engleski

DECOMPENSATION OF CHRONIC HEART FAILURE DUE TO CARDIAC AMYLOIDOSIS-REPORT OF A RARE CASE

Background: Restrictive cardiomyopathy (RC) is a rare form of cardiomyopathy, characterized by diastolic dysfunction and impaired ventricular filling. The most common etiology and archetype of RC is cardiac amyloidosis. Amyloidosis is a rare disease caused by extracellular deposition of insoluble abnormal amyloid f ibrils. Acquired serum amyloid A type (AA) amyloidosis triggered by chronic inflammations, involves the heart in about 2% of cases with systemic AA amyloidosis. Case presentation: We report a case of a 63-year-old female patient with rheumatoid arthritis (RA) and arterial hypertension in her anamnesis and with prior history of myocardial infarction. Patient complained of progressive deterioration in exercise tolerance, occasional chest pain, dyspnea during rest, orthopnea and swollen legs. At admission, blood pressure was 100/60 mmHg with heart rate of 92/min. ECG showed low QRS voltages (standard leads) and voltage criteria for LV hypertrophy. On Chest X-ray, bilateral pleural effusion and myopathic configuration of cardiac silhouette were noticed. BNP was 3600 pmol/L and TnT 374 ng/L. Echocardiography (ECHO) revealed thickened right and left ventricle, ‘ground glass’ appearance of the myocardium, small ventricle cavity size and abnormal diastolic function. Trivial pericardial effusion and moderate mitral regurgitation were also present. All findings revealed the diagnosis of RC most probably due to AA amyloidosis triggered by long-lasting RA, that lead to decompensation of heart failure. Standard therapy for heart failure was introduced. The patient’s condition was constantly deteriorating and she was transferred to intensive care unit. Unfortunately, patient developed cardiac shock and died. On autopsy amyloidosis was confirmed by Congo red staining and polarized-light microscopy. Conclusion: Due to long-lasting autoimmune disorder in anamnesis, typical clinical X-ray, ECG and ECHO f indings cardiac amyloidosis was highly credible. Autopsy has rejected all the possible doubts and confirmed the clinical diagnosis.

Amyloidosis ; Heart failure ; Restrictive cardiomyopathy

nije evidentirano