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increasingly used as a bridge to transplant. Long-term MCS should also be considered as destination therapy for cardiomyopathy patients with advanced heart failure despite optimal medical therapy who are not eligible for transplantation.69

6.10.3 Management of atrial arrhythmias

Atrial fibrillation is the most common arrhythmia in all subtypes of cardiomyopathies and is associated with an increased risk of cardio-embolic events, heart failure, and death.331–333 Data from 3208 consecutive adult patients in the EURObservational Research Programme (EORP) Cardiomyopathy Registry showed an AF prevalence of 28.2% at baseline and 31.1% during followup,331–333 although it differed among cardiomyopathy types (see Table 15). Overall, annual incidence in this registry was 3.0%.332,333 In patients with cardiomyopathies, the presence of AF is associated with more severe symptoms, an increased prevalence of cardiovascular risk factors and comorbidities, and an increased incidence of stroke and death (from any cause and from heart failure).332,334–336 Both the 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation and the 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure recommend an integrated and structured approach to facilitate guideline-adherent management. The Atrial Fibrillation Better Care (ABC) approach has been shown to reduce the risk of stroke and systemic embolism, myocardial infarction, and mortality in the general population.337–361 Although this approach has not been specifically assessed in patients with cardiomyopathies, heart failure was present in ∼20% of the individuals of these studies and, where specified, cardiomyopathy in ∼5.5–6.5%. In particular, two RCTs support integrated care.347,361 The RACE 3, combining the components of the ABC pathway into structured care, resulted in reduced AF burden and better rhythm control among 245 patients with early persistent AF and stable heart failure (119 randomized to targeted and 126 to conventional therapy).347 The mobile Atrial Fibrillation App Trial (mAFA-II), which included 714 patients with heart failure (21.5%), 54 with HCM (1.6%), and 105 with DCM (3.2%), showed the superiority of integrated care supported by mobile technology in the composite outcome of ‘ischaemic stroke/systemic thrombo-embolism, death, and re-hospitalization’ (1.9% vs. 6.0%; hazard ratio [HR] 0.39; 95% confidence interval [CI], 0.22 to 0.67; P < 0.001) and re-hospitalization rates (1.2% vs. 4.5%; HR 0.32; 95% CI, 0.17 to 0.60; P < 0.001).361 Adherence to the mobile health technology beyond 1 year was good, and was associated with a reduction in adverse clinical outcomes.362

6.10.3.1 Anticoagulation

Thrombo-embolic risk varies in different cardiomyopathy phenotypes (see Section 7).332,363–367 Cardiac amyloidosis, HCM, and RCM368 are associated with a particularly increased risk of stroke.332,365,369,370 The EORP registry indicated a worse prognosis for the population with cardiomyopathy and concurrent AF with an annual incidence of stroke/transient ischaemic attack (TIA) about three times higher in the cardiomyopathy group with AF.332,334 Hence, considering anticoagulation is key in patients with any type of AF or atrial flutter. Importantly, patients with cardiomyopathy and AF have more cardio-embolic risk factors, including greater age, more advanced NYHA class and more frequent history of stroke/TIA, hypertension, and diabetes mellitus, among others.332,333 The CHA2DS2-VASc (congestive heart failure or left ventricular dysfunction, hypertension, age ≥75 [doubled], diabetes, stroke [doubled]-vascular disease, age 65– 74, sex category [female]) score has not been specifically tested in patients with cardiomyopathies,369 and retrospective evidence suggests that it may perform suboptimally with respect to stroke prediction in HCM and ATTR amyloidosis.334,365,371–374 For this reason, although there are no RCTs evaluating the role of anticoagulation among patients with HCM, given the high incidence of stroke, prophylactic anticoagulation is recommended in all patients with HCM and AF.334,371,372,374 A similar recommendation is given in patients with AF and RCM or cardiac amyloidosis.375 In patients with DCM, NDLVC, or ARVC and AF, chronic oral anticoagulation should be considered on an individual basis, taking into consideration the CHA2DS2-VASc score, as proposed by the 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation.336 Atrial fibrillation is a rare finding in children with genetic cardiomyopathies and no data are available regarding the performance of CHA2DS2-VASc or any other risk stratification score, nor the risk and benefit of prescribing oral anticoagulation. There are no data on long-term prophylactic anticoagulation in children with DCM in sinus rhythm.

In the general population, direct-acting oral anticoagulants (DOACs) are preferred for the prevention of thrombo-embolic events in patients with AF and without severe mitral stenosis and/or mechanical valve prosthesis, as they have similar efficacy to vitamin K antagonists (VKAs) but a lower risk of intracranial haemorrhage.376 There are no randomized data comparing direct oral anticoagulants with VKAs in patients with cardiomyopathy, although data suggest that they may be used in a similar manner as the general population.373,374,377–380

6.10.3.2 Rate control

Rate control should be considered in any patient with cardiomyopathy presenting with AF.336 A strict rate control (resting heart rate <80 beats per minute [b.p.m.] and heart rate during moderate exercise <110 b.p.m.) did not show any benefit over lenient rate control (resting heart rate <110 b.p.m.) in RACE II381 and a pooled analysis of RACE II and AFFIRM.382 However, only 8–12% of patients had a history of cardiomyopathy (type unspecified) in the RACE II trial, and only 10% of the patients in RACE II and 17% of those in the pooled analysis had a history of heart failure hospitalization or NYHA class II or III, respectively.381,382 No data are available for the different cardiomyopathy subtypes, but observational studies suggest that higher heart rates are associated with worse outcomes in patients with heart failure.383,384 Accordingly, the 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure consider lenient rate control to be acceptable as an initial approach but to target a lower heart rate in case of persistent symptoms or suspicion of associated tachycardia-induced cardiac dysfunction.69 Very little data are available regarding the choice of pharmacological treatment for rate control in patients with cardiomyopathies. Beta-blockers are the preferred choice in patients with cardiomyopathies given their long-established safety in the presence of LV dysfunction.385,386 Digoxin is an alternative, particularly in patients with contraindication or intolerance to beta-blockers and among patients with AF and heart failure symptoms (RATE-AF trial), having shown no difference in quality of life (QoL) at 6 months compared with bisoprolol.387 When administering digoxin, close monitoring of plasma drug

ESC Guidelines                                                                                                                                                                                          3541

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