Abstract
BACKGROUND: This case highlights the management of concomitant acute myocarditis and congenital long QT syndrome with electrical storm and incessant Torsade de Pointes. CASE PRESENTATION: An 18 years-old Southeast Asian para 1 abortus 0 (P1A0) postpartum patient with cesarean section owing to severe preeclampsia, acute lymphocytic myocarditis, and prolonged QT interval owing to long QT syndrome. She has incessant Torsade de Pointes treated with beta-blocker, lidocaine, overdrive pacing with a temporary transvenous pacemaker, left cardiac sympathetic denervation per video-assisted thoracoscopic surgery, and implantable cardioverter-defibrillator implantation. We initially used bisoprolol, then switched to propranolol and finally to carvedilol, which reduced the Torsade de Pointes frequency. The longest QTc interval was 696 ms, and the shortest was 624 ms, 2 months after initial corticosteroid administration and left cardiac sympathetic denervation. Device interrogation at 9 months follow up showed three episodes of ventricular fibrillation, 2 spontaneously resolved and one necessitates shock. CONCLUSION: Management of concomitant acute myocarditis and congenital long QT syndrome with incessant Torsade de Pointes requires beta-blockers, anti-inflammatory drugs, autonomic modulation, and short-term measures, such as overdrive pacing with deep sedation. Implantable cardioverter-defibrillator is vital to prevent sudden cardiac death.