Clinical characteristics and cardiac imaging features of patients with cardiac amyloidosis

Abstract

To summarize the clinical characteristics and cardiac imaging features by focusing the diagnostic value of MRI in patients with cardiac amyloidosis (CA). A total of 31 cases with pathologically proven CA from January 2013 to December 2014 were included in this retrospective study. Patients expressed typical disease manifestations at a late age (54±11) years. Majority patients were male (20 cases, 64.5%) in this cohort. Blood pressure was normal, 13 cases (42.9%) presented with edema in lower limbs, 12 cases (38.7%) with chest distress and dyspnea and 9 cases (29.0%) with abdominal pain and diarrhea. Electrocardiogram (ECG) features were as follows: 20 cases (64.5%) with low voltage in limb leads, 20 cases (64.5%) with poor R-wave progression in precordial leads, 17 cases (54.8%) with pseudo-necrotic Q wave and 27 cases (87.1%) with ST-T changes. Echocardiography examination showed that 25 (80.6%) of 31 cases were with left atrial enlargement, 22 cases (71%) with increased ventricular septal thickness and 12 cases (38.7%) with myocardial ground-glass opacity, 24 cases (77.4%) presented restrictive left ventricular filling pattern, 14 cases (45.2%) showed impaired left ventricular systolic function and 10 cases (32.3%) expressed abnormal left ventricular eject function (<50%). Cardiac MRI features were as follows: among 31 patients, 7 patients underwent cardiac MRI. Left ventricular and interventricular septum hypertrophy were vsulized in 6 cases, increased thickness of interatrial spetum in 3 cases, left atrial enlargement in 4 cases and right atrial enlargement in 3 cases. MRI also revealed a distinct diffuse delayed gadolinium enhancement of subendocardial and interventricular septum in 3 cases, 1 of which was with delayed enhancement of interatrial spetum. Clinically, CA diagnosis should be considered for patients with manifestations of chest distress and edema in lower limbs, ECG features of low voltage in limb leads, poor R-wave progression and pseudo-necrotic Q wave, myocardial hypertrophy with myocardial ground-glass opacity in echocardiography and a characteristic MRI pattern of diffuse subendocardial delayed gadolinium enhancement even without the pathological proof.