Myocardial Non-Compaction

 
   

 

Case Description:

Left ventricular non-compaction in association with a small complex VSD. This case includes both TTE and CMRI images.

Image Legend:

This 4ch CMRI view demonstrates unusually deep trabeculations in the left ventricular wall. There appears to be a complex network of trabeculae traversing the LV septum. Coronary catheterisation confirmed the presence of a small VSD.

Image Courtesy Of:

Royal Brompton and Harefield NHS Foundation Trust.

 

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Other Cases

None at present

 

Description:

A congenital cardiomyopathy in which the muscular wall of the left ventricle apears to be spongy or 'noncompacted' showing unusually prominent 'trabeculations' or channels in the muscular wall. The condition can occasionally be associated with septal defects.

Aetiology:

Considered to be a genetic cardiomyopathy. The condition results from the failure of myocardial development during embryogenesis.

Epidemiology:

As a recently discovered condition it is unknown how common this condition is. It affects both adults and children and has a genetic basis. It is therefore commoner in relatives of affected individuals.

Management:

There is a wide spectrum of severity. Some subjects do not develop significant impairment of cardiac function while others progress on to fulminant cardiac failure. Subjects with non compaction should be closely monitored for the early development of impaired systolic function. Some may develop restrictive physiology due to endocardial fibrosis.

Early medical management with standard therapies such as aspirin, B-blockers and ACE-inhibitiors is key to improving outcome. Complications of the condition, include malignant arrythmias and thromboembolic events. These should be screened for and appropriate secondary prevention measures (ICD/anticoagulation) instituted as necessary. Some individuals progress to cardiac transplantation. Screening of family members should be considered.

Imaging Tips:

Transthoracic Echocardiography:

  • Measure and record all chamber dimensions.
  • Accurately quantify left ventricular systolic and diastolic function. Appropriate measurements include Simpson's biplane ejection fraction and e/e.' Assess right ventricular longitudinal function using TAPSE.
  • Quantify the ratio of the noncompacted trabeculated left ventricular wall thickness to normally compacted wall thickness.
  • Assess carefully for evidence of septal defects in both the atria and ventricles. These can be small and difficult to spot.
  • Assess right heart pressures (TR Vmax + IVC collapse).
  • Watch out for intracardiac thrombi.
  • Assess for evidence of restrictive physiology (E/A wave, E' velocity)

Further Resources:

Wikipedia

Yale University

The Cardiomyopathy Association

 

This site is intended to provide a basic and informal introduction to imaging considerations that surround this condition. It must not be used as a guide to patient management or treatment.
 

 

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