Prognosis of left ventricular hypertrabeculation/noncompaction is dependent on cardiac and neuromuscular comorbidity

Claudia Stöllberger1, Maria Winkler-Dworak, Gerhard Blazek

  • 12nd Medical Department, Krankenanstalt Rudolfstiftung, Juchgasse 25, Wien, Austria. claudia.stoellberger@chello.at <claudia.stoellberger@chello.at>

Insights

Left ventricular hypertrabeculation/noncompaction (LVHT) is linked to neuromuscular disorders (NMD). Prognosis depends on age, cardiac issues like heart failure, and NMD presence, impacting long-term survival.

Area of Science:

  • Cardiology
  • Neurology
  • Genetics

Background:

  • Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality often associated with neuromuscular disorders (NMD).
  • Long-term prognosis data for LVHT patients remain controversial.
  • Assessing long-term outcomes in LVHT is crucial for patient management.

Purpose of the Study:

  • To evaluate the long-term prognosis of patients diagnosed with LVHT.
  • To identify predictors of mortality in LVHT patients.
  • To assess the impact of cardiac and neuromuscular comorbidities on LVHT prognosis.

Main Methods:

  • Echocardiographic diagnosis of LVHT in 86 adult patients.
  • Baseline cardiologic and neurologic investigations were performed.
  • Mean follow-up of 51 months to assess mortality and comorbidities.

Main Results:

  • The overall mortality rate was 5.3% per year.
  • Predictors of increased mortality included advanced age, presence of NMD, exertional dyspnea, edema, and heart failure.
  • Left ventricular systolic dysfunction (fractional shortening <25%) was also a significant predictor.

Conclusions:

  • Mortality in adult LVHT patients is significantly influenced by cardiac and neurologic comorbidities.
  • Key predictors for mortality are increased age, co-existing neuromuscular disorders, heart failure, and left ventricular systolic dysfunction.
Abstract

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