B-PO02-215 MONOMORPHIC PREMATURE VENTRICULAR CONTRACTION TRIGGERING VENTRICULAR FIBRILLATION IN SICKLE CELL CARDIOMYOPATHY

Abstract

The life expectancy of those with sickle cell (SC) continues to rise with treatment but so do the chronic complications including arrhythmias. Report a case of monomorphic PVCs triggering incessant VF in sickle cell cardiomyopathy. N/A A 49-year-old male with SC presented with acute back pain. Hemoglobin (Hb) was 7.2gm/dL and potassium was 6.9mmol/L. ECG revealed sinus rhythm, peaked T waves, and QTc of 460 msec. T waves normalized after hyperkalemia correction. On the 6th day, Hb trickled to 4.7gm/dL. He had frequent monomorphic PVCs triggering/terminating SVT (figure A and B). The following day, he developed abrupt VF triggered by a PVC (figure C) requiring cardiac compression. Echocardiography showed LVEF 35-40% and dilated LA. The patient was commenced on metoprolol tartrate but continued to have monomorphic PVCs and SVT. On the 13th day, the patient underwent EPS, which showed similar PVCs frequently trigger/terminate SVT mediated by a right posterior concealed accessory pathway, which was successfully ablated. No further SVT was induced and the PVC burden decreased with no inducible ventricular arrhythmia. Due to low Hb, LV access for PVC ablation was not pursued. He was implanted an ICD and discharged. Three months later, the ICD interrogation showed 6% PVC burden and no sustained arrhythmia. A week later, the patient collapsed at home and was brought to the ED where he was declared dead. The ICD interrogation showed incessant episodes of VF and the device exhausted all 6 shocks (figure D). Monomorphic PVCs may trigger VF in sickle cell patients that may become incessant and lead to sudden cardiac death despite having a defibrillator.