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............................................................................................................................................................... DCM and HCM phenotypes. Quite commonly individuals with features of LVNC are found in families where other affected relatives have typical HCM or DCM. Therefore, LVNC is not treated as a separate disease entity, but as a separate rare presentation of a genetic susceptibility to either HCM or DCM.939

14.4 Atrial disease

14.4.1 Definition Atrial disease, also termed atrial failure or myopathy, can be defined as a complex of subclinical structural, electrophysiological, and functional changes that affect the atria with the potential to produce clinical consequences.606,940,941 It has been suggested that atrial disease links the pathophysiology of HF, especially HFpEF, with AF, as they often coexist, are closely inter-related and share common risk factors.606,685,942,943

14.4.2 Diagnosis Atrial size and function can be evaluated by multimodality imaging including two- and three-dimensional echocardiography, myocardial deformation, computed tomography (CT) and CMR.944 Cardiac biomarkers, including high-sensitivity cardiac troponins and NPs, may assess pathophysiological aspects of atrial disease.945947 The increased levels of NPs in AF may also be an indicator of an underlying atrial disease.942,948 A comprehensive characterization of atrial disease combining clinical, imaging, biochemical and molecular features is, however, still lacking.

14.4.3 Management Atrial disease is an emerging therapeutic target in the prevention of AF, systemic thromboembolism, and perhaps HFpEF.949 As atrial disease appears to result from the intersection of shared risk factors and comorbidities predisposing to both AF and HF, diabetes mellitus, hypertension, obesity, smoking, and physical inactivity may be of paramount importance for its development.7,950 Effective management of HF and AF (see section 12.1.1), as well as treatment of mitral regurgitation (see section 12.3.3), may also be important to counteract atrial disease progression.

14.5 Myocarditis

14.5.1 Epidemiology and diagnosis The incidence of acute myocarditis is estimated to be 1.5 million cases per year globally.951 The contribution of myocarditis as a cause of HF varies by age and region from approximately 0.5% to 4.0%.918,952 Chronic, EMB proven, inflammation can be found in 9% to 30% of adult patients with a DCM.918,953 The most frequent potential aetiologies triggering acute myocarditis in Europe are reported in Table 30. The clinical presentation of acute myocarditis may vary from mild symptoms to cardiogenic shock. Workup for the diagnosis of acute myocarditis in patients with HF is reported in Table 31 and Figure 20. Specific criteria about biopsies and CMR are reported in Tables 32 and 33.

14.5.2 Treatment Hospitalization for at least 48 h may be useful for patients with acute myocarditis and HF, especially when troponins are elevated and when cardiac dysfunction, and/or arrhythmias are present at initial presentation. Despite the lack of evidence in the specific setting of acute myocarditis, treatment of HFrEF is recommended in the presence of systolic LV dysfunction. Immunosuppression is only indicated in selected cases of acute myocarditis (Table 34). Once cardiac enzymes decrease, arrhythmias are absent, and cardiac systolic dysfunction is stabilized, standard HF therapy should be continued for at least 6 months (see also Figure 20). Immunosuppression has been considered for treatment of patients with chronic cardiac inflammation at EMB and no evidence of active viral infection.918,919 This was associated with an improvement in cardiac function in small studies and with better outcomes in a retrospective observational study.953,963,964 Prospective trials with old or newer immunosuppressive/immunomodulatory drugs are needed. A placebo-controlled trial testing the effects of immunoadsorption with i.v. immunoglobulins on LV function is ongoing and other treatment options are being tested.919

14.6 Amyloidosis

14.6.1 Epidemiology and diagnosis CA or amyloid cardiomyopathy is still an underdiagnosed cause of HF.895,965,966 The two most prevalent forms of CA are light chain immunoglobulin (AL) and transthyretin (ATTR) amyloidosis. ATTR includes the wild-type (>90% of cases), and the hereditary or variant type (<10% of cases). It is estimated that 6% to 16% of all patients

Table 30 Aetiologies to be considered triggering acute myocarditis917

Infectious Viral Parvovirus B19, human herpes virus-6, Epstein- Barr virus, enteroviruses, (coxsackievirus,

adenovirus), CMV, HIV, SARS-CoV-2

Others Borrelia, Coxiella burnetii (Q-fever) Systemic disease Auto-immune and others Sarcoidosis, giant cell myocarditis, eosinophilic

myocarditis, SLE, ANCA-positive vasculitis, rheumatoid arthritis, any other auto-immune disease

Toxic Medications Immune check point inhibitors, anthracyclines,

clozapine, adrenergic drugs, 5-fluorouracil

Other agents Alcohol, amphetamines, cocaine ANCA = antineutrophil cytoplasmic antibody; CMV = cytomegalovirus; HIV = human immunodeficiency virus; SARS-CoV-2 = severe acute respiratory syndrome coronavirus 2; SLE = systemic lupus erythematosus. ESC 2021 Downloaded from academic.oup.com/eurheartj/article/42/36/3599/6358045 by guest on 07 October 2026