A Case of Sarcoidosis Presenting as Refractory Arrhythmias: Quieting the Storm
Aurora L, and Williams CT. A Case of Sarcoidosis Presenting as Refractory Arrhythmias: Quieting the Storm. J Card Fail 2019; 25(8):S126.
J Card Fail
Introduction: Sarcoidosis is a multisystem granulomatous disorder of unknown etiology. Cardiac sarcoidosis can manifest as conduction abnormality, ventricular tachycardia (VT), heart failure or sudden cardiac death. Although well studied in the literature, we present an uncommon case of arrhythmia as a presenting manifestation of this disease. Case: 45-year-old female with no significant past medical history presented with acute onset shortness of breath and palpitations. Initial transthoracic echocardiogram revealed an ejection fraction of 50%. Electrophysiology (EP) study showed a chemically inducible accelerated idioventricular rhythm with right bundle morphology. Left heart catheterization showed angiographically normal vessels and cardiac MRI showed no regional wall motion abnormalities or abnormal delayed enhancement. Patient presented to the hospital again due to recurrence of arrhythmia, found to be VT. Nuclear medicine whole body PET scan showed perfusion defects consistent with cardiac sarcoidosis. A mediastinal lymph node biopsy demonstrated focal non-granulomatous inflammation. Patient was initiated on anti-arrhythmics and underwent a single-chamber ICD placement. She continued to have episodes of wide QRS tachycardia, and was evaluated by Advanced Heart Failure for refractory arrhythmias. Decision-making: Patient was initially managed with Amiodarone and a beta-blocker. Due to unstable VT, she was taken to EP lab where a focal origin of the VT was successfully found in the basal anterolateral endocardial left ventricle and at the base of one of the papillary muscles. Ablations were performed in these areas with reduction in VT rate. Patient was also discovered to have complete atrioventricular block. Subsequently, she developed recurrence of arrhythmia and was admitted again for VT management. Patient was taken back to the EP lab for an endocardial and epicardial ablation. A focal VT source was found in the apical lateral left ventricle. Due to proximity of the phrenic nerve to the site, no ablations were performed. However, due to recurrence of arrhythmia, she was taken back to the lab for a third ablation in the epicardial area with resulting success. Antiarrhythmics were changed to Flecainide which has since suppressed her VT. Patient has been maintained on Methotrexate, Hydroxychloroquine, and Prednisone for continued sarcoid therapy. We are using periodic F-FDG PET imaging to monitor the severity of myocardial inflammation. Based on patient's clinical status and PET imaging, we have determined the time to wean immunosuppressant agents. Conclusion: Cardiac sarcoidosis affects at least 25% of sarcoidosis patients and accounts for substantial mortality and morbidity.Ventricular arrhythmias may be the presenting symptom of cardiac sarcoidosis and are challenging to treat. Risk stratification through proposed techniques such as advanced cardiac imaging and EP studies is integral in this population. Recognition of sarcoidosis as a possible etiology of arrhythmias through integration of clinical and imaging findings can lead to appropriate lifesaving treatments.