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Published on: June 11, 2019

A novel clinical entity: triglyceride deposit cardiomyovasculopathy

Ken-Ichi Hirano1

  • 1Department of Cardiovascular Medicine, Graduate School of Medicine, Osaka University, Japan. khirano@imed2.osaka-u.ac.jp

Journal of Atherosclerosis and Thrombosis
|September 5, 2009
PubMed

Insights

A unique patient with severe heart failure had massive triglyceride deposits in the heart and blood vessels, despite normal blood triglyceride levels. This suggests a new condition, "Obesity of the heart," linked to genetic defects in triglyceride metabolism.

Area of Science:

  • Cardiology
  • Genetics
  • Metabolic Disorders

Background:

  • Heart diseases like atherosclerotic cardiovascular disease and congestive heart failure are leading causes of mortality worldwide.
  • Cholesterol is a known risk factor, but the role of triglycerides in heart disease pathogenesis is not fully understood.
  • A unique patient case highlights potential novel mechanisms in heart disease.

Purpose of the Study:

  • To investigate the role of triglycerides in a patient with severe heart failure and cardiac transplantation needs.
  • To characterize a novel clinical entity involving triglyceride accumulation in the heart and vasculature.
  • To explore the genetic basis and implications of triglyceride deposit cardiomyovasculopathy.

Main Methods:

  • Clinical case study of a patient with severe congestive heart failure.
  • Histopathological examination of coronary atherosclerotic lesions and myocardium for triglyceride accumulation.
  • Genetic analysis to identify mutations in key lipid metabolism enzymes.

Main Results:

  • Massive accumulation of triglycerides observed in coronary atherosclerotic lesions and myocardium.
  • Plasma triglyceride levels were normal in the patient.
  • A homozygous mutation in adipose triglyceride lipase was identified, crucial for intracellular triglyceride hydrolysis.

Conclusions:

  • The findings suggest a novel clinical entity, termed "Triglyceride deposit cardiomyovasculopathy" or "Obesity of the heart."
  • Genetic defects in triglyceride metabolism can lead to severe cardiac and vascular disease, independent of plasma triglyceride levels.
  • This case has significant implications for understanding heart disease and developing new therapeutic strategies.

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