[The rapid progress of heart failure due to systemic amyloidosis with cardiac involvement--case report]

Amyloidosis is a disease having many different faces. Different symptoms may appear, depending on which organ is involved. That's why correct diagnosis can be difficult. Cardiac involvement must always be considered because of poor prognosis (30 to 68 % patients survive one year). Also in case of rapid progress of cardiac wall thickening, amyloidosis should be taken into account.

we present a case of a female patient with rapid progress of heart failure due to systemic amyloidosis with cardiac involvement.

48-old female, with no prior medical history, admitted to cardiology ward because of dyspnea on exertion and leg edema. Couple days before admission hypertrophic cardiomyopathy was diagnosed. Laboratory test revealed elevated troponin I, d-dimers and BNP (natriuretic peptide type B). Electrocardiogram showed low QRS voltage in limb leads. Echocardiography confirmed concentric thickening of left ventricular walls and reduced ejection fraction (40%). We performed cardiac magnetic resonance. Morphology of the delayed enhancement and an increased signal in T2 dependent sequences suggested overlap of general inflammatory process and hypertrophic cardiomyopathy. Because of amyloidosis suspicion, gingival and subcutaneous adipose tissue biopsies were performed. Sirius red stain identified amyloid only in the walls of gingival blood vessels. Diagnosis of amyloidosis was established and further diagnostics planned. Soon after patients condition worsened. Finally, in intensive care unit, after cardiac arrest patient died.

Amyloidosis with cardiac involvement has a very poor prognosis. Multiple tissue biopsy and histopathological assessment should lead to correct diagnosis and proper treatment.