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Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Updated: Jul 10, 2026

Benefits of Cardiac Resynchronization Therapy in an Asynchronous Heart Failure Model Induced by Left Bundle Branch Ablation and Rapid Pacing
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Cardiac resynchronization therapy in left ventricular hypertrabeculation/non-compaction and myopathy.

Claudia Stöllberger1, Gerhard Blazek, Elisabeth Bucher

  • 12. Medizinische Abteilung, Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030 Wien, Osterreich. claudia.stoellberger@chello.at

Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology
|November 22, 2007
PubMed
Summary

Cardiac resynchronization therapy (CRT) improved functional capacity in all patients with left ventricular hypertrabeculation/non-compaction (LVHT) and heart failure. However, systolic function improved in only half, suggesting comorbidities like myopathy impact CRT response.

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Area of Science:

  • Cardiology
  • Electrophysiology
  • Heart Failure Research

Background:

  • Left ventricular hypertrabeculation/non-compaction (LVHT) is a rare congenital cardiomyopathy.
  • The efficacy of cardiac resynchronization therapy (CRT) in LVHT patients with heart failure remains poorly understood.
  • Comorbidities, particularly neuromuscular disorders, may influence treatment outcomes in these patients.

Purpose of the Study:

  • To evaluate the response to cardiac resynchronization therapy (CRT) in patients diagnosed with left ventricular hypertrabeculation/non-compaction (LVHT) and heart failure.
  • To investigate the impact of comorbidities, such as myopathy, on CRT outcomes in this specific patient population.

Main Methods:

  • Retrospective analysis of 8/102 patients with diagnosed LVHT who underwent CRT implantation.
  • Neurological investigations were performed, with a focus on identifying myopathy.
  • Assessment of clinical improvement (NYHA class), left ventricular dimensions, and systolic function (fractional shortening) during a mean follow-up of 39 months.

Main Results:

  • All patients demonstrated improvement in New York Heart Association (NYHA) functional class by at least one class.
  • Left ventricular end-diastolic diameter decreased in most patients, with significant reductions (>30%) in two.
  • Left ventricular systolic function, measured by fractional shortening, increased substantially in half of the patients.
  • Seven out of eight patients had an identified myopathy.
  • Two patients died during the follow-up period.

Conclusions:

  • Biventricular pacing via CRT leads to functional capacity improvement in all LVHT patients with heart failure and myopathy.
  • Systolic function improvement was observed in approximately half of the patients.
  • The suboptimal response to CRT in some LVHT patients may be attributed to patient selection criteria or the presence of comorbidities, especially neuromuscular disorders.