Acute Coronary Syndrome Increases Risk of In-Hospital Mortality in TTP

Investigators found patients with acute coronary syndrome were 3 times more likely to die in the hospital.

Patients with thrombotic thrombocytopenic purpura (TTP) face a higher risk of morbidity and mortality if they also have acute coronary syndrome (ACS), according to a new analysis.

The report opens up new questions about how best to manage cardiac involvement in these patients, the authors said. Their findings were published in Cureus.

TTP is caused by a deficiency of the von Willebrand factor–cleaving protease ADAMTS13. Therapeutic plasmapheresis has long been used to manage patients and reduce mortality among people with the disease, explained the study authors. Still, even with appropriate treatment, the disease has a 10% mortality rate, and commonly leads to morbidities such as myocardial ischemia.

The study investigators wanted to better understand how ACS affected the outcomes of patients hospitalized with TTP. They used the National Inpatient Sample dataset to search for patients who were hospitalized with thrombotic microangiopathy between 2002 and 2017 and who also received plasma exchange therapy during their hospital stay. They found 6214 patients. Those patients were then categorized based on whether they had ACS at the time of admission to compare outcomes between the 2 subgroups. The ACS group included 390 patients.

Those with ACS tended to be older, and they were more likely to have a history of coronary artery disease, dyslipidemia, diabetes, essential hypertension, chronic kidney disease, and heart failure, the authors found.

ACS also was associated with significantly worse outcomes. The in-hospital mortality rate in the ACS group was 19.5% compared with 8.4% in the non-ACS group. Those with ACS stayed an average 4 days longer in the hospital, the investigators found. The ACS subgroup was also at a higher risk of a host of other complications, including acute kidney injury, acute respiratory failure, sepsis, and acute congestive heart failure.

The study authors said previous research has found a wide range of risk factors associated with in-hospital mortality in people with TTP, including older age, congestive heart failure, and cancer. Those findings line up with the data in the new report, the authors said, which found that older age, a history of heart failure, and coronary artery disease were independent predictors of ACS.

The investigators said their large data set confirms that cardiac involvement has a major impact on the prognosis of patients with TTP.

“This should raise providers’ awareness of such complications and call for further studies to determine whether cardiac treatment would alter the course of the disease and improve the prognosis,” they wrote.

They noted several limitations to their research. As with any claims database, they said there is a possibility for coding errors, underreporting of comorbidities, and other omissions. Also, the design of the survey, which is based on discharge-level information, did not allow them to evaluate long-term outcomes for these patients, including rates of recurrence.

Additional studies will be needed, they emphasized, to understand the incidence and impact of cardiac involvement in TTP and to better understand the impact of interventions on long-term outcomes.


Khalil F, Ali M, Ellithi M. Impact of acute coronary syndrome on clinical outcomes in patients with thrombotic thrombocytopenic purpura. Cureus. Published online March 7, 2023. doi:10.7759/cureus.35878

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