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

Abstract

Aim This executive summary of the hypertrophic cardiomyopathy clinical practice guideline provides recommendations and algorithms for clinicians to diagnose and manage hypertrophic cardiomyopathy in adult and pediatric patients as well as supporting documentation to encourage their use. Methods A comprehensive literature search was conducted from January 1, 2010, to April 30, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Collaboration, Agency for Healthcare Research and Quality reports, and other relevant databases. Structure Many recommendations from the earlier hypertrophic cardiomyopathy guidelines have been updated with new evidence or a better understanding of earlier evidence. This summary operationalizes the recommendations from the full guideline and presents a combination of diagnostic work-up, genetic and family screening, risk stratification approaches, lifestyle modifications, surgical and catheter interventions, and medications that constitute components of guideline directed medical therapy. For both guideline-directed medical therapy and other recommended drug treatment regimens, the reader is advised to follow dosing, contraindications and drug-drug interactions based on product insert materials.

Pulmonary Hypertension in Patients With Heart Failure With Mid-Range Ejection Fraction

“…Still, we think that the use of these potentially effective drugs should be considered if there PH, i.e., a manifestation of advanced HF. In addition, specific mechanisms of LA pressure and mPAWP elevation must be targeted, e.g., tachycardia, atrial fibrillation, myocardial ischemia, infiltrative diseases, and functional MR. Several mechanism may contribute to LA pressure elevation (Figure 1), and careful non-invasive and invasive diagnostic evaluation is prerequisite for a tailored therapy (Table 2) (27,(65)(66)(67)(68)(69)(70)(71). The role of AF seems to be particularly important.…”

Cardiomyopathy Etiologies, Symptoms and Management

“…Syncope not due to vasovagal or cardiogenic causes is an increased risk for SCD, especially when occurring in young patients. Predictors of SCD includes experiencing at least one syncopal episode include a family history of SCD from HCM, massive LVH, unexplained syncope, LV apical aneurysm, HCM with LV systolic dysfunction [13,14].…”

Assesment of Markers of Endothelial Dysfunction and Myocardial Stress in Patients with Hypertrophic Cardyomyopathy

“…Эндотелиальная дисфункция и миокардиальный стресс при ГКМП Введение Гипертрофическая кардиомиопатия (ГКМП) представляет собой генетически гетерогенное заболевание, характеризующееся ассиметричной гипертрофией миокарда левого желудочка, беспорядочным расположением кардиомиоцитов и мышечных волокон, а также миокардиальным фиброзом. За последние 20 лет большинство исследований демонстрируют, что распространенность ГКМП составляет 1:500 в общей популяции и может варьировать в разных возрастных когортах от 1:500 до 1:200 [1,2]. Однако из-за мощного развития инновационных и более точных методов диагностики сердечно-сосудистых заболеваний может оказаться, что частота встречаемости ГКМП в общей популяции значительно выше, чем предполагалось ранее [3].…”

“…А.А.Остроумова (Сеченовский Университет), которые были разделены на две группы по вариантам течения: пациенты с симптомным стабильным течением ГКМП (группа 1; n=14) или с прогрессирующим течением симптомной ГКМП (группа 2; n=34). Диагноз ГКМП устанавливался согласно клиническим рекомендациям по диагностике и лечению больных ГКМП [1,2].…”