Hypertrophic Cardiomyopathy
small-vessel disease and myocardial scarring observed on DHE-MRI.29 It is also demonstrated that scarring seen on DHE-MRI is associated with arrhythmias and disease severity.30,31
Conclusions
The histopathological hallmarks of HCM are general myocyte hypertrophy, regions of myocyte disarray, different types of fibrosis and small-vessel disease. Myocardial disarray is found to a greater extent in young HCM patients and is associated with SCD in these
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20.
Factor SM, Butany J, Sole MJ, et al, Pathologic fibrosis and matrix connective tissue in the subaortic myocardium of patients with hypertrophic cardiomyopathy, J Am Coll Cardiol, 1991;17:1343–51.
21.
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30. 26. 27. 24.
young patients. There are indications that myocardial disarray is also responsible for the early development of diastolic dysfunction in HCM patients.32
Increasing amounts of fibrosis and ischaemia occur in the natural history of HCM, and the amount of fibrosis is associated with increasing septal thickness, progression to end-stage heart failure and SCD. The aetiology of the electrical instability leading to malignant arrhythmias and SCD in HCM patients is still unknown. Myocyte disarray, acute or subacute ischaemia leading to scarring and different types of fibrosis may play an important role. n
sudden cardiac death, J Am Coll Cardiol, 2000;35:36–44.
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25.
Blauwet LA, Ackerman MJ, Edwards WD, et al., Myocardial fibrosis in patients with symptomatic obstructive hypertrophic cardiomyopathy: correlation with echocardiographic measurements, sarcomeric genotypes, and pro-left ventricular hypertrophy polymorphisms involving the renin-angiotensin- aldosterone system, Cardiovasc Pathol, 2009;18:262–8.
Arteaga E, de Araujo AQ, Bernstein M, et al., Prognostic value of the collagen volume fraction in hypertrophic cardiomyopathy, Arq Bras Cardiol, 2009;92:210–20.
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28.
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Kwon DH, Smedira NG, Rodriguez ER, et al., Cardiac magnetic resonance detection of myocardial scarring in hypertrophic cardiomyopathy: correlation with histopathology and prevalence of ventricular tachycardia, J Am Coll Cardiol, 2009;54:242–9.
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