Intracardiac Thrombosis and Embolism in Patients With Cardiac Amyloidosis

Abstract

Background-Patients with primary amyloidosis (AL type) have a poor prognosis, in part due to frequent cardiac involvement. Although intracardiac thrombus has been reported in anecdotal cases, neither its frequency nor its role in causing mortality is known. Furthermore, the clinical and echocardiographic variables that may be associated with thromboembolism in cardiac amyloidosis have not been defined. Methods and Results-A total of 116 autopsy or explanted cases of cardiac amyloidosis (55 AL and 61 other type) were identified in the Mayo Clinic. Forty-six fatal nonamyloid trauma cases served as controls. Each heart was examined for intracardiac thrombus. The cause of death was determined from autopsy and clinical notes. Intracardiac thrombosis was identified in 38 hearts (33%). Twenty-three had 1 thrombus, whereas 15 had 2 to 5 thrombi. Although subjects in the AL group were younger and had less atrial fibrillation than those with other types of amyloidosis, the AL group had significantly more intracardiac thrombus (51% versus 16%, PϽ0.001) and more fatal embolic events (26% versus 8%, PϽ0.03). Control hearts had no intracardiac thrombus.

Assessing the Risk and Prognosis of Thrombotic Complications in Cancer Patients

“…Cardiovascular thrombotic events are found in 26% to 33% of patients with primary amyloidosis, despite preserved left ventricular ejection fraction and absence of cardiac arrhythmias. 41 Feng et al 41 reported a 26% mortality rate in instances of amyloidosis-related thrombosis. At the time of diagnosis, the prevalence of thrombosis is 34% to 39% and 10% to 29% in polycythemia vera and essential thrombocythemia patients, respectively, 42 and 64% to 96.7% of these episodes have an arterial origin.…”

Intracardiac Thrombosis, Embolism and Anticoagulation Therapy in Patients with Cardiac Amyloidosis – Inspiration from a Case Observation

“…4,13 Furthermore, in our autopsy series, systemic embolism was a significant cause of mortality. 13 However, it is possible that autopsy series may over emphasize the frequency of intracardiac thrombosis and there only have been a few anecdotal reports in the literature on intracardiac thrombosis detected by TEE or TTE in live patients. [9][10][11][12][15][16][17] The prevalence of intracardiac thrombus and the effects of anticoagulation in living cardiac amyloid patients has not been reported.…”

“…We searched the Mayo Clinic Tissue Registry database for cases of cardiac amyloidosis from1996 to 2005. 13 …”

“…Other abstracted information included results of tissue biopsies, urine and serum protein electrophoresis, immunofixation, serum free light chain assay, genetic testing, and family history. 13 …”

“…TTE and TEE studies were reviewed independently without knowledge of the clinical and pathological data and was reported previously 13 The TEE studies were reviewed for presence or absence of intracardiac thrombus and their location and size. 14 Left atrial appendage (LAA) and LA spontaneous contrast and their severity were semi-quantified as none, mild, moderate or severe (0, 1, 2, or 3 respectively).…”