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Left ventriculoplasty for dilated cardiomyopathy in Fukuyama-type muscular dystrophy

Masataka Yoda1, Hiroaki Tanabe, Ichizo Nishino

  • 1Department of Cardiovascular Surgery, The Cardiovascular Institute, Tokyo, Japan. myoda@cvi.or.jp

European Journal of Cardio-Thoracic Surgery : Official Journal of the European Association for Cardio-Thoracic Surgery
|February 1, 2011
PubMed

Insights

This case report details a rare instance of Fukuyama-type muscular dystrophy with dilated cardiomyopathy. Surgical intervention improved cardiac function, offering new hope for managing this complex condition.

Area of Science:

  • Cardiology
  • Neuromuscular Disorders
  • Genetics

Background:

  • Dilated cardiomyopathy (DCM) is a severe heart condition often linked to neuromuscular diseases.
  • Fukuyama-type muscular dystrophy (FCMD) is a rare genetic disorder with known cardiac implications, though its association with DCM is infrequently reported.

Observation:

  • A 29-year-old male patient presented with heart failure and diagnosed DCM.
  • Elevated creatine kinase (hyperCKemia) and calf pseudohypertrophy were noted.
  • Muscle biopsy confirmed Fukuyama-type muscular dystrophy.

Findings:

  • The patient underwent partial left venticulectomy, papillary muscle approximation, and mitral and tricuspid valve annuloplasty for DCM.
  • One-year follow-up showed no progression of neuromuscular symptoms.
  • Significant improvement in left ventricular function was observed, with LVDd decreasing from 77-66 mm, LVDs from 73-59 mm, and EF increasing from 26-30%.

Implications:

  • This report presents the first case of left ventriculoplasty in a patient with Fukuyama-type muscular dystrophy.
  • The successful surgical outcome suggests potential therapeutic benefits of cardiac intervention in FCMD patients with DCM.
  • Further research is warranted to explore surgical management strategies for cardiomyopathy in rare neuromuscular disorders.

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