Why the study?
Catecholaminergic polymorphic ventricular tachycardia is a fatal cardiac ion channelopathy causing sudden unexpected death in the young, requiring specialized perioperative anesthetic management to prevent fatal arrhythmias.
Careful anesthetic management avoiding perioperative stress with premedication and postoperative sedation can prevent fatal arrhythmias in children with CPVT undergoing ICD insertion.
Anecdotal success with midazolam-fentanyl-remifentanil in pediatric CPVT ICD implantation; leaves open standardized anesthetic protocols for rare channelopathies.
BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a fatal cardiac ion channelopathy that causes sudden unexpected death in the young. CASE PRESENTATION: The patient was a 3-year-old girl with CPVT. Insertion of an implantable cardioverter defibrillator (ICD) using epicardial pacing was scheduled. After premedication of rectal midazolam was given, general anesthesia was induced with midazolam, fentanyl, and rocuronium, and maintained with midazolam, fentanyl, remifentanil, and rocuronium. The operation was performed without any complications. Dexmedetomidine and fentanyl were continuously infused after the operation until she was extubated in the morning of postoperative day 1. Fatal arrhythmia due to perioperative stress did not occur. CONCLUSIONS: We report the anesthetic management of a child with CPVT who underwent insertion of an ICD. CPVT-induced fatal arrhythmia did not occur perioperatively by carefully avoiding perioperative stress with premedication and post-operative sedation.
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Omiya et al. (2020) studied this question.
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