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Cardiac Involvement in Patients with Sarcoidosis: Diagnostic and Prognostic Value of Outpatient Testing

^aZena and Michael Wiener Cardiovascular Institute, Mount Sinai School of Medicine, New York ^bDivision of Cardiology, Department of Medicine, New York University School of Medicine, New York ^cDivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, Mount Sinai School of Medicine, New York ^dCardiology Division, Department of Medicine, and Department of Radiology, Columbia University Medical Center, New York ^eDivision of Nuclear Medicine, Department of Radiology, Mount Sinai School of Medicine, New York

steven.lubitz{at}mssm.edu

Abstract

Background: Cardiac sarcoidosis (CS) causes substantial morbidity and sudden death. Early diagnosis and risk stratification is warranted.

Methods: Ambulatory patients with sarcoidosis were interviewed to determine if they experienced palpitations, syncope, or presyncope, and were evaluated with an electrocardiogram (ECG), Holter monitoring, and echocardiography (TTE). Those with symptoms or abnormal results were studied with cardiac magnetic resonance imaging (CMR) or positron emission tomography (PET). The diagnosis of CS was based on abnormalities detected by these imaging studies. Patients with CS were referred for risk stratification by electrophysiology study (EPS).

Results: Among the 62 patients evaluated, the prevalence of CS was 39%. Patients with CS had more cardiac symptoms than those without CS (46% vs. 5%, P<0.001), and were more likely to have an abnormal Holter (50% vs. 3%, P<0.001), and TTE (25% vs. 5%, P=0.02). The degree of pulmonary impairment did not predict CS. Two of the 17 patients who underwent EPS had abnormal tests, and received implantable cardioverter-defibrillators. No patients died, had ventricular arrhythmias that triggered defibrillator therapy, or developed heart failure during almost two years of follow-up. This diagnostic approach was more sensitive than established criteria for identifying CS.

Conclusion: CS is common among patients with sarcoidosis. A structured clinical assessment incorporating advanced cardiac imaging with PET or CMR is more sensitive than established criteria for the identification of CS. Sarcoidal lesions on CMR or PET do not predict arrhythmias in ambulatory patients with preserved cardiac function, who appear to be at low risk of short-term mortality.

Key Words: sarcoidosis • cardiomyopathy • sudden cardiac death • magnetic resonance imaging • positron emission tomography