Underdiagnosis of VT due to cycle length variation among cardiac sarcoidosis patients having ICD: Problem with...

Implantable cardioverter defibrillator (ICD) is recommended for patients with ventricular tachycardia (VT) due to cardiac sarcoidosis (CS). Programming supraventricular tachycardia (SVT) discriminators (onset, stability, and morphology/template match) is generally recommended to minimize inappropriate therapies. However, VT in patients with CS is known to show cycle length variability (CLV) and pleomorphism.

To determine whether the stability criterion, designed to prevent inappropriate therapy during atrial fibrillation with rapid ventricular rates, could potentially lead to incorrect classification of VT as SVT and inappropriately delay or inhibit ICD therapy.

Cases of biopsy-proven CS with VT were analyzed. For patients with implanted devices, all recorded electrograms of tachycardia episodes and ICD therapies were analyzed at last follow up.

A total of 142 patients were included (mean age 38 years, 87 males). One hundred and three of 142 patients had implanted devices (ICD or CRT-D). Thirty eight of 103 (36.9%) patients received appropriate ICD therapies over 3 ± 2.2 years follow up. Four of 38 (10.5%) of patients experienced delayed-detection or underdetection of VT related to CLV, resulting in VT counters being repeatedly "reset" (classified as "unstable" rhythms). Retrospective analysis of other VT episodes in 70 of 103 (68%) patients revealed that 25 of 80 (31.3%) episodes had > 50 ms cycle length oscillations.

Among CS patients with VT, CLV is a common occurrence seen in two-thirds of VT episodes. Routine programming of the stability criterion may result in underdetection of VT in a subset of such patients. We recommend that the stability criterion should be programmed "OFF" for patients with CS, unless the patient has documented atrial fibrillation.