CARDIAC FAILURE DUE TO PRIMARY AMYLOIDOSIS

Field: Medicine (Health Sciences)

From the Department of Cardiology, the Middlesex Hospital Primary amyloidosis is considered to be a rare disease.In 1939 Lindsay and Knorp reviewed 49 cases, and since then approximately 30 more have been recorded.The condition is important in the differential diagnosis of congestive cardiac failure of obscure origin.It may present a difficult clinical problem, as in the case reported here. Case ReportIn September, 1953, a woman of 46 years, who had previously been well, developed swelling of the ankles.This gradually increased until in February, 1954, she was admitted to hospital.She was treated in bed with a low sodium diet and mersalyl, and in March she was discharged home, free from cedema.Two months later the symptoms returned and she was readmitted.The congestive failure again responded to rest and mersalyl.She was transferred to the Middlesex Hospital in August with a diagnosis of either constrictive pericarditis or myocarditis with failure.She was a pale, well-covered woman with a slightly hoarse voice.Pulse regular, 96 a minute.Blood pressure, 110/80.Venous pulsation was visible one inch above the sternal angle at 450, and the liver was enlarged three finger breadths below the costal margin.There was ascites and gross cedema of sacrum and legs.There were no palpable cardiac pulsations.The heart sounds were faint.There was an apical presystolic gallop but no murmurs were heard.The lungs were dry and the spleen was impalpable.Urine: heavy albuminuria; total urinary protein 10 9 g. in 24 hr.; deposit: some leucocytes, epithelial cells, and hyaline casts in some specimens.Electrocardiogram showed sinus rhythm and low voltage QRS complexes with flat T waves.Chest X-ray: increased transverse diameter of heart; lung fields clear; no pericardial calcification.Radioscopy: diminished cardiac pulsation.Blood count and haemoglobin normal.E.S.R., 39 mm. in one hour.W.R. negative.Blood urea normal.Urea clearance, renal function 49 per cent.Serum cholesterol, 560 mg. per 100 ml.Serum proteins, total, 4-7 g. per 100 ml.(albumin 2 1; globulin 2 6).Electrophoretic pattern of serum proteins showed grossly increased alpha 2 band.No amyloid seen in gum biopsy, and no " L.E." cells in blood smear.B.M.R., plus 1 per cent.Cardiac catheterization and angiocardiography were unsuccessful because of a venous anomaly which prevented the catheter from being passed beyond the neck.In spite of treatment in bed with a low sodium high protein diet, mercurial diuretics, and katonium, the cedema increased rapidly and heavy albuminuria continued.Acupuncture was performed and 17 pints of oedema fluid were drained from the legs in 24 hours.Drainage was then discontinued as the patient be- came drowsy and complained of nausea.The serum electrolytes at that time were within normal limits.The venous pressure remained raised and she continued to retain fluid.The blood urea rose from normal to 79 mg. per 100 ml.On November 16, auricular fibrillation commenced and the blood pressure dropped to 90/60 mm.Hg.Suddenly death took place on November 26.Necropsy.There was congestive cardiac failure with 5 pints of ascitic fluid, systemic cedema, and a nutmeg liver.No evidence of pericardial fibrosis was found, but there was generalized hypertrophy of the myocardium which contained a number of greyish waxy plaques.The lungs, kidneys, and spleen appeared normal.There was no evidence of chronic suppuration or tuberculosis.Histology.The myocardium was infiltrated by an amyloid-like substance, deposited in the walls of capillaries and arterioles and around the myocardial fibres.Deposits of a similar material were present in the walls of capillaries, arterioles, and small arteries throughout the body, particularly those of the liver, spleen, suprarenals, and tongue.There was heavy infiltration of the basement membrane of sweat glands 422 on August 19,