the pacing threshold was 0.6 V at 0.5 ms. Defibrillation threshold testing was not performed because of concern for post-shock pulseless electrical activity, which has been reported in Uhl’s anomaly.
For supplemental videos and their legends, please see the online version of this article.
Dr. Cooper is a consultant for St. Jude Medical, Medtronic, Boston Scientific, Biotronik, and Biosense Webster. Dr. Deyell is a consultant for Medtronic Canada. Dr. Gerasimon has reported that he has no relationships relevant to the contents of this paper to disclose.
- Received September 8, 2015.
- Revision received October 7, 2015.
- Accepted October 15, 2015.
- American College of Cardiology Foundation