OBJECTIVES: This report characterizes a syndrome of granulomatous infiltration presenting as sustained monomorphic ventricular tachycardia (SMVT) with mediastinal adenopathy in patients with preserved ventricular function. BACKGROUND: Unlike truly idiopathic ventricular tachycardia, SMVT due to granulomatous infiltration responds poorly to radiofrequency ablation and has a poor prognosis. METHODS: Patients without obstructive coronary artery disease and with normal ventricular function presenting with SMVT other than posterior fascicular morphology were evaluated. Computed chest tomograms, cardiac magnetic resonance imaging, and 18-fluorodeoxyglucose positron emission tomographic scans ((18)FDG PET-CT) were performed. Significant lymph nodes were evaluated for tuberculosis and sarcoidosis. Initial treatment included antiarrhythmic drugs ± radiofrequency ablation. Additionally, patients with evidence of tuberculosis received anti-tuberculosis therapy; the rest were treated as sarcoidosis. RESULTS: Mediastinal adenopathy with mid-myocardial scar and/or focal myocardial inflammation was observed in 14 patients; lymph nodes revealed noncaseating granulomas in all. Evidence of tuberculosis was present in 79%. During follow-up (median duration 25 months), SMVT recurred despite initial treatment in 92%. Addition of disease-specific therapy abolished further recurrences in 64% of them. Decrease in SMVT correlated with resolution of myocardial inflammation on serial (18)FDG PET-CTs. Appropriate therapies occurred in 67% of patients receiving implantable cardioverter-defibrillators. CONCLUSIONS: A subset of patients with SMVT with preserved ventricular function has a syndrome of arrhythmogenic myocarditis with granulomatous mediastinal adenopathy due to myocardial tuberculosis or cardiac sarcoidosis. This entity is optimally managed with a combination of disease-specific therapy and antiarrhythmic measures.
OBJECTIVES: This report characterizes a syndrome of granulomatous infiltration presenting as sustained monomorphic ventricular tachycardia (SMVT) with mediastinal adenopathy in patients with preserved ventricular function. BACKGROUND: Unlike truly idiopathic ventricular tachycardia, SMVT due to granulomatous infiltration responds poorly to radiofrequency ablation and has a poor prognosis. METHODS:Patients without obstructive coronary artery disease and with normal ventricular function presenting with SMVT other than posterior fascicular morphology were evaluated. Computed chest tomograms, cardiac magnetic resonance imaging, and 18-fluorodeoxyglucose positron emission tomographic scans ((18)FDG PET-CT) were performed. Significant lymph nodes were evaluated for tuberculosis and sarcoidosis. Initial treatment included antiarrhythmic drugs ± radiofrequency ablation. Additionally, patients with evidence of tuberculosis received anti-tuberculosis therapy; the rest were treated as sarcoidosis. RESULTS:Mediastinal adenopathy with mid-myocardial scar and/or focal myocardial inflammation was observed in 14 patients; lymph nodes revealed noncaseating granulomas in all. Evidence of tuberculosis was present in 79%. During follow-up (median duration 25 months), SMVT recurred despite initial treatment in 92%. Addition of disease-specific therapy abolished further recurrences in 64% of them. Decrease in SMVT correlated with resolution of myocardial inflammation on serial (18)FDG PET-CTs. Appropriate therapies occurred in 67% of patients receiving implantable cardioverter-defibrillators. CONCLUSIONS: A subset of patients with SMVT with preserved ventricular function has a syndrome of arrhythmogenic myocarditis with granulomatous mediastinal adenopathy due to myocardial tuberculosis or cardiac sarcoidosis. This entity is optimally managed with a combination of disease-specific therapy and antiarrhythmic measures.
Authors: Henri Roukoz; Mandar Shah; Lawrence Jesuraj Masilamani; Ajit Thachil; Prem K Jayakumar; David G Benditt; Calambur Narasimhan Journal: Indian Heart J Date: 2015-04-30
Authors: Joseph P Lynch; Jennifer Hwang; Jason Bradfield; Michael Fishbein; Kalyanam Shivkumar; Roderick Tung Journal: Semin Respir Crit Care Med Date: 2014-07-09 Impact factor: 3.119