October 17, 2016
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Age, lack of cardiac device predicts mortality for cardiac sarcoidosis

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Significant, independent predictors of mortality for patients with cardiac sarcoidosis include age of 46 years or older and lack of an implanted pacemaker or defibrillator, according to recent study findings.

“Cardiac sarcoidosis is a potentially life-threatening condition characterized by the formation of non-caseating granulomas in the heart and is associated with significant mortality,” Ying Zhou, of the department of internal medicine at the University of Cincinnati Medical Center, and colleagues wrote.

They added, “Due to small case series with limited follow up, the risk factors for cardiac mortality are difficult to assess.”

Zhou and colleagues performed a large cohort study within a single institution sarcoidosis clinic and collected data on patient demographics, local and systemic treatments and clinical outcomes to identify cardiac risk factors for survival in patients with cardiac sarcoidosis.

Seventy-three of 1,815 patients with sarcoidosis who were seen over a 6-year period met the updated World Association of Sarcoidosis and Other Granulomatous disease criteria for highly probable or probable cardiac sarcoidosis and were included in the study. Participants had a median diagnosis age of 46 years and were followed for a median of 8.8 years.

Data showed that reduced left ventricular ejection fraction (LVEF) occurred in more than half (54.8%) of patients. Of arrhythmic patients with or without reduced LVEF, 35.6% experienced ventricular tachycardia and 19.2% experienced severe heart block.

Cardiac PET and/or MRI scans were performed on 61.6% of patients (n = 45). Of those, 91.1% tested positive for suspicious involvement (n = 41).

The researchers used Kaplan-Meier survival curves to determine that the 5-year survival rate was 95.5% and the 10-year survival rate was 93.4%. Follow-up analysis revealed that transplant was conducted in 3 patients. Seven patients died from sarcoidosis.

Improved survival was seen in patients with a diagnosis age of 46 years or younger, implanted pacemaker or defibrillator, mycophenolate treatment and/or an LVEF greater than 40%. Independent predictors of mortality included age of 46 years or older and lack of pacemaker or defibrillator. Patients with reduced LVEF less than 40% were found to have worse prognosis.

“Previous reports suggest that cardiac sarcoidosis is associated with poor prognosis because of severe arrhythmias or congestive heart failure. Due to the early recognition and expanded use of immunomodulatory medication and implanted defibrillators and pacemakers, the overall prognosis seems to have improved,” Zhou and colleagues concluded. “Further work is needed to develop a specific treatment algorithm, including the possible role of mycophenolate and other cytotoxic drugs.” – by Alaina Tedesco

Disclosure: Zhou reports no relevant financial disclosures. Please see full study for complete list of disclosures from all other authors.