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Left ventricular hypertrabeculation/noncompaction with and without neuromuscular disorders.

Claudia Stöllberger1, Maria Winkler-Dworak, Gerhard Blazek

  • 12nd Medical Department, Krankenanstalt Rudolfstiftung, Steingasse 31/18, A-1030 Wien, Osterreich, Austria. claudia.stoellberger@chello.at

International Journal of Cardiology
|September 1, 2004
PubMed
Summary

Left ventricular hypertrabeculation/noncompaction (LVHT) is linked to neuromuscular disorders (NMD). This study found few clinical differences in LVHT patients with or without NMD, suggesting cardiac findings are similar.

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Area of Science:

  • Cardiology
  • Neurology
  • Genetics

Background:

  • Left ventricular hypertrabeculation/noncompaction (LVHT) is often associated with neuromuscular disorders (NMD).
  • The clinical presentation of LVHT patients with and without NMD has not been well-defined.
  • Investigating differences can improve patient management and understanding of these conditions.

Purpose of the Study:

  • To determine if clinical, electrocardiographic (ECG), or echocardiographic findings differ between LVHT patients with and without NMD.
  • To compare patient characteristics and cardiac parameters based on neurological status.
  • To identify potential distinguishing features for LVHT associated with NMD.

Main Methods:

  • Retrospective analysis of 77 LVHT patients diagnosed between 1995 and 2003.

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  • All patients underwent cardiologic examination.
  • 59 patients were additionally investigated neurologically to assess for NMD.

    • Main Results:

    • Neurologically normal LVHT patients reported more anginal chest pain compared to those with definite NMD or NMD of unknown etiology.
    • Patients without NMD were more often in NYHA class 0 or I.
    • LVHT patients without NMD had a significantly thinner interventricular septum than those with NMD.

    Conclusions:

    • Cardiac abnormalities show minimal differences between LVHT patients with and without NMD.
    • Clinical presentation, particularly regarding chest pain and functional class, may vary.
    • Further research is needed to fully elucidate the relationship between LVHT and NMD subtypes.