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Additional iron-induced complications, such as liver disease and endocrine abnormalities, further contribute to cardiac deterioration.986,987 The end result is the development of iron overload cardiomyopathy (IOCM), which may have either a restrictive or a dilated phenotype, the former potentially evolving to the latter as the disease advances. Myocardial iron deposition can be accurately estimated by the CMRT2* technique; T2* values are correlated with left and right ventricular systolic function and predict the development of iron-induced HF or arrhythmias.984

Prevention of IOCM is successfully accomplished with iron chelators, including deferoxamine, deferiprone, and deferasirox, while established IOCM may be completely reversed by intensified and combined iron chelation therapy.985

14.8 Adult congenital heart disease

The management of ACHD has been reviewed in detail in a recent ESC guideline.988 HF is a common problem affecting 2050% of the ACHD population, and an important cause of death.989 The pathophysiology of cardiac dysfunction in ACHD is often different from non-congenital (acquired) heart disease, in particular in those with: a systemic right ventricle (RV), a failing subpulmonary ventricle, a single ventricle,988 surgery-related injury, chronic pressure/volume overload in systemic and sub-pulmonary ventricles, and those with hypertrophy or non-compaction induced by gene mutations. Therefore, extrapolation of current HF treatment guidelines to ACHD patients is not always appropriate. In addition, the few available data on HF treatments in ACHD patients are often inconclusive and are derived from small patient cohorts. As a consequence, ACHD specific recommendations are mostly based on clinical experience or position statements.990 Importantly, ACHD patients with HF should be referred to expert centres. The general principles of management, while awaiting transfer to specialist centres, is summarized in Table 36.

Table 35 “Red flags” for most common forms of cardiac amyloidosis

Type Red Flag TTR AL Extracardiac Polyneuropathy X X

Dysautonomia X X Skin bruising X Macroglossia X Deafness X Bilateral carpal tunnel syndrome X Ruptured biceps tendon X Lumbar spinal stenosis X Vitreous deposits Xa Family history Xa Renal insufficiency X Proteinuria X

Cardiac Clinical

Hypotension or normotensive if previously hypertensive X X ECG Pseudo-infarct ECG pattern Low/decreased QRS voltage to

degree of LV thickness AV conduction disease X X

X X X

X

Laboratory Disproportionally elevated NTproBNP to degree of HF Persisting elevated troponin levels X

X X

X

Echocardiography Granular sparkling of myocardium Increased right ventricular wall

thickness Increased AV valve thickness Pericardial effusion Reduced longitudinal strain with

apical sparing pattern X

X

X X X X

X

X X X

CMR Subendocardial LGE Elevated native T1 values Increased extracellular volume Abnormal gadolinium kinetics X X

X X X X

X X

AL = light chain immunoglobulin; AV = atrio-ventricular; CA = cardiac amyloidosis; CMR = cardiac magnetic resonance; ECG = electrocardiogram; HF = heart failure; LGE = late gadolinium enhancement; LV = left ventricular; NT-proBNP = Nterminal pro-B-type natriuretic peptide; QRS = Q, R and S waves (combination of three of the graphical deflections); TTR = transthyretin. Modified from973. aHereditary TTR-CA. Recommendations for the treatment of transthyretin amyloidosis-cardiac amyloidosis

Recommendations Classa Levelb

Tafamidis is recommended in patients with genetic testing proven hTTR-CA and NYHA class I or II symptoms to reduce symptoms, CV hospitalization and mortality.979 I B Tafamidis is recommended in patients with wtTTR-CA and NYHA class I or II symptoms to reduce symptoms, CV hospitalization and mortality.979 I B

CA = cardiac amyloidosis; CV = cardiovascular; hTTR = hereditary transthyretin; NYHA = New York Heart Association; wtTTR = wild-type transthyretin. aClass of recommendation. bLevel of evidence.

ESC 2021 ESC 2021

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