Cardiac amyloidosis causing cardiac dysfunction: Analysis of 54 necropsy patients

Clinical and morphologic findings are described in 54 necropsy patients (32 men [59%]) aged 21 to 97 years (mean 64) with cardiac amyloid deposits extensive enough to cause fatal cardiac dysfunction. Chronic congestive heart failure (CHF) was present in 46 (85%). The duration of CHF, known in 39 patients, ranged from 1 to 108 months (mean 18) and lasted ≤ 12 months in 25 patients (64%). All 8 patients without CHF died suddenly and unexpectedly. Systemic arterial pressures were recorded in the last 3 months of life in 43 patients: the peak indirect systolic pressure was ≤ 130 mm Hg and the diastolic pressure < 90 mm Hg in all. Electrocardiograms, recorded in the last 6 months of life in 40 patients, were abnormal in each: low voltage in 35 (63%); “myocardial infarction pattern” in 33 (83%); abnormal QRS axis in 29 (73%); arrhythmias in 29 (73% ); first, second, or third degree heart block in 28 (45%); and complete bundle branch block in 7 (18%). In 30 patients, the QRS amplitude in all 12 leads was measured: in the 15 men it ranged from 60 to 197 mm (mean 99) (10 mm = 1 mV) and in the 15 women from 58 to 199 mm (mean 109). Diagnosis of amyloidosis was established by biopsy of noncardiac organs or tissues during life in only 18 (33%) patients. During life the condition simulated hypertrophic cardiomyopathy in 5 patients, constrictive pericardial disease in 3, and coronary heart disease (because of angina pectoris) in 4.At necropsy, the hearts ranged in weight from 300 to 900 g (mean 554), and all but 1 had a “rubbery,” noncompliant consistency. In addition to their presence in myocardial interstitium (53 patients) and in intramural coronary arteries (54 patients), amyloid deposits were present grossly in mural endocardium in all 54 patients and in valvular endocardium in 46 (85% ). The cardiac ventricles were not dilated in 43 patients (80%), but both atria were dilated in all 54 patients. Intracardiac thrombi were present in 14 patients (26% ). Cardiac amyloidosis must be considered in any elderly patient with chronic CHF unassociated with chest pain when blood pressure is normal and the electrocardiogram discloses low voltage and a pattern of “healed myocardial infarction.”