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Cardiac Involvement in Patients with Sarcoidosis^*

Diagnostic and Prognostic Value of Outpatient Testing

^* From the Zena and Michael Wiener Cardiovascular Institute (Drs. Mehta, Lubitz, and Goldman), and the Division of Pulmonary, Critical Care, and Sleep Medicine (Drs. Wisnivesky and Teirstein), Department of Medicine, and Division of Nuclear Medicine (Dr. Machac), Department of Radiology, Mount Sinai School of Medicine, New York, NY; the Division of Cardiology (Dr. Frankel), Department of Medicine, New York University School of Medicine, New York, NY; and the Cardiology Division (Dr. Einstein), Department of Medicine, Columbia University Medical Center, New York, NY.

Correspondence to: Steven Lubitz, MD, Mount Sinai Medical Center, One Gustave L Levy Pl, Box 1030, New York, NY 10029; e-mail: steven.lubitz{at}mssm.edu

Abstract

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

Methods: Ambulatory patients with sarcoidosis were interviewed to determine whether they experienced palpitations, syncope, or presyncope, and were evaluated with ECG, Holter monitoring, and echocardiography (transthoracic echocardiogram [TTE]). Those with symptoms or abnormal results were studied with cardiac MRI (CMRI) or positron emission tomography (PET) scanning. 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%, respectively; p < 0.001), and were more likely to have abnormal Holter monitoring findings (50% vs 3%, respectively; p < 0.001) and TTE findings (25% vs 5%, respectively; p = 0.02). The degree of pulmonary impairment did not predict CS. Two of the 17 patients who underwent EPS had abnormal test findings and received implantable cardioverter-defibrillators. No patients died, had ventricular arrhythmias that triggered defibrillator therapy, or had heart failure develop during almost 2 years of follow-up. This diagnostic approach was more sensitive than the established criteria for identifying CS.

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

Key Words: cardiomyopathy • MRI • positron emission tomography • sarcoidosis • sudden cardiac death

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