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Mechanical Control of Relaxation Using Intact Cardiac Trabeculae
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Neuromuscular implications in left ventricular hypertrabeculation/noncompaction

Josef Finsterer1, Claudia Stöllberger, Gerhard Blazek

  • 1Krankenanstalt Rudolfstiftung, Austria. duarte@aonmail.at

International Journal of Cardiology
|December 21, 2005
PubMed

Insights

Left ventricular hypertrabeculation/noncompaction (LVHT), a cardiomyopathy, is linked to neuromuscular disorders (NMD). Early cardiac screening for NMD patients and neurological evaluation for LVHT patients are crucial for timely diagnosis and management.

Area of Science:

  • Cardiology
  • Neurology
  • Genetics

Background:

  • Left ventricular hypertrabeculation/noncompaction (LVHT) is an unclassified cardiomyopathy.
  • LVHT is increasingly recognized in association with various neuromuscular disorders (NMD).

Purpose of the Study:

  • To review recent advances in understanding the association between LVHT and NMD.
  • To highlight the importance of integrated cardiological and neurological evaluations.

Main Methods:

  • Literature review of studies investigating LVHT in patients with NMD.
  • Analysis of reported cases and prevalence data.

Main Results:

  • LVHT is found in patients with dystrophinopathy, laminopathy, myotonic dystrophy, Barth syndrome, and mitochondrial disorders, among others.
  • NMD is detected in a significant proportion (up to 80%) of patients with LVHT.
  • Barth syndrome and mitochondrial disorders show the highest frequency of LVHT co-occurrence.

Conclusions:

  • Early and comprehensive cardiological assessment is recommended for all NMD patients.
  • Thorough neurological investigation is warranted for all LVHT patients.
  • Integrated diagnostic approaches are essential for managing patients with co-existing LVHT and NMD.

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