A Patient of Limb-girdle Muscular Dystrophy Type 1B Presenting with Heart Failure and Cardiac Conduction Defects: A Case Report

Limb-girdle muscular dystrophy

Authors

  • Abdul Wadud Chowdhury Department of Cardiology, Dhaka Medical College Hospital, Dhaka, Bangladesh
  • Nasir Uddin Department of Cardiology, Dhaka Medical College Hospital, Dhaka, Bangladesh
  • Md Khalequzzaman Department of Cardiology, Sir Salimullah Msedical College & Mitford Hospital, Dhaka, Bangladesh
  • Mahfuze Sarkar Department of Medicine, Dhaka Medical College Hospital, Dhaka, Bangladesh
  • Hashmi Sina Department of Neurology, Dhaka Medical College Hospital, Dhaka, Bangladesh
  • Abu Taher Md Mahfuzul Haque Department of Cardiology, Dhaka Medical College Hospital, Dhaka, Bangladesh
  • Azizul Haque Department of Cardiology, Dhaka Medical College Hospital, Dhaka, Bangladesh

DOI:

https://doi.org/10.3329/bmrcb.v48i1.60664

Keywords:

Muscular dystrophies, Cardiomyopathies, Lamins, Heart conduction system

Abstract

Background: Limb-girdle muscular dystrophy type 1B (LGMD1B) are characterised by cardiac dysrhythmias, late-onset cardiomyopathy, slowly progressive skeletal myopathy and contractures of the neck, elbows and ankles. The causative mutation is in lamin A/C gene (autosomal dominant LGMD1B).

Objective: To assess the clinical outcome of a patient of limb-girdle muscular dystrophy type 1B with heart failure and cardiac conduction defects.

The case: We report a case of 47-yr-old male with proximal muscle weakness who presented with repeated exertional dyspnea and repeated presyncope. ECG revealed junctional bradycardia. Echocardiography revealed global wall motion abnormality with EF 48%. EMG showed myopathic pattern. Next genome sequencing showed that the patient has a missense mutation in exon 4 region of LMNA gene (c.G662A/p.Arg221His). Cardiac involvement is very common in this disease which includes AV block, bradycardia, atrial tachycardia, atrial fibrillation and ventricular dysrhythmia causing sudden death. Our patient presented with symptomatic junctional bradycardia which necessitates implantation of permanent pacemaker.

Conclusion: Cardiologists should be aware of these unusual genetic diseases with conduction defects, especially in young adults.

Bangladesh Med Res Counc Bull 2022; 48(1): 76-80           

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Published

2022-09-05

How to Cite

Chowdhury, A. W. ., Uddin, N. ., Khalequzzaman, M., Sarkar, M. ., Sina, H. ., Mahfuzul Haque, A. T. M. ., & Haque, A. . (2022). A Patient of Limb-girdle Muscular Dystrophy Type 1B Presenting with Heart Failure and Cardiac Conduction Defects: A Case Report: Limb-girdle muscular dystrophy. Bangladesh Medical Research Council Bulletin, 48(1), 76–80. https://doi.org/10.3329/bmrcb.v48i1.60664

Issue

Vol. 48 No. 1 (2022)

Section

Case Report

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