National Reference Centre for rare pulmonary diseases, Competence centre for pulmonary arterial hypertension, Louis Pradel hospital, Claude Bernard University Lyon 1, UMR 754, Lyon, France.
MSD France, Courbevoie, France.
PLoS One. 2019 Apr 18;14(4):e0214649. doi: 10.1371/journal.pone.0214649. eCollection 2019.
Chronic thromboembolic pulmonary hypertension (CTEPH), a rare pulmonary vascular disease, is often misdiagnosed due to nonspecific symptoms. The objective of the study was to develop, refine and validate a case ascertainment algorithm to identify CTEPH patients within the French exhaustive hospital discharge database (PMSI), and to use it to estimate the annual number of hospitalized patients with CTEPH in France in 2015, as a proxy for disease prevalence. As ICD-10 coding specifically for CTEPH was not available at the time of the study, a case ascertainment algorithm was developed in close collaboration with an expert committee, using a two-step process (refinement and validation), based on matched data from PMSI and hospital medical records from 2 centres. The best-performing algorithm (specificity 95%, sensitivity 70%) consisted of ≥1 pulmonary hypertension (PH) diagnosis during 2015 and any of the following criteria over 2009-2015: (i) CTEPH interventional procedure, (ii) admission for PH and pulmonary embolism (PE), (iii) PE followed by hospitalization in competence centre then in reference centre, (iv) history of PE and right heart catheterization. Patients with conditions suggestive of pulmonary arterial hypertension were excluded. A total of 3,138 patients hospitalized for CTEPH was estimated for 2015 (47 cases/million, range 43 to 50 cases/million). Assuming that patients are hospitalized at least once a year, the present study provides an estimate of the minimal prevalence of CTEPH and confirms the heavy burden of this disease.
慢性血栓栓塞性肺动脉高压（CTEPH）是一种罕见的肺血管疾病，由于其非特异性症状，常常被误诊。本研究的目的是开发、完善和验证一种病例确定算法，以在法国详尽的医院出院数据库（PMSI）中确定 CTEPH 患者，并使用该算法来估计 2015 年法国每年因 CTEPH 住院的患者人数，以此作为疾病流行率的替代指标。由于在研究时 ICD-10 编码没有专门针对 CTEPH，因此与一个专家委员会合作，使用基于 PMSI 和来自 2 个中心的医院病历的匹配数据的两步法（完善和验证），开发了一种病例确定算法。表现最佳的算法（特异性 95%，敏感性 70%）包括在 2015 年有≥1 个肺动脉高压（PH）诊断和以下任何标准在 2009-2015 年期间至少出现过 1 次：（i）CTEPH 介入程序，（ii）因 PH 和肺栓塞（PE）入院，（iii）PE 后在能力中心住院然后在参考中心住院，（iv）PE 病史和右心导管检查。排除了疑似肺动脉高压的患者。估计 2015 年有 3138 例 CTEPH 住院患者（47 例/百万，范围 43 至 50 例/百万）。假设患者每年至少住院一次，本研究提供了 CTEPH 最小流行率的估计值，并证实了该疾病的沉重负担。
