Hypertrophic Cardiomyopathy 2020

Kogut, James; Popjes, Eric

doi:10.1007/s11886-020-01381-3

Cited by 11 publications

(56 citation statements)

References 64 publications

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“…63 Its phenotypic expression ranges from mild symptoms and almost standard lifelength expectancy up to sudden cardiac death (SCD) in seemingly healthy young people. [64][65][66] Hypertrophic cardiomyopathy is characterized by LV muscle hypertrophy which is not secondary to increased afterload (i.e., with no identifiable cause). Histopathological findings are myocytes hypertrophy, disarray and fibrosis.…”

Section: Hypertrophic Cardiomyopathymentioning

confidence: 99%

“…The first symptom may be SCD in young adults and adolescents. 64,67,68 In patients with symptomatic hypertrophic obstructive cardiomyopathy (HOCM), hitherto pharmacotherapy, based on β-blockers or nondihydropyridine calcium channel blockers with or without disopyramide, is often inadequate, poorly tolerated and not disease-specific. 67,68 Mavacamten is a cardiac myosin inhibitor.…”

Section: Hypertrophic Cardiomyopathymentioning

confidence: 99%

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Phenotyping in heart failure with preserved ejection fraction: A key to find effective treatment

2022

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Heart failure with preserved ejection fraction (HFpEF) is an increasingly widespread medical condition, with excessive morbidity and mortality. Recently, for the first time in HFpEF, a reduction in the primary composite outcome of cardiovascular death or HF hospitalization was shown with empagliflozin. The failure of previous clinical trials in HFpEF might have resulted from suboptimal patient selection and inclusion of patients without "true" or clinically significant HFpEF. Another important factor might be the heterogeneity of HFpEF, and thus there is a growing interest in HFpEF phenotyping. This phenotyping can be based on clinical presentation (e.g., subtypes with predominant atrial fibrillation, obesity, pulmonary hypertension and right ventricular failure, coronary artery disease (CAD), or noncardiac comorbidities), but also on HFpEF etiology. Specific therapies, such as tafamidis in transthyretin-related amyloidosis (ATTR) or mavacamten in hypertrophic cardiomyopathy, have demonstrated their efficacy. However, pathomechanisms leading to the development of different phenotypes of HFpEF seem more complex and subtle. Machine learning and neural network models might help identify specific subgroups within the HFpEF population that either cluster patients with similar genetic, biochemical, echocardiographic or clinical characteristics, or respond similarly to a given treatment. Herein, we review different approaches to HFpEF phenotyping and present some distinct HFpEF subtypes.

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Section: Hypertrophic Cardiomyopathymentioning

confidence: 99%

Section: Hypertrophic Cardiomyopathymentioning

confidence: 99%

Phenotyping in heart failure with preserved ejection fraction: A key to find effective treatment

2022

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“…1 HCM is defined as the presence of a markedly hypertrophied, nondilated left ventricle (LV) in the absence of other processes known to cause hypertrophy of the myocardium. 1 Often, this hypertrophy is asymmetric and most commonly involves the interventricular septum. 1 When examined histologically, myocyte hypertrophy and disarray are observed, along with interstitial fibrosis and microvascular dysfunction.…”

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confidence: 99%

“…1 Often, this hypertrophy is asymmetric and most commonly involves the interventricular septum. 1 When examined histologically, myocyte hypertrophy and disarray are observed, along with interstitial fibrosis and microvascular dysfunction. 1 Genetically, it is often associated with a variation in sarcomere proteins that is expressed in an autosomal dominant fashion with variable penetrance.…”

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confidence: 99%

“…2 The majority of patients with nonobstructive HCM remain asymptomatic or mildly symptomatic. 1 Additionally, patients with nonobstructive HCM are at low risk for progressive heart failure [New York Heart Association (NYHA) functional classes III/IV] at 1.6% per year as opposed to 3.2% per year in patients with latent obstruction and 7.4% per year in patients with obstruction at rest. 1 HCM-related mortality rates are about 0.5% per year, with a 99% 5-year survival rate and a 97% 10-year survival rate overall.…”

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confidence: 99%

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Mavacamten: A Novel Disease-Specific Treatment for Hypertrophic Cardiomyopathy

Capilupi

Frishman

2022

Cardiology in Review

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Hypertrophic cardiomyopathy (HCM) is characterized by an abnormal thickening of the myocardium, leading to left ventricular outflow tract obstruction. Current treatments for HCM include non–disease-specific medications such as beta blockers or invasive interventions. Mavacamten has been studied for its effects on adenosine triphosphatase, myocardial-specific sarcomeric proteins, and myocardial tissue calcium sensitivity. Given these properties, mavacamten could be used as a disease-specific treatment for HCM. Clinical trials of mavacamten have shown improvements in left ventricular outflow tract obstruction among other favorable improvements in biochemical markers and the clinical symptoms of the disease. While trials to date have been relatively small, mavacamten shows promise as a future disease-specific treatment for HCM.

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