After 60 Years Hypertrophic Cardiomyopathy is Finally Recognized as a Contemporary Treatable Disease With Low Mortality and Morbidity, But is This Paradigm Under-Recognized in the Literature?

Maron, Barry J.; Rowin, Ethan J.; Maron, Martin S.

doi:10.1016/j.amjcard.2020.11.030

Cited by 2 publications

(2 citation statements)

References 15 publications

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“…Perhaps even more important, it has become increasingly evident that HC patients suffer from substantial morbidity and mortality related to HF and stroke, for which no specific risk prediction tool is available. 13,14 The mainstay of risk prediction in HC have been the conventional clinical SCD risk factors based on history taking, Holter monitoring and echocardiography.…”

Section: Discussionmentioning

confidence: 99%

Usefulness of High-Sensitivity Cardiac Troponin T to Predict Long-Term Outcome in Patients with Hypertrophic Cardiomyopathy

Gommans

Cramer

Fouraux

et al. 2021

The American Journal of Cardiology

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Section: Discussionmentioning

confidence: 99%

Usefulness of High-Sensitivity Cardiac Troponin T to Predict Long-Term Outcome in Patients with Hypertrophic Cardiomyopathy

Gommans

Cramer

Fouraux

et al. 2021

The American Journal of Cardiology

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“…About half of HCM patients develop LVOTO at some point during their life, and oHCM is often associated with heart failure symptoms (dyspnea, exercise intolerance), syncope, and angina [ 1 , 2 ]. After its first description, the clinical and preclinical research has shifted the perception of HCM from a rare malignant condition to a relatively common disease with an often stable course and favorable outcome, with a low mortality and morbidity when compared with other inherited heart conditions [ 10 ]. Although several HCM patients may show a normal life expectancy, LVOTO, atrial fibrillation, or ventricular arrhythmias may significantly affect the outcome of individual patients [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

Pharmacological Management of Hypertrophic Cardiomyopathy: From Bench to Bedside

Palandri

Santini

Argirò

et al. 2022

Drugs

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Hypertrophic cardiomyopathy (HCM), the most common inherited heart disease, is still orphan of a specific drug treatment. The erroneous consideration of HCM as a rare disease has hampered the design and conduct of large, randomized trials in the last 50 years, and most of the indications in the current guidelines are derived from small non-randomized studies, case series, or simply from the consensus of experts. Guideline-directed therapy of HCM includes non-selective drugs such as disopyramide, non-dihydropyridine calcium channel blockers, or β-adrenergic receptor blockers, mainly used in patients with symptomatic obstruction of the outflow tract. Following promising preclinical studies, several drugs acting on potential HCM-specific targets were tested in patients. Despite the huge efforts, none of these studies was able to change clinical practice for HCM patients, because tested drugs were proven to be scarcely effective or hardly tolerated in patients. However, novel compounds have been developed in recent years specifically for HCM, addressing myocardial hypercontractility and altered energetics in a direct manner, through allosteric inhibition of myosin. In this paper, we will critically review the use of different classes of drugs in HCM patients, starting from “old” established agents up to novel selective drugs that have been recently trialed in patients.

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After 60 Years Hypertrophic Cardiomyopathy is Finally Recognized as a Contemporary Treatable Disease With Low Mortality and Morbidity, But is This Paradigm Under-Recognized in the Literature?

Cited by 2 publications

References 15 publications

Usefulness of High-Sensitivity Cardiac Troponin T to Predict Long-Term Outcome in Patients with Hypertrophic Cardiomyopathy

Usefulness of High-Sensitivity Cardiac Troponin T to Predict Long-Term Outcome in Patients with Hypertrophic Cardiomyopathy

Pharmacological Management of Hypertrophic Cardiomyopathy: From Bench to Bedside

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