2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary

Myocardial Deformation Analysis in MYBPC3 and MYH7 Related Sarcomeric Hypertrophic Cardiomyopathy—The Graz Hypertrophic Cardiomyopathy Registry

“…Patients carrying mutations in other sarcomere genes were omitted. Left ventricular outflow tract (LVOT) obstruction was defined as maximal left ventricular outflow tract gradient ≥ 30 mmHg either at rest or during provocations such as Valsalva maneuver or bicycle stress testing [18]. Septal reduction therapy was defined as either surgical myectomy or percutaneous transluminal septal myocardial ablation (PTSMA).…”

Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): health status analysis of a randomised, double-blind, placebo-controlled, phase 3 trial

“…A principal goal of treating symptomatic patients with oHCM is to alleviate their symptoms so as to improve their function and quality of life. 6 The recent 2020 American Heart Association/American College of Cardiology professional treatment guidelines for patients with HCM identified a clear unmet need for novel trial designs and patient-reported outcome tools to assess the impact of new therapies on meaningful endpoints, such as quality of life. Evidence supporting the health status benefits of alternative therapeutic approaches was limited and the benefits of direct myosin modulation were not available.…”

“…For example, the intended reduction in ejection fraction with a direct myosin inhibitor resulted in some patients having a transient ejection fraction below 50%, but the KCCQ benefits in these patients were still substantial. Such work is especially important given that the recent guidelines emphasize the importance of shared medical decision making 6 and it would be important to be able to explain to patients how treatment with mavacamten would be expected to improve their health status.…”

“…3,4 The primary goal of treatment for oHCM focuses on alleviating symptoms, but symptomatic improvement has not been prospectively studied with any currently recommended therapies. 5,6 Guideline-recommended pharmacological therapy is thus administered on an empirical basis and includes β-blockers or nondihydropyridine calcium channel blockers, as well as disopyramide for individuals refractory to firstline therapy. These medications were originally developed for other cardiovascular diseases but can be beneficial for some patients with oHCM, although their tolerability can be limited by side effects and they often do not provide optimal control of LV outflow tract (LVOT) gradients and symptoms.…”

Selección de lo mejor del año 2021 en cardiopatías congénitas

“…La American Heart Association publica una guía clínica de miocardiopatía hipertrófica 3 cuyo aspecto pediátrico más importante hace referencia a la estratificación del riesgo de muerte súbita (MS) y la recomendación de implante de desfibrilador automático implantable, siempre una indicación muy individualizada en pediatría. Aunque los autores remarcan la existencia de escalas pediátricas predictoras de riesgo, reconocen que aún no están validadas en la clínica.…”