تتطلب إدارة تسرع القلب البطيني متعدد الأشكال الكاتيكولاميني (CPVT) لدى الأفراد الحاملين لطفرة مسببة للمرض مؤكدة، والذين لم تظهر عليهم أعراض قط ولم يُوثَّق لديهم اضطراب نظم أو توقف قلبي مُنقذ، نهجاً دقيقاً ومعايَراً بعناية — يختلف عن الإدارة في حالات المرض المعبَّر عنها ظاهرياً.
DOI: 10.1093/eurheartj/ehac262
It is recommended that CPVT is diagnosed in patients who are carriers of a mutation in disease-causing genes.
Therapy with beta-blockers should be considered for genetically positive CPVT patients without phenotype.
This panel has confirmed the indication to treat genetically positive family members with beta-blockers, even in the absence of documented exercise- or stress-induced VAs.
ICD implantation should be considered in patients with CPVT who experience arrhythmogenic syncope and/or documented bidirectional/PVT while on highest tolerated beta-blocker dose and on flecainide.
LCSD should be considered in patients with diagnosis of CPVT when the combination of beta-blockers and flecainide at therapeutic dosage are either not effective, not tolerated, or contraindicated.
View source ↗