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 , as a proxy for disease prevalence.
As ICD 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. Patients with conditions suggestive of pulmonary arterial hypertension were excluded. 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.
This is an open access article distributed under the terms of the Creative Commons Attribution License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Data Availability: Data cannot be shared publicly because they contain potentially identifying information.
Data are available from the French technical agency for information on hospitalization ATIH after agreement of the French data protection authority, CNIL, to institutions who meet the criteria for access to confidential data.
The funders had no role in data collection and analysis, but participated in the study design, interpretation of the results, preparation of the manuscript and decision to publish.
