Sustained ventricular tachycardia associated with sarcoidosis: Assessment of the underlying cardiac anatomy and the prospective utility of programmed ventricular stimulation, drug therapy and an implantable antitachycardia device

Abstract

The presentation, cardiac anatomy and utility of programmed ventricular stimulation in seven patients with sustained ventricular tachycardia associated with sarcoidosis are described. The mean patient age was 38 +/- 8 years. Pulmonary involvement was apparent in three patients and no systemic manifestations of sarcoidosis were present in one patient. All patients had electrocardiographic abnormalities at rest and six had a left ventricular ejection fraction less than 45%. All seven patients had left ventricular wall motion abnormalities and five had mitral valve dysfunction. Sustained ventricular tachycardia was easily induced in all patients. Spontaneous sustained ventricular tachycardia was not prevented with corticosteroid administration. Despite antiarrhythmic drug therapy, two patients had sudden cardiac death and an additional four had recurrence of ventricular tachycardia. Four patients had an automatic cardioverter-defibrillator implanted and received drug therapy; all four received appropriate shocks. This report represents the largest descriptive series of consecutive patients with sustained ventricular tachycardia associated with sarcoidosis. Antiarrhythmic drug therapy of ventricular tachycardia in patients with sarcoidosis, even when guided with programmed ventricular stimulation, is associated with a high rate of arrhythmia recurrence or sudden death, or both. Thus, implantation of an automatic antitachycardia device (cardioverter-defibrillator) should be considered as primary therapy in such patients. Furthermore, sarcoidosis should be excluded, with Kveim skin testing if necessary, in any patient with sustained ventricular tachycardia of unknown origin.

Joint SNMMI–ASNC expert consensus document on the role of 18F-FDG PET/CT in cardiac sarcoid detection and therapy monitoring

“…92 For this reason, an ICD is recommended in CS patients who have sustained ventricular tachycardia or ventricular fibrillation. Other indications for ICD placement in CS patients include prior cardiac arrest or an LVEF of 35% or less despite optimal medical therapy.…”

The Role of Endomyocardial Biopsy in the Management of Cardiovascular Disease

“…64,68,69 Implantable cardiac defibrillators may be effective in treating arrhythmias in patients with ventricular tachycardia related to sarcoidosis. 70,71 After extensive fibrosis of the left ventricle, steroid use is probably of little benefit. Therefore, EMB is reasonable in the clinical setting of unexplained heart failure of Ͼ3 months' duration associated with a dilated left ventricle and new ventricular arrhythmias, Mobitz type II second-or third-degree AV heart block, or failure to respond to usual care within 1 to 2 weeks.…”

Primary Prevention of Sudden Cardiac Death in Silent Cardiac Sarcoidosis

“…8,14 -16 PES has been reported to have some benefit in risk stratification of patients with CS. 10,17 In a prospective study of 32 patients with sarcoidosis completed at this institution, 15 of whom received an ICD, Aizer et al demonstrated that induction of ventricular tachycardia during PES was associated with subsequent arrhythmic event, with a relative hazard of 4.5 in their cohort overall, and a higher hazard rate of 7.0 in patients without prior arrhythmia. 10 Nine of 12 patients with inducible sustained ventricular tachycardia received appropriate ICD therapy over an average follow-up of 84 months.…”