Fact-sheet: Left ventricular noncompaction


Updated on 09/30/2019 at 6:12 PM

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Definition

Persistence of prominent ventricular trabeculations separated by deep recesses

- rare congenital condition
- genetically heterogeneous: familial or sporadic forms, 7 genes identified, autosomal dominant inheritance most likely
- first described in 1984
- classified as a genetic cardiomyopathy caused by an abnormality of endocardial and myocardial embryogenesis (between the 4th and 10th week of gestation) in utero
- may be isolated or associated with other cardiac abnormalities
- male > female

- Poorly known prevalence, 1/100,000

Clinical features

Incidental discovery (echocardiography)
Heart failure
Arrhythmia
Thromboembolic event

Ultrasound

Jenni criteria (2001)
• Absence of coexisting cardiac abnormality.
• Two-layer myocardial structure: a compacted epicardial layer and a non-compacted endocardial layer.
Non-compacted/compacted myocardium ratio > 2 in end-systole
• Predominant segmental location
• Color Doppler evidence of deeply perfused intertrabecular recesses

Stollberg criteria (2002):

  • > 3 trabeculations
  • Beyond the papillary muscles
  • Intertrabecular recesses
  • Apical view

MRI

Petersen criterion (2005):
- Numerous excessive trabeculations in the LV with predominant involvement of the apical and mid segments of the lateral and inferior walls
- Thinning of the LV wall during diastole
- Presence of a two-layer myocardial structure with a non-compacted to compacted myocardial ratio > 2.3

Jacquier criteria (2010):

  • Multiple trabeculations
  • Trabeculated mass > 20% of total LV mass
  • Measurement in diastole

Ventricular thrombi

Management

Genetic counseling

Symptomatic treatment if presence of:

  • Heart failure
  • Arrhythmia
  • Anticoagulation to be discussed given the increased thromboembolic risk

Differential diagnosis

Difficult diagnosis: distinguishing between physiological trabeculations and congenital or acquired pathological trabeculations:

- Elite athlete

- Pregnant woman

- African American

- Secondary causes: hypertension, valvular disease, Ischemic heart disease, DCM, HCM, aortic stenosis