Incessant Ventricular Tachycardia Secondary to Giant-Cell Myocarditis Treated with Stellate Ganglion Blockade

Abstract

Background Giant cell myocarditis (GCM) is a cardiac inflammatory disease that is characterized by the widespread infiltration of multinucleated giant cells interspersed in the myocardium, cardiomyocyte necrosis and eventually fibrosis. This disorder is rare, rapidly progressive and frequently fatal. Typically, it manifests with fulminant heart failure, atrioventricular blocks and/or ventricular tachyarrhythmias. Current therapies rely on the use of immunosuppression, ventricular assist devices and ultimately cardiac transplantation. Introduction We describe the successful cessation of ventricular tachyarrhythmias in the setting of GCM with the use of a stellate ganglion blockade. Case A 47-year-old female with a history of Hashimoto's thyroiditis presented to an outside hospital with flu-like symptoms and dyspnea. Had a NT-proBNP of 6,080 pg/mL and TroponinT of 1.3 ng/mL. EKG demonstrated ST depressions in the anterolateral leads. Echocardiography showed severe LV dysfunction with global hypokinesis and an LVEF 25%. Telemetry revealed multiple runs of polymorphic sustained ventricular tachycardia (VT). Coronary angiography demonstrated normal coronary arteries. The patient was started on IV amiodarone, IV lidocaine, a continuous magnesium drip and transferred to our facility. A cardiac MRI showed extensive late gadolinium enhancement of the subendocardium at the inferobasal septum, mid wall and the epicardium along the mid septum. She continued to have incessant multifocal VT, despite being on three AAD's. Thus, we intubated her and placed her under general anesthesia, but she continued to have VT. The decision was made to perform a left stellate ganglion blockade, which was immediately successful at suppressing the electrical storm. A right heart catheterization revealed a cardiac index of 1.4 L/min/m2 and a mean PCWP of 27 mmHg. The patient underwent IABP insertion followed by an endomyocardial biopsy of the RV. Biopsy was consistent with GCM and the patient was started on cyclosporine and high dose steroids while she was evaluated for cardiac transplantation. Two days later she received a heart and has been doing well post-operatively. Conclusion To our knowledge this is the first case reported where incessant VT in GCM is treated with a stellate ganglion nerve blockade. This technique should be considered in GCM patients presenting with electrical storm refractory to AAD's.