| Background: Amyloidosis is characterised by the extracellular deposition and accumulation of insoluble fibrillar proteins, with concomitant destruction of normal tissue structure and function. There are similarities and differences in the clinical manifestations of amyloidosis resulting from different precursor proteins. All amyloid fibrils share apple green birefringence under polarized light with Congo-red staining, and are arranged in beta-pleated structure on electron microscopy. More than 18 proteins have been identified that may give rise to amyloid in vivo including light chain immunoglobulins, transthyretin, acute phase reactant - protein A, fibrinogen Aa, apolipoprotein A etc. Exciting progress is being made in our understanding of the process of amyloidogenesis, mechanisms of tissue damage due to amyloidosis, in genetics of amyloidosis and related areas raising the hope of effective treatment of the disease. Cardiovascular system often was involved and up to 50% of patients with amyloidosis have significant deposition of amyloid in the heart, termed cardiac amyloidosis (CA).The hallmark of this disorder is the "stiff heart syndrome" , characterized by early impairment of diastolic function and relatively preserved systolic functionuntil late in the disease. The presentation of cardiac amyloidosis is restritive cardiomyopathy, thus it is typical and natural model for studying cardiac diastolic dysfunction. Destination: Previous studies have consistently shown that diastolic function of cardiac amyloidosis is abnormal secondary to amyloid infiltration. The purpose of this study was to investigate the catalog of diastolic function in patients of amyloidosis with evidence of heart involvement with normal or abnormal systolic function. Standard mitral inflow and pulmonary vein flow characteristics were measured. Tissue Doppler echocardiography was used to measure E' at the lateral annulus. Color M-mode flow propagation velocity (Vp) was analyzed for all of patient' s studies.Methods: From January 1992 to November 2003,in The Cleveland Clinic Foundation , seventy-four patients with primary cardiac amyloidosis confirmed by biopsy (55 men;age 65. 7 + 10 years) were involved in this study. Patients were divided into two groups according LV ejection fraction. Group I : normal systolic function (EF>50%), Group II (EF^50%). The peak velocities of early(E) and late(A) filling waves, early/late filling ratio of peak velocities, and deceleration time of early filling waves were measured from transmitral flow velocities, and the peak velocities of systolic, diastolic, and atrial contractile waves, and diastolic/systolic ratio of peak velocities were also measured from the pulmonary venous flow velocities. Color M-mode flow propagation velocity (Vp) and E/Vp were measured. Peak systolic wall motion velocities, peak earlydiastolic wall motion velocities, and peak late diastolic wall motion velocities were measured with TDI. According the mitral inflow and pulmonary venous Doppler pattern, we classified the patients into three stages of diastolic dysfunction. Results: There were 32 patients (26 male;age 67 ±10 years) with Group I , and 42 patients (29 male;age 64 ±10 years) with abnormal Group II . Vp , E' and A' in group II decreased significantly than that in group I .While the filling pressure index E/Vp and E/E' are increased significantly in Group II than that in group I .The total patients have abnormal diastolic flow pattern. There were most (69 %) of patients with restrictive diastolic pattern , which significantly higher than LV EF normal group (27 %), p<0.005.Conclusions: There were 43 % of patients with cardiac amyloidosis having normal systolic function when diagnosis confirmed by biopsy. The diastolic function was significantly worse in patients with abnormal LVEF than whom with normal LVEF. Vp and TDI can help to value early diastolic dysfunction.
|
PDF Full Text Request