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Mechanical Control of Relaxation Using Intact Cardiac Trabeculae
Published on: February 17, 2023
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.
Background And Objectives:
Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality frequently associated with neuromuscular disorders (NMD). The data about long-term prognosis of patients with LVHT are controversial. Aim of the study in a cohort of LVHT patients was to assess the long-term prognosis regarding mortality, cardiac and neuromuscular comorbidity.
Methods And Results:
In 86 patients LVHT was diagnosed echocardiographically between June 1995 and December 2004 (21 female, mean age 52 +/- 14, range 14-94 years). All patients underwent a baseline cardiologic investigation and were invited for a neurologic investigation. A specific NMD was diagnosed in 21, a NMD of unknown etiology in 33, the neurologic investigation was normal in 13 and 19 patients refused. During a mean follow-up of 51 months (range 3-106 months) the mortality rate was 5.3%/year. Predictors for an increased mortality were increased age (p=0.0134), presence of NMD (p=0.0324), exertional dyspnoea (p=0.0329), edema (p=0.0049), heart failure (p=0.0048), left anterior hemiblock (p=0.0078) and a left ventricular fractional shortening <25% (p=0.0648).
Conclusion:
The mortality of LVHT in adult patients depends on cardiac and neurologic comorbidity. Predictors for mortality are increased age, neuromuscular disorder, heart failure, left ventricular dilatation and systolic dysfunction.
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