Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology

Abstract

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. In DCM, HF with reduced ejection fraction (HFrEF) has high incidence and prevalence and represents the most frequent cause of death, despite improvements in treatment. In addition, advanced HF in DCM is one of the leading indications for heart transplantation. In HCM, HF with preserved ejection (HFpEF) affects most patients with obstructive, and ∼10% of patients with non-obstructive HCM. A timely treatment is important, since development of advanced HF, although rare in HCM, portends a poor prognosis. In RCM, HFpEF is common, while HFrEF occurs later and more frequently in amyloidosis or iron overload/haemochromatosis. Irrespective of RCM aetiology, HF is a harbinger of a poor outcome. Recent advances in our understanding of the mechanisms underlying the development of HF in cardiomyopathies have significant implications for therapeutic decision-making. In addition, new aetiology-specific treatment options (e.g. enzyme replacement therapy, transthyretin stabilizers, immunoadsorption, immunotherapy, etc.) have shown a potential to improve outcomes. Still, causative therapies of many cardiomyopathies are lacking, highlighting the need for the development of effective strategies to prevent and treat HF in cardiomyopathies.

Expression of the Novel Cardiac Biomarkers sST2, GDF-15, suPAR, and H-FABP in HFpEF Patients Compared to ICM, DCM, and Controls

“…Moreover, ventricular hypertrophy primarily triggered by arterial hypertension might add to this shortage [4]. Nevertheless, former studies have also shown a considerable prevalence of storage diseases such as amyloidosis and Morbus Fabry resulting in HFpEF [24]. Additionally, also an impairment in coronary microcirculation by means of coronary microvascular endothelial inflammation increasing resting tension through a reduction in nitric oxide bioavailability, cyclic guanosine monophosphate content and protein kinase G (PKG) activity found in HFpEF patients contributes to a shortage in myocardial oxygen supply [25].…”

Hypertrophic cardiomyopathy: the future of treatment

“…Patients with symptomatic oHCM are generally offered first‐line pharmacotherapy with β‐blockers or the non‐dihydropyridine calcium‐channel blockers . Disopyramide is effective as an add‐on therapy, although it can be poorly tolerated.…”

March 2020 at a glance: heart failure with preserved ejection fraction, left atrial myopathy, atrial fibrillation and cardiac amyloidosis

“…The two most common types of cardiac amyloidosis are transthyretin (TTR)-related and light-chain amyloidosis (AL). 22 Milandri et al 23 evaluated the prevalence and impact of carpal tunnel syndrome (CTS) in patients with TTR and AL amyloidosis, compared to the general population. CTS was most common in male, elderly patients with TTR amyloidosis and cardiac involvement.…”