p.97 抽出テキスト

Proteinuria Renal impairment

Kidney

CV

Dyspnoea

Peripheral oedema

AF/flutter

HFpEF or unexplained right HF Hypotension or syncope

Nerves

Orthostatic hypotension Peripheral polyneuropathy Polyneuropathy

GI

Constipation /diarrhoea Macroglossia Malabsorption/weight loss/nausea

Bruising Skin

Laboratorya

ECGb

TTEc

Characteristic echo findingse

ECHO score ≥8d

CMRf

Non-invasive diagnosis of AL-CA

Clinical features Investigations

Figure 43 Non-invasive diagnosis of amyloid light-chain cardiac amyloidosis. a′, late diastolic velocity of mitral annulus obtained by tissue Doppler imaging; AF, atrial fibrillation; AL-CA, amyloid light-chain cardiac amyloidosis; CMR, cardiac magnetic resonance; CV, cardiovascular; E, mitral inflow early diastolic velocity obtained by pulsed wave; e′, early diastolic velocity of mitral annulus obtained by tissue doppler imaging; ECG, electrocardiogram; echo, echocardiography; ECV, extracellular volume fraction; GI, gastrointestinal; GLS, global longitudinal strain; HF, heart failure; HFpEF, heart failure with preserved ejection fraction; IVS, interventricular septum; LGE, late gadolinium enhancement; LV, left ventricular; LVEDD, left ventricular end diastolic diameter; NT-proBNP, N-terminal pro-B-type natriuretic peptide; PW, left ventricular posterior wall; s′, systolic velocity of tricuspid annulus obtained by Doppler tissue imaging; SPEP, serum protein electrophoresis; TAPSE, tricuspid annular plane systolic excursion; TTE, transthoracic echocardiography; UPEP, urine protein electrophoresis. Individually, the clinical manifestations and findings on cardiac testing for AL-CA are non-specific. Integration of all clinical and diagnostic findings is necessary when assessing the likelihood of the diagnosis. aDisproportionately high NT-proBNP; persisting elevated troponin levels; abnormal free light-chain levels (AL-CA); positive SPEP and/or UPEP (AL-CA). bDisproportionally low QRS voltage; early conduction system disease; pseudo-infarct pattern. cUnexplained LV thickness ≥12 mm + 1 or 2 characteristic echo findings or ECHO score ≥8; idiopathic pericardial effusion. dECHO score: relative LV wall thickness (IVS + PW/LVEDD) . 0.6 (3 points), Doppler E/e′ . 11 (1 point); TAPSE ≤19 mm (2 points); GLS ≥−13% (1 point); systolic longitudinal strain apex to base ratio.2.9 (3 points). eCharacteristic echocardiography findings: grade ≥2 diastolic dysfunction; reduced s′, e′, and a′ velocities (,5 cm/s); decreased GLS to ≥−15%. fDiffuse subendocardial or transmural LGE; elevated native T1 values; abnormal gadolinium kinetics (myocardial nulling preceding or coinciding with the blood pool); ECV ≥0.40% (strongly supportive). Downloaded from academic.oup.com/eurheartj/article/43/41/4229/6673995 by guest on 07 October 2026