PRIMARY SYSTEMIC AMYLOIDOSIS WITH CARDIAC INVOLVEMENT

History and findings: A Vietnamese woman, now 68 years old, had for te n years been known to have a monoclonal lambda Light-chain gammopathy. Two years before the present admission her resting ECG had shown abse nt R waves in V-2 to V-4, first-degree A-V block and preterminal negat ive T waves. Results of left heart catheterization and echocardiograph y were essentially normal. Gradually increasing dyspnoea over the prec eding 9 months, recently even at rest, and a poor general state with c linical signs of heart failure led to her hospital admission. Tests: L aboratory tests confirmed the known gammopathy without evidence of pla smacytoma. An echocardiogram now demonstrated a moderately enlarged le ft atrium and hypertrophied left ventricular wall, as well as restrict ive function with an increased ratio of early to late diastolic fillin g velocity and shortened deceleration time. Myocardial echogenicity wa s increased. Rectal biopsy showed numerous interstitial and paravascul ar amyloid fibrillae. All these findings indicated restrictive cardiom yopathy as part of primary systemic amyloidosis.Treatment and course: After treatment with frusemide (80 mg twice daily) and pacemaker impla ntation she was discharged, her cardiac status now in NYHA class III. Neither chemotherapy nor cardiac transplantation is contemplated.