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needed with a grade 23 positivity of scintigraphy with SPECT (Figure 21).973

14.6.2 Therapy of amyloidosis and heart failure Maintenance of euvolaemia is central to management but is challenging due to the markedly reduced ventricular capacitance.977 If HF symptoms are present, a loop diuretic, possibly with an MRA, may be given, but orthostatic hypotension may cause intolerance. Beta-blockers, digitalis, ACE-I, ARBs, or ARNI may not be well tolerated because of hypotension, and their place in CA treatment is unsettled. Their withdrawal must be often considered due to hypotension and/or bradycardia.973,974 CCB should be avoided as they may cause severe hypotension and fatigue, or form complexes with amyloid.966 Amyloid infiltration of the atrial wall leads to atrial myopathy and electromechanical dissociation with high embolic risk. Patients with CA and history of AF should receive anticoagulation. There is no evidence to support anticoagulation for patients in SR, yet.7,978 Amiodarone is the preferred antiarrhythmic agent.973 Therapy of AL-CA is based on treatment of the underlying haematological problem with chemotherapy or autologous stem-cell transplant.

TTR stabilization and reduction of its production are the basis of TTR-CA treatment. Liver and/or cardiac transplantation can be considered only in end-stage disease of familial TTR-CA. Tafamidis reduced all-cause mortality and CV hospitalizations in cardiac or non-cardiac biopsy-proven hereditary and wtTTR-CA, mainly in those patients with NYHA class I and II at baseline. Functional improvement occurred within 6 months, whereas the decrease in mortality took nearly 2 years to occur.979,980 Intravenous patisiran, a small RNA interfering molecule, or subcutaneous inotersen, antisense oligonucleotide against TTR, may be considered in those patients with combined hTTRpolyneuropathy and CA (Figure 21).981,982 Off-label use of diflunisal may be considered in wtTTR-CA in combination with a proton pump inhibitor.983

14.7 Iron overload cardiomyopathy

Iron overload results either from genetically determined increased intestinal iron absorption in the context of hereditary haemochromatosis (primary iron overload) or from multiple blood transfusions required for the management of haematological conditions such as beta-thalassaemia (secondary iron overload).984 In iron overload, the iron binding capacity of transferrin is saturated and non-transferrin-bound iron enters cardiomyocytes through Ltype calcium channels, causing oxidative myocardial damage.985

Table 33 Cardiac magnetic resonance in patients with suspected myocarditis955,956

Indication Indicated at baseline, in all patients with clinical history þ ECG, elevated troponin or echocardiographic abnormalities, and significant CAD excluded or unlikely. Advised at follow-up in patients with persistent dysfunction at echocardiography, arrhythmias or ECG abnormalities.a Main findings At baseline: T1-weighted (inflammation, injury) and T2-weighted (oedema) sequences, extracellular volume and LGE within 2 weeks after symptom onset.956,960 At follow up: LGE to evaluate the degree of scarring, T1 and T2 to identify persistent inflammation.a Diagnostic significance At least one T2-based criterion (global or regional increase of myocardial T2 relaxation time or an increased signal intensity in T2-weighted images), with at least one T1-based criterion (increased myocardial T1, extracellular volume, or LGE) in the acute phase. Only one (i.e., T2-based or T1-based) marker may still support a diagnosis of acute myocardial inflammation in an appropriate clinical scenario, albeit with less specificity in the acute phase. A negative T1/T2 scan does not exclude a still ongoing inflammatory process in the chronic phase.a

CAD = coronary artery disease; CMR = cardiac magnetic resonance; ECG = electrocardiogram; LGE = late gadolinium enhancement. aIt takes at least 3 months before CMR signs of oedema (secondary to inflammation in the acute phase/baseline) disappear. At 6 months, T1 or T2 signs of oedema should have disappeared if inflammation would be completely absent. Still, the absence of T1 or T2 oedema does not exclude chronic low-grade inflammation. Table 34 Treatment and follow-up of acute myocarditis

HF therapy should be started if LV systolic dysfunction is present at presentation and should be continued for at least 6 months upon complete functional recovery (EF >50%).918,919 Immunosuppression for at least 612 months is required in acute myocarditis with clinical or EMB evidence of auto-immune disease, including giant cell myocarditis, vasculitis or sarcoidosis.98,917919,953,954,961 Immunosuppression is not advised on a routine basis in acute myocarditis without clinical or EMB-based evidence of auto-immune disease.917 Initial empirical administration of i.v. corticosteroids may be taken into consideration in cases of high suspicion of immune-mediated myocarditis especially if complicated by acute HF, malignant arrhythmias and/or high degree AV block.954,962 Intense sporting activities should be avoided as long as symptoms, cardiac enzymes elevated or ECG/imaging abnormalities are present and last for at least 6 months since complete recovery.936 Yearly follow-up for at least 4 years, with an ECG and echocardiography, is needed as acute myocarditis may lead to DCM in up to 20% of cases. AV = atrio-ventricular; DCM = dilated cardiomyopathy; ECG = electrocardiogram; EF = ejection fraction; EMB = endomyocardial biopsy; HF = heart failure; i.v. = intravenous; LV = left ventricular. ESC 2021 ESC 2021

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