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Left ventricular hypertrabeculation/noncompaction, cardiac phenotype, and neuromuscular disorders.

C Stöllberger1,2, C Wegner3, J Finsterer4

  • 1Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Vienna, Austria. claudia.stoellberger@chello.at.

Herz
|April 8, 2018
PubMed
Summary

Patients with left ventricular hypertrabeculation/noncompaction (LVHT) and a dilated cardiac phenotype face a worse prognosis, particularly when co-occurring with neuromuscular disorders (NMDs). This highlights the critical role of cardiac phenotype and NMDs in LVHT patient outcomes.

Keywords:
Dilated cardiomyopathyHeart failureHypertrophic cardiomyopathyMortalityNeuromuscular diseases

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Area of Science:

  • Cardiology
  • Genetics
  • Neurology

Background:

  • Left ventricular hypertrabeculation/noncompaction (LVHT) prognosis and its link to neuromuscular disorders (NMDs) remain debated.
  • Understanding these associations is crucial for patient management.

Purpose of the Study:

  • To determine if LVHT patient prognosis depends on cardiac phenotype.
  • To investigate the impact of co-existing NMDs on LVHT prognosis.

Main Methods:

  • Retrospective analysis of 273 LVHT patients diagnosed between 1995-2016.
  • Cardiac phenotype classification: dilated, hypertrophic, intermediate, and normal based on echocardiographic parameters.
  • Neurological examination data analyzed for NMD presence.

Main Results:

  • Dilated phenotype was most common (46%), followed by normal (29%), intermediate (17%), and hypertrophic (8%).
  • NMDs were identified in 76% of patients who underwent neurological examination.
  • Highest mortality observed in the dilated phenotype group, with increased risk in those also having NMDs.

Conclusions:

  • LVHT patients with a dilated cardiac phenotype exhibit a poorer prognosis.
  • The presence of NMDs further worsens the prognosis for patients with the dilated LVHT phenotype.