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Peer-reviewedCC BY-NCEuropean heart journal. Case reports

TRPM4-related left bundle branch block-induced cardiomyopathy reversed by left bundle branch area pacing-based cardiac resynchronization therapy: a case report.

  • Sijin Wu1
  • Xinyue Wang1
  • Bin Zhou2
  • Tong Zou1
  • Xue Yu1
  1. 1Department of Cardiology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 DaHua Road, Dong Dan, Dongcheng District, Beijing 100730, China.
  2. 2Arrhythmia Center, Fuwai Shenzhen Hospital, Chinese Academy of Medical Sciences, No. 12 Langshan Road, Nanshan District, Shenzhen 518057, China.
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Abstract

Background

Progressive cardiac conduction disease (PCCD) is a rare inherited disorder characterized by degeneration of the His-Purkinje system. Although primarily considered an electrical disease, chronic left bundle branch block (LBBB) may result in mechanical dyssynchrony and reversible cardiomyopathy.Case summary

A 59-year-old man presented with progressive left ventricular (LV) dilation and systolic dysfunction. Electrocardiography showed complete LBBB with a QRS duration of 165 ms. Ischaemic and infiltrative cardiomyopathies were ruled out. Cardiac magnetic resonance imaging revealed LV enlargement without late gadolinium enhancement but with marked mechanical dyssynchrony. Genetic testing identified a likely pathogenic TRPM4 c.393G>C variant, considered contributory to the conduction phenotype. LBBB-induced cardiomyopathy and heart failure with reduced ejection fraction were diagnosed. The patient underwent left bundle branch area pacing (LBBAP)-based cardiac resynchronization therapy. The QRS duration narrowed to 115 ms, and follow-up echocardiography demonstrated marked reverse remodelling with LV ejection fraction improving from 26% to 48%.Discussion

This case suggests that TRPM4-related PCCD may present with isolated LBBB leading to dyssynchrony-mediated cardiomyopathy. LBBAP can effectively restore electrical synchrony and promote reverse LV remodelling. This favourable response highlights the importance of timely physiological resynchronization in potentially reversible LBBB-related cardiomyopathy.

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Cite

BibTeXRIS
BibTeX
@article{wu2026trpm4related,
  title = {{TRPM4-related left bundle branch block-induced cardiomyopathy reversed by left bundle branch area pacing-based cardiac resynchronization therapy: a case report.}},
  author = {Sijin Wu and Xinyue Wang and Bin Zhou and Tong Zou and Xue Yu},
  journal = {European heart journal. Case reports},
  year = {2026},
  month = sep,
  doi = {10.1093/ehjcr/ytag718},
  url = {https://europepmc.org/article/MED/42927274},
  note = {CC BY-NC},
}
RIS
TY  - JOUR
TI  - TRPM4-related left bundle branch block-induced cardiomyopathy reversed by left bundle branch area pacing-based cardiac resynchronization therapy: a case report.
AU  - Sijin Wu
AU  - Xinyue Wang
AU  - Bin Zhou
AU  - Tong Zou
AU  - Xue Yu
PY  - 2026
DA  - 2026/09/21
JO  - European heart journal. Case reports
DO  - 10.1093/ehjcr/ytag718
UR  - https://europepmc.org/article/MED/42927274
AB  - Background  Progressive cardiac conduction disease (PCCD) is a rare inherited disorder characterized by degeneration of the His-Purkinje system. Although primarily considered an electrical disease, chronic left bundle branch block (LBBB) may result in mechanical dyssynchrony and reversible cardiomyopathy.Case summary  A 59-year-old man presented with progressive left ventricular (LV) dilation and systolic dysfunction. Electrocardiography showed complete LBBB with a QRS duration of 165 ms. Ischaemic and infiltrative cardiomyopathies were ruled out. Cardiac magnetic resonance imaging revealed LV enlargement without late gadolinium enhancement but with marked mechanical dyssynchrony. Genetic testing identified a likely pathogenic TRPM4 c.393G>C variant, considered contributory to the conduction phenotype. LBBB-induced cardiomyopathy and heart failure with reduced ejection fraction were diagnosed. The patient underwent left bundle branch area pacing (LBBAP)-based cardiac resynchronization therapy. The QRS duration narrowed to 115 ms, and follow-up echocardiography demonstrated marked reverse remodelling with LV ejection fraction improving from 26% to 48%.Discussion  This case suggests that TRPM4-related PCCD may present with isolated LBBB leading to dyssynchrony-mediated cardiomyopathy. LBBAP can effectively restore electrical synchrony and promote reverse LV remodelling. This favourable response highlights the importance of timely physiological resynchronization in potentially reversible LBBB-related cardiomyopathy.
SN  - 2514-2119
ER  -