Background and objectives During the last decades, the survival rates in patients with congenital heart disease have increased dramatically, particularly in patients with complex heart malformations. However, the survival in patients with simple defects is still unknown. We aimed to determine the characteristics and the risk of mortality in patients with isolated pulmonary valve stenosis (PS). Methods Swedish inpatient, outpatient and cause of death registries were used to identify patients born between 1970 and 2017 with a diagnosis of PS, without any other concomitant congenital heart lesion. For each patient with PS, 10 control individuals without congenital heart disease were matched by birth year and sex from the total population registry. We used median-unbiased method and Kaplan-Meier survival analysis to examine the risk of mortality. Results We included 3910 patients with PS and 38 770 matched controls. The median age of diagnosis of PS was 0.7 years (IQR 0.3–7.0). During a median follow-up of 13.5 years (IQR 6.5–23.5), 88 patients with PS and 192 controls died; 500 patients with PS (12%) underwent at least one transcatheter or surgical valve intervention. The overall mortality rate was significantly higher in patients with PS compared with matched controls (HR 4.67, 95% CI 3.61 to 5.99, p=0.001). Patients with an early diagnosis of PS (0–1 year) had the highest risk of mortality (HR 10.99, 95% CI 7.84 to 15.45). Conclusions In this nationwide, register-based cohort study, we found that the risk of mortality in patients with PS is almost five times higher compared with matched controls. Patients with an early diagnosis of PS appears to be the most vulnerable group and the regular follow-up in tertiary congenital heart units may be the key to prevention. Data are available on reasonable request. All data relevant to the study are included in the article or uploaded as online supplemental information.

中文翻译：

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孤立性肺动脉瓣狭窄患者的长期生存：一种不那么良性的疾病？

背景和目标 在过去的几十年里，先天性心脏病患者的存活率急剧增加，特别是在患有复杂心脏畸形的患者中。然而，单纯缺陷患者的生存率仍然未知。我们旨在确定孤立性肺动脉瓣狭窄 (PS) 患者的特征和死亡风险。方法 瑞典住院、门诊和死因登记系统用于识别出生于 1970 年至 2017 年之间且被诊断为 PS 且没有任何其他先天性心脏病的患者。对于每个患有 PS 的患者，10 名没有先天性心脏病的对照个体按出生年份和来自总人口登记的性别进行匹配。我们使用中值无偏方法和 Kaplan-Meier 生存分析来检查死亡风险。结果 我们纳入了 3910 名 PS 患者和 38 770 名匹配的对照。PS 诊断的中位年龄为 0.7 岁（IQR 0.3-7.0）。在中位随访 13.5 年（IQR 6.5-23.5）期间，88 名 PS 患者和 192 名对照组死亡；500 名 PS 患者 (12%) 接受了至少一次经导管或外科瓣膜干预。与匹配的对照组相比，PS 患者的总体死亡率显着更高（HR 4.67，95% CI 3.61 至 5.99，p=0.001）。早期诊断为 PS（0-1 年）的患者死亡风险最高（HR 10.99，95% CI 7.84 至 15.45）。结论 在这项全国性的、基于注册的队列研究中，我们发现 PS 患者的死亡风险几乎是匹配对照组的 5 倍。早期诊断为 PS 的患者似乎是最脆弱的群体，在三级先心病单位定期随访可能是预防的关键。可应合理要求提供数据。与研究相关的所有数据都包含在文章中或作为在线补充信息上传。