Zicans Matiss, Sablinskis Kristaps, Cesnieks Haralds, Rudzitis Ainars, Kaulins Ricards, Skride Andris
Riga Stradins University Riga Latvia.
University of Latvia Riga Latvia.
Pulm Circ. 2025 Sep 3;15(3):e70159. doi: 10.1002/pul2.70159. eCollection 2025 Jul.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious disease caused by persistent thromboembolic obstruction or narrowing of the pulmonary arteries. Its prevalence varies, and many cases remain undiagnosed, contributing to a substantial clinical burden. This study aimed to summarize all CTEPH cases diagnosed in Latvia in 2024, calculate the annual incidence, and present additional epidemiological data from Latvian pulmonary hypertension (PH) registry. Additionally, we sought to document the presence of risk factors in individual patients and analyze their diagnostic pathways leading to a diagnosis of CTEPH. This observational study aimed to analyze diagnostic pathways and identify risk factors in all patients diagnosed with CTEPH in Latvia in 2024. Diagnosis was confirmed at Pauls Stradins Clinical University Hospital according to ESC guideline criteria. Patients completed the emPHasis-10 questionnaire and underwent a structured interview. In 2024, 15 patients were diagnosed with CTEPH in Latvia, corresponding to an annual incidence of 8.01 cases per million. Prevalence was 31.51 per million, and mortality-3.74 per 100 person-years. Patients diagnosed within 2 years of symptom onset had lower pulmonary vascular resistance compared to those diagnosed later. The most common risk factor was prior acute pulmonary embolism (80%). In 60% of cases, diagnosis was delayed by more than 2 years, reflecting late referral to a PH centre. CTEPH remains frequently misdiagnosed and undertreated. Improved recognition of risk factors and stronger collaboration with specialized PH centres are essential to optimize patient management and outcomes.
慢性血栓栓塞性肺动脉高压(CTEPH)是一种罕见但严重的疾病,由肺动脉持续的血栓栓塞性阻塞或狭窄引起。其患病率各不相同,许多病例仍未得到诊断,导致了沉重的临床负担。本研究旨在总结2024年在拉脱维亚诊断出的所有CTEPH病例,计算年发病率,并展示来自拉脱维亚肺动脉高压(PH)登记处的其他流行病学数据。此外,我们试图记录个体患者中危险因素的存在情况,并分析他们导致CTEPH诊断的诊断途径。这项观察性研究旨在分析2024年在拉脱维亚所有被诊断为CTEPH的患者的诊断途径并识别危险因素。根据欧洲心脏病学会(ESC)指南标准,在保罗·斯特拉迪恩斯临床大学医院确诊。患者完成了emPHasis - 10问卷并接受了结构化访谈。2024年,拉脱维亚有15名患者被诊断为CTEPH,年发病率为每百万人口8.01例。患病率为每百万人口31.51例,死亡率为每100人年3.74例。症状出现后2年内确诊的患者与之后确诊的患者相比,肺血管阻力较低。最常见的危险因素是既往急性肺栓塞(80%)。在60%的病例中,诊断延迟超过2年,这反映了向PH中心转诊较晚。CTEPH仍然经常被误诊和治疗不足。提高对危险因素的认识以及加强与专业PH中心的合作对于优化患者管理和改善预后至关重要。