There is a disproportionately high rate of hereditary long QT syndrome (LQTS) in Northern British Columbia First Nations people, largely due to a novel missense variant in KCNQ1 (p.V205M). The variant has been previously described predisposing those affected to syncope, arrhythmia, and sudden death. Although the biological aspects of LQTS have been explored extensively, less research has been done into the impact of living with a genetic variant that predisposes one to sudden death, and no previous studies have provided cultural insights from a First Nations community. The goal of this study was to explore what facilitates and hinders resiliency and coping for those living with LQTS. Participants were invited to partake in their choice of one‐to‐one interviews, Photovoice, and Talking Circles. This paper presents the findings from the interview portion of the study. Interviews were recorded, transcribed, and analyzed qualitatively using the systematic text condensation method. Ten women shared their personal experiences of living with LQTS through individual interviews. Half of the women had tested positive for the p.V205M variant, and the other half were awaiting results. In general, learning about a LQTS diagnosis was perceived as traumatic, with gradual acceptance that led to coping. The main factors found to facilitate resiliency and coping were positive family relationships, spirituality, and knowledge about LQTS. The main factors found to hinder resiliency and coping were a poor understanding of the biological or clinical aspects of LQTS, conflicting medical advice (especially regarding physical activity) and LQTS not being taken seriously by social contacts and healthcare providers. It appears that learning to live with LQTS is an ongoing process, requiring balance and interconnectedness between all aspects of well‐being.

中文翻译：

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了解长期QT综合征对个人和社区的影响：来自Gitxsan妇女的观点。

在不列颠哥伦比亚省北部原住民中，遗传性长QT综合征（LQTS）的发病率异常高，这主要是由于KCNQ1中出现了一种新型的错义变异（第V205M页）。先前已经描述了该变体，使易患晕厥，心律不齐和猝死的人易变。尽管已经对LQTS的生物学方面进行了广泛的研究，但对于易患猝死的遗传变异所产生的影响的研究很少，而且以前的研究都未提供原住民社区的文化见解。这项研究的目的是探讨哪些因素可以促进和阻碍LQTS患者的适应力和应对能力。邀请参与者参加一对一访谈，Photovoice和Talking Circles的选择。本文介绍了研究访谈部分的发现。使用系统的文本压缩方法，对访谈进行记录，记录和定性分析。十名妇女通过个人访谈分享了与LQTS一起生活的个人经历。一半的女性对p.V205M变体测试呈阳性，另一半正在等待结果。通常，了解LQTS诊断被认为是创伤性的，逐渐被接受导致应对。发现有助于恢复能力和应对力的主要因素是积极的家庭关系，灵性和对LQTS的了解。发现阻碍弹性和应对力的主要因素是对LQTS的生物学或临床方面的了解不足，医学建议相抵触（尤其是关于身体活动）以及社会接触者和医疗保健提供者未认真对待LQTS。似乎学习使用LQTS是一个持续的过程，