Clinicopathological Features of Cardiac Amyloidosis

  Objective  To explore the clinical features of cardiac amyloidosis (CA).

  Methods  The clinical data of 17 CA patients who were diagnosed in our hospital from September 2006 to April 2010 were retrospectively analyzed.

  Results  Of these 17 patients, 14 (82.4%) were over 40 years old, and the ratio of male to female was 1:1. Sixteen patients (94%) had two or more systems involved, and 9 patients (52.9%) had three or more systems involved. Patients were manifested as fatigue (41%) and/or skin petechia/ecchymosis. In the circulation system, seven patients (41.2%) showed a trend of hypotension, and echocardiography showed 14 patients (82.4%) had thickened ventricular walls(14.6±2.27) mmand interventricular septal thickness/left ventricular wall thickness >1.1 mm. Fourteen patients (82.4%) had impaired diastolic function of ventriculus sinister. Thirteen patients (76.5%) had increased echogenicity of the myocardium with a granular appearance. Fourteen patients (82.4%) had abnormal electrocardiogram findings. Eight patients (47%) had gastrointestinal systems, which included glossal hypertrophy (11.7%) and enlargement of the liver (17.6%). Seven patients (41.2%) showed renal insufficiency. Immunofixation electrophoresis of blood and urine detected monoclonal light chain in 52.9% of patients (λ light chain, n=8; κ light chain, n=1). One patient (5.9%) experienced nervous symptom.

  Conclusion  CA can affect multiple systems, with heart, gastrointestinal system, and kidney as the most commonly involved target organs.