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CASE REPORT article

Front. Cardiovasc. Med.
Sec. Pediatric Cardiology
Volume 11 - 2024 | doi: 10.3389/fcvm.2024.1477359

Case report: The Unrelenting Journey -Successful Resolution of Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) through Right Cardiac Sympathetic Denervation in a Teenager After Left Cardiac Sympathetic Denervation

Provisionally accepted
Hei To Leung Hei To Leung 1Sit Yee Kwok Sit Yee Kwok 1*Ming Lau Ming Lau 2Kwok-fai Lucius Lee Kwok-fai Lucius Lee 2Sabrina Tsao Sabrina Tsao 3
  • 1 Hong Kong Children's Hospital, Kowloon, Hong Kong, SAR China
  • 2 Queen Mary Hospital, Hong Kong, Hong Kong, SAR China
  • 3 The University of Hong Kong, Pokfulam, Hong Kong, SAR China

The final, formatted version of the article will be published soon.

    Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited arrhythmia disorder characterized by ventricular arrhythmia triggered by adrenergic stimulation.Case presentation: A 9-year-old boy presented with convulsions following physical exertion. Bidirectional ventricular tachycardia (VT) during a treadmill test led to the diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT). Genetic testing revealed a pathogenic variant of RYR2:c.720G>A (p.ArG2401His). Nadolol was initially started. However, he experienced aborted VT arrest three years later.Flecainide was thus added as dual therapy and he underwent left cardiac sympathetic denervation (LCSD). Subsequently, a transvenous implantable cardioverterdefibrillator (ICD) was implanted because he still had several episodes of bidirectional VT. Despite a good compliance to medication, the patient still had exercise induced VT episodes with new onset of atrial fibrillation. High dose nadolol was required and amiodarone was added. Despite maximizing the dosage of these three antiarrhythmics, the patient continued to experience multiple episodes of ventricular fibrillation with appropriate ICD shocks and persistent atrial arrhythmias. Right cardiac sympathetic denervation (RCSD) was performed as the last modality of treatment. Patient had a total elimination of VT post bilateral sympathectomy. He remained asymptomatic on follow up. A follow-up treadmill test showed no recurrence of exercise-induced PVCs and VT.We illustrated the challenges and the complex decision-making process encountered in managing refractory CPVT. In patients unresponsive to conventional therapies, RCSD in additional to LCSD is a safe and effective alternative treatment. A history of LCSD should not preclude physicians from considering RCSD in children with refractory CPVT.

    Keywords: Catecholaminergic polymorphic ventricular tachycardia (CPVT), Right cardiac sympathetic denervation, Left cardiac sympathetic denervation, Bilateral Cardiac Sympathectomy, Paediatric, case report

    Received: 07 Aug 2024; Accepted: 25 Nov 2024.

    Copyright: © 2024 Leung, Kwok, Lau, Lee and Tsao. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Sit Yee Kwok, Hong Kong Children's Hospital, Kowloon, Hong Kong, SAR China

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