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heart failure therapy, which often is not well tolerated, apart from diuretics (see Section 6.10.2).1072,1073 The natural history of cardiac amyloidosis associates electrical conduction disease of the heart with symptomatic bradycardia and advanced AV block.375,1074,1075 The clinical threshold for pacemaker indication should be low, as the disease progresses and implantation of the device allows rate response to exercise and medication adjustment.375,1074 The role of ICD in cardiac amyloidosis for SCD prevention is not clearly known, but available data do not support their use in primary prevention.1076,1077 7.7.4.1 Specific therapies Therapy for AL cardiac amyloidosis is based on treatment of the underlying haematological problem with chemotherapy or autologous stemcell transplant.1064 Transthyretin stabilization and reduction of its production are the basis of TTR cardiac amyloidosis treatment. Tafamidis reduced all-cause mortality and cardiovascular hospitalizations in ATTR, with the largest effect achieved in patients at NYHA functional class I and II.1078 Additional studies are being conducted with other stabilizing agents and other molecules that reduce TTR production.1078a 8 Other recommendations 8.1 Sports 8.1.1. Cardiovascular benefits of exercise Regular physical activity and systematic exercise confer several cardiovascular, psychological, and QoL benefits. Through curbing risk factors for atherosclerosis, such as obesity and insulin resistance,1079 hypertension,1080 and hyperlipidaemia,1081 regular physical activity is associated with an up to 50% risk reduction in an adverse event from CAD in middle-aged and older individuals.1082,1083 Individuals who exercise regularly live 5–7 years longer than their sedentary counterparts,1084 Scintigraphy grade Haematologic tests AL/ATTR cardiac amyloidosis unlikely If suspicion persists consider repeat CMR followed by biopsy Scintigraphy grade Haematologic tests Grade Cardiac ATTR amyloidosis TTR genetic testing ATTRwt/ATTRv Grade Histological confirmation (cardiac/ extracardiac) to diagnose Scintigraphy grade Haematologic tests Histological confirmation (usually cardiac) to subtype Scintigraphy grade Haematologic tests AL amyloidosis? CMR Amyloidosis unlikely Histological confirmation (cardiac/ extracardiac) to diagnose CMR or inconclusive Signs and symptoms, ECG, ECHO, or CMR suggestive of cardiac amyloidosis 99mTc-DPD/PYP/HMDP scintigraphy with SPECT Haematologic tests (serum free light chain quantification and serum and urine immunofixation) 0 - 1–3 - 0 + 1–3 + I 2–3 - + Figure 22 Diagnosis of cardiac amyloidosis. AL, amyloid light chain; ATTR, transthyretin amyloidosis; ATTRv, variant transthyretin amyloidosis; ATTRwt, wild-type transthyretin amyloidosis; CMR, cardiac magnetic resonance; DPD, 3,3-diphosphono-1,2-propanodicarboxylic acid; ECG, electrocardiogram; ECHO, echocardiogram; HMDP, hydroxymethylene diphosphonate; PYP, pyrophosphate; TTR, transthyretin. ESC Guidelines 3581 Downloaded from academic.oup.com/eurheartj/article/44/37/3503/7246608 by guest on 07 October 2026