Concentric hypertrophic cardiomyopathy

Last revised by Joachim Feger on 17 Dec 2021

Concentric or symmetric hypertrophic cardiomyopathy is a morphological variant or phenotype of hypertrophic cardiomyopathy (HCM) characterized by fairly symmetrical or diffuse thickening of the myocardium and a reduction of the left ventricular cavity.

The term ‘concentric left ventricular hypertrophy’ might be more appropriate in the context of a known or possible secondary cause as hypertensive heart disease, aortic stenosis athletes heart syndrome or infiltrative disease etc.

Statements on the frequency of concentric hypertrophic cardiomyopathy are quite variable among studies and range from about 5-10% 1-4 to 42% 5-7. This variability might be a result of including secondary causes of left ventricular hypertrophy as cardiac amyloidosis, Fabry disease or sarcoidosis etc.

Concentric hypertrophic cardiomyopathy might be also associated with left ventricular outflow obstruction 5.

In concentric hypertrophic cardiomyopathy, myocardial wall thickness is increased in a fairly symmetrical and circumferential fashion and the left ventricular cavity is decreased.

A wall thickness of ≥15 mm in adults or a z-score of ≥2 in children is considered diagnostic 1.

Microscopically hypertrophic cardiomyopathy is characterized by the following features 4-6:

  • cardiomyocyte hypertrophy: transverse diameter exceeding 40µm
  • cardiomyocyte fiber disarray: disordered myocyte bundles/contractile elements within sarcomeres
  • interstitial fibrosis or replacement fibrosis
  • bizarre enlarged nuclei with nuclear hyperchromasia and pleomorphism

Concentric hypertrophic cardiomyopathy is characterized by a circumferential fairly symmetrically increased myocardial wall thickness (≥15 mm) without significant differences between the walls or segments 5.

Echocardiography can visualize symmetric hypertrophy and can provide additional information on cardiac function including cardiac strain. Besides echocardiography allows assessment of diastolic dysfunction and detect abnormal filling patterns 5.

Cardiac MRI can demonstrate symmetric left ventricular hypertrophy and can assess cardiac function including cardiac volumes and cardiac strain. It can provide additional prognostic information by demonstrating myocardial fibrosis and detect potential secondary causes of left ventricular hypertrophy 1,5-7.

The radiological report should include a description of the following 5:

Clinical conditions that also might present with concentric left ventricular hypertrophy include the following 1-7:

In addition concentric, it should be distinguished from other hypertrophic phenotypes including:

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