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Published on: August 8, 2022
Acquired noncompaction associated with coronary heart disease and myopathy
Josef Finsterer1, Claudia Stöllberger, Elisabeth Bonner
1Krankenanstalt Rudolfstiftung, Vienna, Austria, Europe. fipaps@yahoo.de
Insights
This case study reveals acquired left ventricular hypertrabeculation (LVHT) in a patient with neuromuscular disease. LVHT may be an adaptive response in impaired myocardium.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- A 77-year-old male with extensive cardiovascular history presented with worsening heart failure.
- Transthoracic echocardiography revealed left ventricular hypertrabeculation (LVHT), a finding absent in prior investigations.
Observation:
- The patient exhibited symptoms suggestive of a metabolic myopathy, including ptosis, madarosis, hearing loss, muscle pain, absent reflexes, and gait ataxia.
- Elevated creatine kinase levels, with normal troponin-T, further supported a myopathic process.
Findings:
- Autopsy confirmed severe coronary heart disease and LVHT in the left ventricular apex.
- The absence of LVHT in previous echocardiograms indicated an acquired condition.
Implications:
- This case suggests that LVHT can be acquired, particularly in the context of neuromuscular diseases.
- LVHT might represent an adaptive mechanism in response to myocardial impairment.
Abstract:
In a 77-year-old man with a history of arterial hypertension, coronary heart disease, dilative cardiomyopathy, mitral and tricuspid insufficiency, arteriovenous block III, implantation of a pacemaker, atrial fibrillation, and heart failure, left ventricular hypertrabeculation (LVHT) was detected on transthoracic echocardiography during hospitalization for worsening heart failure. Revision of previous echocardiography did not show LVHT in any of the previous investigations why LVHT was interpreted as acquired. The additional presentation with bilateral ptosis, madarosis (absent eyelashes), bilateral hypoacusis, sore neck muscles, absent tendon reflexes, weakness for foot extension, ataxic stance, and recurrently elevated creatine kinase with normal troponin-T suggested a metabolic myopathy. Autopsy after death resulting from intractable heart failure, 17 months later, confirmed severe coronary heart disease and LVHT in the apex. The case confirms that LVHT may be acquired in single cases with neuromuscular disease and may represent an adaptive mechanism of an impaired myocardium.
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