Cardiovascular Complications and Their Association With Mortality in Patients With Thrombotic Thrombocytopenic Purpura

Background: Despite widespread availability of plasmapheresis, the mortality in thrombotic thrombocytopenic purpura remains high. Cardiovascular complications have been reported as an important cause of morbidity in these patients. The burden and prognostic implications of these complications have not been well studied. We analyzed the rates of cardiovascular complications in thrombotic thrombocytopenic purpura, temporal trends, and studied its impact on in-hospital mortality. Methods: We analyzed the National Inpatient Sample (NIS) from January 2005 to September 2015 to identify adult patients with thrombotic thrombocytopenic purpura. This group was further refined by excluding patients who did not receive therapeutic plasmapheresis, and other conditions that can mimic thrombotic thrombocytopenic purpura. We identified the age- and sex-stratified rates of cardiac arrhythmias, cardiac conduction system disorders, heart failure, acute coronary syndrome, myocarditis, pericarditis, takotsubo cardiomyopathy, cardiogenic shock, cardiac arrest, and stroke. We also compared in-hospital mortality with and without cardiovascular complications. Results: Among 15,054 thrombotic thrombocytopenic purpura hospitalizations (mean age 46.4 years, 69% in the 18- to 54-age group, 66.2% women, and 42.9% white), a cardiovascular complication was observed in 3802 (25.3%) hospitalizations. The following cardiovascular complications were identified: stroke (10.4%), heart failure (8.3%), acute coronary syndrome (6.4%), atrial tachyarrhythmia (5.9%), ventricular tachyarrhythmia (2.0%), cardiogenic shock (0.5%), takotsubo cardiomyopathy (0.1%), atrioventricular block (0.2%), myocarditis or pericarditis (0.3), and cardiac arrest (1.9%). Rates of several cardiovascular complications were significantly higher in patients 55 years or older compared to a younger age group, whereas males had higher rates of acute coronary syndrome and tachyarrhythmias compared to females. Overall, the cardiovascular complication rate was stable during the study period. The presence of a major cardiovascular complication was associated with a significantly higher in-hospital mortality (19.7%) as compared with no major cardiovascular complication (4.1%) (adjusted odds ratio 2.09, 95% confidence interval 1.41-3.09, P <0.001). Results were generally consistent in age and sex subgroups. Conclusion: Cardiovascular complications were frequently observed at a rate of 1 in 4 in patients hospitalized for thrombotic thrombocytopenic purpura and were associated with substantially higher in-hospital mortality. These findings underscore the need to promptly identify and treat these complications to improve outcomes.