Clinical analysis of 104 cases with cardiac amyloidosis

Objective To analyze clinical data of cardiac amyloidosis(CA) to enrich the knowledge of it. Methods Clinical manifestations, laboratory tests, image data and medical therapy were analysed retrospectively in 104 patients with CA. Results One hundred and four patients with an average age of 55.26±13.21, including 57(54.8%) males and 77(74.0%) type AL cases were enrolled. Troponin I and BNP/NT-proBNP were elevated in 52.8% and 79.8% cases, respectively. The most common symptoms were limb edema (74.1%) and shortness of breath(64.4%). Fifty three(51.0%) cases had poor heart function of NYHA Ⅲ or Ⅳ, and forty one(39.4%) were complicated by renal involvement. Positive congo red dye was found in 21 (91.3%) out of 23 patients who underwent endomyocardial biopsy(EMB) and 10 (90.9%) out of 11 patients who received renal biopsy. Electrocardiogram showed various arrhythmias in 30(28.8%) cases, low voltage of limb lead in 62(59.6%) and poor R-wave progression in 52 (50.0%). Echocardiography revealed left ventricular hypertrophy in 84 (80.8%) cases, restrictive diastolic dysfunction in 60 (57.7%) and pericardial effusion in 35 (33.7%). Ten cases underwent cardiovascular magnetic resonance(CMR), in which six cases with global, three with subendocardial and one with localized late gadolinium enhancemen(LGE). Fifty seven(74.0%) cases underwent different chemotherapy regimens with dexamethasone(80.7%), melphalan(50.9%), thalidomide(42.1%), cyclophosphamide(31.2%), bortezomib(26.3%), prednisone(22.3%), lenalidomide(8.7%), rituximab(3.4%) and chlorambucil(1.8%). Conclusions CA is frequently complicated with renal involvement, heart failure, arrhythmia and characterized by low voltage in limb leads combined with left ventricular hypertrophy. EMB and renal biopsy show the highest positive rate of amyloidosis among all the biopsy.Myocardial late gadolinium enhancement pronounced in the subendocardial layers in CA. Chemotherapy with glucocorticoid, melphalan, thalidomide, cyclophosphamide or bortezomib are the main options in the CA patients with type AL. Key words: Amyloidosis; Myocardium; Retrospective studies