Disease Directory Left Ventricular Noncompaction
Cardiovascular

Left Ventricular Noncompaction

Also known as: LVNC, isolated LVNC, noncompaction cardiomyopathy

Prevalence

0.05–0.3 per 100,000 (estimated; likely underdiagnosed)

Onset

Any age; congenital to adulthood

Type

Autosomal dominant; X-linked (TAZ); de novo

Gene

MYH7, LMNA, TAZ

About Left Ventricular Noncompaction

Left Ventricular Noncompaction is a structural cardiomyopathy characterised by a heavily trabeculated left ventricular myocardium with deep intertrabecular recesses, thought to result from arrest of the normal embryonic compaction process, and associated with heart failure, ventricular arrhythmias, and thromboembolic events. The condition may occur in isolation or in association with congenital heart defects, neuromuscular diseases, or metabolic disorders, and can be inherited or sporadic. TAZ gene mutations cause Barth syndrome, a syndromic form presenting in infant males with LVNC, skeletal myopathy, neutropenia, and growth retardation.

Common Clinical Features

Heart failure with reduced or preserved ejection fraction Palpitations and ventricular arrhythmias Thromboembolic events including systemic embolism and stroke Prominent left ventricular trabeculations on echocardiography or MRI (NC:C ratio >2.3) Conduction abnormalities and bundle branch block Exercise intolerance and fatigue Sudden cardiac death risk In Barth syndrome: skeletal muscle weakness and recurrent infections

Clinical Trial Eligibility Tips

What to know before applying to Left Ventricular Noncompaction trials.

Echocardiographic or cardiac MRI confirmation of LVNC using standardised criteria (e.g., Jenni or Petersen criteria) is required for enrolment; bring recent imaging with quantified trabeculation ratios.

Genetic testing is recommended both for diagnosis and to identify co-inherited pathogenic variants (e.g., LMNA, MYH7) that influence prognosis and trial-specific eligibility.

Anticoagulation status and thromboembolic history are relevant to trial safety criteria; report all prior embolic events and current anticoagulation therapy.

Patient Resources

Patient Organization

Cardiomyopathy UK

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Natural History Registry

SHaRe Registry

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Orphanet

European reference resource for rare diseases (ORPHA:54260)

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NORD

National Organization for Rare Disorders

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Find recruiting Left Ventricular Noncompaction trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Left Ventricular Noncompaction. Updated daily.

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