Trans‐2,3‐enoyl‐CoA reductase‐like‐related catecholaminergic polymorphic ventricular tachycardia with regular ventricular tachycardia and response to flecainide

Abstract

IntroductionWe describe a unique case of TECRL‐CPVT presented with cardiac arrest.MethodsPost resuscitation, the patient developed regular ventricular tachycardia featuring a left purkinje system morphology.ResultsThere was clear suppression of arrhythmia with the addition of flecainide and isolated ventricular ectopy causing secondary T‐wave changes.ConclusionA high index of suspicion was required to eventually make the diagnosis through whole exome sequencing.

Novel variants inTECRLleading to catecholaminergic polymorphic ventricular tachycardia

“…Two recent case reports describe similar pathogenic variants in the TECRL gene leading to an eventual diagnosis of CPVT. First, Ebrahim et al (2023) report a case of an 11-yr-old boy with cardiac arrest, subsequent prolonged QTc after return of spontaneous circulation, and inducible unstable polymorphic VT with exercise in the electrophysiology laboratory. Whole-exome sequencing was notable for a previously described homozygous TECRL splice-site variant c.331+1G>A ( Ebrahim et al, 2023 ).…”

“…First, Ebrahim et al (2023) report a case of an 11-yr-old boy with cardiac arrest, subsequent prolonged QTc after return of spontaneous circulation, and inducible unstable polymorphic VT with exercise in the electrophysiology laboratory. Whole-exome sequencing was notable for a previously described homozygous TECRL splice-site variant c.331+1G>A ( Ebrahim et al, 2023 ). Secondly, two affected Lebanese siblings experienced exercise-induced symptoms with an ECG notable for abnormal QT prolongation, abnormal T-wave morphology, and bidirectional ventricular ectopic beats during an exercise stress test.…”

Novel Compound Heterozygous Variants in Trans-2,3-Enoyl-Coenzyme A Reductase-Like Gene Associated With Catecholaminergic Polymorphic Ventricular Tachycardia

“…The patient has been free of symptoms since starting combination therapy with a β-blocker and flecainide, which is consistent with those previously reported cases. 5 , 10 …”

Advancements in the diagnosis and management of premature ventricular contractions in pediatric patients

“…However, the long-term efficacy and safety of propafenone require careful consideration, as treatment response and patient compliance can vary. Antiarrhythmic medications, including Class I agents like flecainide, ivabradine, or Class III agents such as amiodarone, may be reserved for more complex or therapy-resistant PVCs due to their proarrhythmic potential and side effect profile ( 46 , 47 ). The use of these agents necessitates close monitoring for efficacy and toxicity.…”