Abstract 063: Diagnostic and Treatment Patterns of Cardiac Sarcoidosis: a Single Center Experience
Background: Clinically manifest cardiac involvement occurs in approximately 5% of patients with sarcoidosis. The principal manifestations of cardiac sarcoidosis (CS) are conduction abnormalities, ventricular arrhythmias, and heart failure. In 2014, the first international guideline for the diagnosis and management of CS was published. There are emerging data to support cardiac MRI and PET imaging in the diagnosis of CS. Additionally, despite a paucity of data, immunosuppression therapy (primarily with corticosteroids) has been advocated for the treatment of clinically manifest CS. Device therapy, primarily with implantable cardioverter-defibrillators, is often recommended for patients with clinically manifest disease. There are few contemporary descriptions of treatment and outcomes related to CS. In this case series, we describe diagnostic and treatment patterns of thirty patients with CS. Methods: We performed a retrospective chart review of 30 patients with diagnosed CS. We used a spreadsheet with deidentified patient information to describe baseline patient characteristics including age, presenting symptoms, diagnostic modality, duration of follow up, medication regimen and follow up imaging, as well as the variables listed above concerning those who are deceased. Results: Of thirty patients included in this case series, age at diagnosis ranged from 28-69, the diagnostic modalities included autopsy of explanted heart, cardiac MRI in combination with mediastinal lymph node biopsy, endomyocardial biopsy, cardiac MRI, and cardiac PET. For the majority of patients, dyspnea was the presenting symptom. Most patient presented with NYHA class I or II symptoms. Half of patients underwent ICD implantation. Eleven patients had documented arrhythmias, including ventricular tachycardia, complete heart block, and atrial fibrillation. Of our cohort, eight patients have concomitant pulmonary sarcoidosis. Follow up duration has ranged between 1 and 12 years. Five patients proceeded to cardiac transplantation. Two patients are deceased, including one patient who suffered recurrence of cardiac sarcoidosis following orthotropic heart transplantation. Conclusions: In this case series of thirty patients with cardiac sarcoidosis, we describe that patients are commonly undergoing cardiac MR and PET imaging to support the diagnosis of CS. Tissue diagnosis remains a standard. Immunomodulators and cardiac device are frequently used, and further follow up data would be helpful in describing outcomes related to these therapies. This descriptions may prompt longer term observational studies that document diagnostic and therapeutic modalities, as well as cardiovascular outcomes, related to cardiac sarcoidosis.