Department of Cardiology, Herlev-Gentofte Hospital, University Hospital of Copenhagen, Copenhagen, Denmark.
Department of Cardiology, Rigshospitalet, University Hospital Copenhagen, Copenhagen, Denmark.
Open Heart. 2019 Oct 28;6(2):e001161. doi: 10.1136/openhrt-2019-001161. eCollection 2019.
Studies have suggested a shared genetic aetiology between congenital long QT syndrome (LQTS) and diabetes, epilepsy and mental disorders. We investigated the prevalence of metabolic, neurological and psychiatric comorbidities in LQTS patients.
This retrospective cohort study was based on data from nationwide Danish registries, 2003-2017. LQTS patients were matched 1:5 with controls on sex and age.
We matched 463 LQTS patients with 2315 controls from the background population. Mean age was 35.7 (SD 21.0) years, and 38% were males in both groups. LQTS patients had a higher prevalence of atrial fibrillation (6.5% vs 2.3%, p<0.001), diabetes (3.7% vs 1.8 %, p=0.011) and hearing loss (3.2% vs 1.7%, p=0.027). LQTS patients had a higher prevalence of psychiatric disorders overall (13.0% vs 9.1%, p=0.01) but the difference could not be attributed to a specific psychiatric disease subgroup. LQTS patients had a higher prevalence of neurological disorders (22.0% vs 13.2%, p<0.001), largely driven by epilepsy (6.7% vs 1.6%, p<0.001). In 20/27 (74%) of the LQTS patients, the epilepsy diagnosis did not reappear in the registries after the LQTS diagnosis was established.
In this nationwide cohort, patients with LQTS had a significantly increased burden of diabetes, neurological and psychiatric comorbidities, compared with the background population. The higher prevalence of neurological comorbidities was largely driven by epilepsy, despite a high rate of potentially misdiagnosed patients prior to LQTS diagnosis. Our data support that LQTS may be considered a multiorgan disease and suggest that patient management should be adjusted accordingly.
研究表明先天性长 QT 综合征(LQTS)与糖尿病、癫痫和精神障碍之间存在共同的遗传病因。我们调查了 LQTS 患者的代谢、神经和精神共病的患病率。
这是一项基于 2003 年至 2017 年全国丹麦登记处数据的回顾性队列研究。LQTS 患者按性别和年龄与对照组 1:5 匹配。
我们将 463 名 LQTS 患者与来自背景人群的 2315 名对照者进行匹配。两组的平均年龄为 35.7(21.0)岁,男性比例分别为 38%。LQTS 患者心房颤动的患病率更高(6.5% vs. 2.3%,p<0.001)、糖尿病(3.7% vs. 1.8%,p=0.011)和听力损失(3.2% vs. 1.7%,p=0.027)。总体而言,LQTS 患者的精神障碍患病率更高(13.0% vs. 9.1%,p=0.01),但差异不能归因于特定的精神疾病亚组。LQTS 患者的神经障碍患病率更高(22.0% vs. 13.2%,p<0.001),主要由癫痫(6.7% vs. 1.6%,p<0.001)引起。在 27 例 LQTS 患者中,有 20 例(74%)的癫痫诊断在 LQTS 诊断确立后不再出现在登记处。
在这项全国性队列研究中,与背景人群相比,LQTS 患者的糖尿病、神经和精神共病负担显著增加。神经共病患病率较高主要是由癫痫引起的,尽管在 LQTS 诊断之前,存在很高的潜在误诊患者比例。我们的数据支持 LQTS 可能被视为一种多器官疾病,并表明应相应调整患者管理。