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Case Reports
. 2020 Jun 15;11(6):4147-4150.
doi: 10.19102/icrm.2020.110601. eCollection 2020 Jun.

A Different Breed of Wolf: First Known Case of Wolf-Hirschorn Syndrome with Third-degree Atrioventricular Block Requiring Pacemaker Implantation

Robert Lee Percell Jr  1 , Hyacinth C Percell  1 , Keith A Miller  1
Affiliations
Case Reports

A Different Breed of Wolf: First Known Case of Wolf-Hirschorn Syndrome with Third-degree Atrioventricular Block Requiring Pacemaker Implantation

Robert Lee Percell Jr et al. J Innov Card Rhythm Manag. .

Abstract

We describe a case of a 44-year-old male with a history of Wolf-Hirschorn syndrome (WHS) with seizures and mental retardation who was evaluated for what was thought to be a seizure. He was found to be severely bradycardic with a heart rate of 24 bpm. The electrocardiogram revealed third-degree atrioventricular block and he subsequently underwent an uncomplicated single-chamber pacemaker implantation procedure. This is a unique report given its status as the first described case of bradycardic rhythm abnormalities in a patient with WHS.

Keywords: Arrhythmia; Wolf–Hirschorn syndrome; heart block.

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Conflict of interest statement

Dr. Percell reports that he is on speaker bureaus for Abbott Medical, Biosense Webster, Janssen, Pfizer, and Boehringer Ingelheim and is the founder of the SANS FLUORO Association, outside the scope of the submitted work.

Figures

Figure 1:
Figure 1:
Initial electrocardiogram revealing third-degree AV block with an atrial rate of 75 bpm and a junctional escape rate of 30 bpm with a normal axis.
Figure 2:
Figure 2:
Electrocardiogram showing ventricular pacing with a rate of 60 bpm.
Figure 3:
Figure 3:
Chest radiograph revealing the patient’s single-chamber pacemaker with the ventricular lead placed in the septum.
Figure 4:
Figure 4:
Transesophageal echocardiogram revealing a 3.2-cm × 2.4-cm mass attached to the RV lead, extending to the RV free wall.
See this image and copyright information in PMC

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