[Chronic heart failure in patients with cardiac amyloidosis].Klin Med (Mosk). 2009; 87(3):26-30.KM
Amyloidosis is a pathology caused by tissue deposition of amyloid, a compound composed of insoluble fibrillar proteins. AL-amyloidosis (primary amyloidosis) most frequently leads to cardiac disorders 50% of which are cases of chronic heart failure (CCF). The study was dictated by the rarity of this pathology among other causes of CCF, its severity, and poor prognosis in the absence of specific therapy. Cardiohemodynamics (CHD) and clinical course of CCF were examined in 12 patients with cardiac amyloidosis (CA) and clinical manifestations of CCF. All the patients died during the study period. The diagnosis was verified at autopsy in 5 patients, by gingival, rectal or pleural biopsy in 4, and from combination of clinical and instrumental findings in the remaining three. The longevity since the onset of CCF was 28 +/- 8.8 and 5.9 +/- 3.8 months in patients under and above 70 respectively. 83.3% of the patients with CA had suffered renal disorders (proteinuria, nephrotic syndrome, insufficiency) and loss of weight before they developed CCF. CCF concurrent with CA was characterized by severely disturbed systemic hemodynamics and refractivity to standard therapy. Cardiac disorders were dominated by changes in myocardium due to the substantial thickening of its walls. The weight of left ventricular myocardium was 358. 77 +/- 58.08g (by echoCG) and the total heart weight 552 +/- 98.4g (at autopsy). Patients with CCF and CA had CHD changes suggesting restrictive cardiomyopathy in 83.3% of the cases and dilatational cardiomyopathy in 16.7%.