Sydney Medical School, University of Sydney, Sydney, NSW, Australia.
Respiratory Department, Sunshine Coast University Hospital, Sunshine Coast Region, QLD, Australia.
Respirology. 2020 Aug;25(8):863-871. doi: 10.1111/resp.13768. Epub 2020 Jan 30.
Early diagnosis of PAH is clinically challenging. Patterns of diagnostic delay in Australian and New Zealand PAH populations have not been explored in large-scale studies. We aimed to evaluate the magnitude, risk factors and survival impact of diagnostic delay in Australian and New Zealand PAH patients.
A cohort study of PAH patients from the PHSANZ Registry diagnosed from 2004 to 2017 was performed. Diagnostic interval was the time from symptom onset to diagnostic right heart catheterization as recorded in the registry. Factors associated with diagnostic delay were analysed in a multivariate logistic regression model. Survival rates were compared across patients based on the time to diagnosis using Kaplan-Meier method and Cox regression.
A total of 2044 patients were included in analysis. At diagnosis, median age was 58 years (IQR: 43-69), female-to-male ratio was 2.8:1 and majority of patients were in NYHA FC III-IV (82%). Median diagnostic interval was 1.2 years (IQR: 0.6-2.7). Age, CHD-PAH, obstructive sleep apnoea and peripheral vascular disease were independently associated with diagnostic interval of ≥1 year. No improvement in diagnostic interval was seen during the study period. Longer diagnostic interval was associated with decreased 5-year survival.
PAH patients experience significant diagnostic interval, which has not improved despite increased community awareness. Age, cardiovascular and respiratory comorbidities are significantly associated with longer time to diagnosis. Mortality rates appear higher in patients who experience longer diagnostic interval.
肺动脉高压（PAH）的早期诊断具有临床挑战性。在大规模研究中，尚未探讨澳大利亚和新西兰 PAH 人群的诊断延迟模式。我们旨在评估澳大利亚和新西兰 PAH 患者诊断延迟的程度、危险因素和对生存的影响。
对 2004 年至 2017 年期间 PHSANZ 登记处诊断的 PAH 患者进行了队列研究。诊断间隔是从症状发作到登记处记录的诊断右心导管检查的时间。使用多元逻辑回归模型分析与诊断延迟相关的因素。使用 Kaplan-Meier 方法和 Cox 回归比较基于诊断时间的患者生存率。
共纳入 2044 例患者进行分析。在诊断时，中位年龄为 58 岁（IQR：43-69），男女比例为 2.8:1，大多数患者处于纽约心脏协会功能分级（NYHA）III-IV 级（82%）。中位诊断间隔为 1.2 年（IQR：0.6-2.7）。年龄、先心病相关 PAH、阻塞性睡眠呼吸暂停和外周血管疾病与诊断间隔≥1 年独立相关。在研究期间，诊断间隔没有改善。诊断间隔较长与 5 年生存率降低相关。
PAH 患者经历了显著的诊断间隔，尽管社区意识增强，但间隔时间并未改善。年龄、心血管和呼吸系统合并症与诊断时间延长显著相关。在经历较长诊断间隔的患者中，死亡率似乎更高。
