Key result
Atrial leadless pacing enables antiarrhythmic up-titration and ICD deferral in severe CPVT-related bradycardia.
Case Report (n=1)
In patients with CPVT and coexisting sinus node dysfunction, atrial leadless pacing can safely treat limiting bradycardia to allow maximal guideline-directed beta-blocker and flecainide therapy while deferring ICD implantation.
Case illustrates beta-blocker dosing limits in CPVT with sinus node dysfunction; leaves open optimal management and requires prospective study.
Beta-blockade is the cornerstone of therapy in catecholaminergic polymorphic ventricular tachycardia (CPVT), but coexisting sinus node dysfunction can prevent adequate dosing. A woman aged in her late 20s presented with exertional syncope. She had profound sinus bradycardia and reproducible exercise-induced multiform ventricular ectopy in a structurally normal heart, supporting a diagnosis of CPVT, later confirmed with genetic testing (pathogenic RYR2 variant). Resting heart rates in the low 30s precluded beta-blocker initiation. After shared decision-making in which she declined transvenous leads, an atrial leadless pacemaker was implanted, permitting up-titration of nadolol with flecainide and deferral of an implantable cardioverter-defibrillator. Sinus node dysfunction is recognized in CPVT but is seldom framed as a modifiable obstacle to guideline-directed therapy. Treating the bradycardia allowed medical therapy to be maximized before device therapy, consistent with consensus recommendations. Bradycardia that limits beta-blockade in CPVT is a pacing problem rather than drug intolerance.
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Timilsina et al. (2026) conducted a case report in Catecholaminergic polymorphic ventricular tachycardia (CPVT) with profound sinus bradycardia (n=1). Atrial leadless pacemaker implantation followed by nadolol and flecainide was evaluated on Up-titration of guideline-directed medical therapy (nadolol with flecainide) and deferral of an implantable cardioverter-defibrillator.
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