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Case Reports
. 2020 Jan;25(1):e12681.
doi: 10.1111/anec.12681. Epub 2019 Sep 1.

Pearson marrow-pancreas syndrome with cardiac conduction abnormality necessitating prophylactic pacemaker implantation

Mats Steffi Jennifer  1 , Daniel Cortez  1
Affiliations
Case Reports

Pearson marrow-pancreas syndrome with cardiac conduction abnormality necessitating prophylactic pacemaker implantation

Mats Steffi Jennifer et al. Ann Noninvasive Electrocardiol. 2020 Jan.

Abstract

Pearson marrow-pancreas syndrome (PS), an exceedingly rare mitochondrial disorder, involves multiple systems including hematologic system and pancreas. Other mitochondrial disorders have been associated with progressive infrahisian block but this has not yet been described as a major feature of PS. We report a 7-year-old girl with classical features of PS and cardiac conduction defect. Her electrocardiogram revealed QRS prolongation with right bundle and left anterior fascicular blocks. Follow-up Holter revealed bifascicular block, alternating left and right bundle branch blocks, supraventricular tachycardia (with alternating bundles), and suspicion for nonsustained ventricular tachycardia. She underwent successful transvenous single-chamber ventricular pacemaker.

Keywords: Pearson syndrome; bundle branch blocks; fascicular block; pacemaker.

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Conflict of interest statement

The authors declare that they have no competing interests. This research received no grant from any funding agency in the public, commercial, or not‐for‐profit sectors.

Figures

Figure 1
Figure 1
12 Lead EKG showing bifascicular block
See this image and copyright information in PMC

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