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9.4 Amyloid light-chain cardiac

amyloidosis Amyloid light-chain amyloidosis is a plasma cell dyscrasia, which is typically treated with therapies very similar to those used in MM, including PI-based therapy.792 It can occur in conjunction with myeloma or independently as a light-chain proteinproducing disorder. Amyloid light-chain amyloidosis is a systemic disease 793,794 and it is critical to have a high degree of suspicion for the diagnosis of cardiac involvement (amyloid light-chain cardiac amyloidosis [AL-CA]) because a combination of specialized tests is needed to make an accurate diagnosis (Figure 43).290,793,795,796

Valve replacement or repair surgery is recommended in symptomatic patients with severe carcinoid mitral or aortic VHD and an expected survival ≥12 months.783,785 I C © ESC 2022

MDT, multidisciplinary team; NP, natriuretic peptides; RV, right ventricular; VHD, valvular heart disease. aClass of recommendation. bLevel of evidence. cIncluding saline contrast infusion at baseline to rule out patent foramen ovale. dWith controlled serotonin concentrations.

Flushing

Bronchoconstriction

Diarrhoea

Ascites

Peripheral oedema

Pleural effusion Carcinoid heart disease: clinical featuresand diagnostic tests

Clinical features Diagnostic and pronostic tools

TTE

Circulating serotonin

NP

Urinary 5HIAA

CMR

Figure 42 Carcinoid heart disease: clinical features and diagnostic tests. 5HIAA, 5-hydroxyindoleacetic acid; CMR, cardiac magnetic resonance; NP, natriuretic peptides; TTE, transthoracic echocardiography. Downloaded from academic.oup.com/eurheartj/article/43/41/4229/6673995 by guest on 07 October 2026