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什么是CPVT?

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac condition that causes sudden rhythm disturbances, calledarrhythmias,在其他健康的孩子中。这些异常的节奏通常发生在肾上腺素水平高的时候,例如运动或强烈的情绪。

CPVT is a rare disorder that affects about one in 10,000 people. Left untreated, CPVT may cause the heart to stop beating.

What are the symptoms of CPVT?

心律不齐通常发生在运动或情绪压力期间。它可能引起快速心跳(pal),晕厥(晕厥)或心脏骤停。

CPVT的原因是什么?

CPVT是由特定心脏蛋白的遗传密码变化引起的,这会导致钙泄漏到心脏中。额外的钙会影响心脏的电气系统,从而引起心脏下部腔室的额外心跳。

How we care for CPVT

波士顿儿童医院的心脏病专家拥有多年治疗CP欧宝彩票平台VT的经验,以及其他类型的复杂心律失常。这遗传心律失常计划at Boston Children’s specializes in family-based services for children with CPVT, including counseling.

家庭心律不齐诊所meets with and assesses entire families all at once, usually in one day. Our specialists use clinical and genetic testing to identify if any family member is at risk of developing the condition.

儿茶酚胺能多态性心室心动过速|Diagnosis & Treatments

How is CPVT diagnosed?

任何小孩或年轻人儿茶酚的症状aminergic polymorphic ventricular tachycardia (CPVT) during periods of exertion or strong emotion should be evaluated by an experienced team.

Because CPVT is only observable during periods of adrenaline, standard electrocardiograms and echocardiograms are not good diagnostic tests. Instead, if CPVT is suspected, your child will have anexercise stress test。该测试利用跑步机或自行车,并在运动过程中记录心脏的功能。年轻的患者太小,无法使用跑步机Holter Monitor

运动胁迫测试可以揭示心室(心室肾上腺)的额外节拍模式,随着肾上腺素释放的增加,它们变得越来越频繁且更加复杂。这些额外的节拍通常会发展为称为“双向”或“多态性心室心动过速”的复杂心律不齐。

基因检测也是CPVT总体诊断工作的重要组成部分。这通常需要专家解释,通常是在全户家庭评估的背景下。

CPVT有哪些治疗选择?

CPVT有几种治疗方法。您孩子的确切治疗方法将基于心律不齐的率或严重程度。

CPVT的药物

许多形式都可以用药物治疗,包括β受体阻滞剂和抗心律失常。β受体阻滞剂通过阻断β受体并防止肾上腺素影响心脏而起作用。这有助于减慢心律。

A common antiarrhythmic medication used for CPVT is flecainide, which works by affecting the sodium and calcium exchange within the cardiac cell. While very effective in some people with CPVT, it does not work for everyone.

Implantable cardioverter defibrillators

植入式心脏扭曲器除颤器是经常在被认为是高风险的患者中使用的设备,或者在其他治疗后继续出现症状的设备。他们可以检测心室心律不齐,并通过给出精确的电脉冲来恢复正常心律。

Left cardiac sympathetic denervation

当医疗治疗无效以阻止心律不齐时,您的孩子的医生可能会建议一日外科手术,称为左心交感神经去神经(交感神经切除术)。在此过程中,外科医生将神经划分为心脏。这可以防止肾上腺素刺激心脏并引起心律不齐。

儿茶酚胺能多态性心室心动过速|程序和服务

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