P38 | Heart failure…or maybe not?

Premise: AL amyloidosis is a rare systemic disease that can present with a variety of signs and symptoms, including proteinuria, edema, hepatosplenomegaly, and heart failure.Description of the Case report: 80 year old male presented with dyspnea and sloping edema in known heart failure with pleural effusion. Echocardiogram shows a small and markedly hypertrophic left ventricle, normal systolic function, dilated atria, dilated and hypertrophic right ventricle. The ECG showed a low voltage QRS. The severe biventricular hypertrophy pointed the diagnosis towards a storage disease. The patient was hospitalized to continue the diagnostic-therapeutic process. Blood tests show an increase in serum free kappa chains and in the kappa/lambda ratio and troponin. Abdominal ultrasound revealed hepatomegaly. A bone scan was subsequently performed with no evidence of areas of accumulation of the radiopharmaceutical in the heart muscle. Instead, cardiac magnetic resonance imaging with gadolinium showed the left ventricle with reduced volumes, a hypertrophic appearance with preserved ejection fraction and radiological criteria compatible with the suspicion of AL amyloidosis.Conclusions: To have a certain diagnosis it is necessary to perform a biopsy of the periumbilical fat, which however was negative. Therefore it was decided to perform an osteomedullary biopsy compatible with the diagnosis of multiple myeloma with evidence of Congo Red-positive amyloid deposits.