| Literature DB >> 30998690 |
V Cottin1, D Avot2, L Lévy-Bachelot2, C A Baxter3, D R Ramey4, L Catella5, S Bénard5, O Sitbon6, S Teal7.
Abstract
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.Entities:
Mesh:
Year: 2019 PMID: 30998690 PMCID: PMC6472741 DOI: 10.1371/journal.pone.0214649
Source DB: PubMed Journal: PLoS One ISSN: 1932-6203 Impact factor: 3.240
Fig 1Flow diagram of the refinement step.
Performance of tested case ascertainment algorithms on training and validation sets (medical charts records as the reference standard).
| True-positive/ | True-negative/ | Sensitivity (%) | Specificity (%) | PPV (%) | NPV (%) | |
|---|---|---|---|---|---|---|
| 1st algorithm ( | 132/123 | 17/750 | 88.6 (83.5–93.7) | 85.9 (83.6–88.2) | 51.8 (45.6–57.9) | 97.8 (96.7–98.8) |
| 2nd algorithm | 121/102 | 28/771 | 81.2 (74.9–87.5) | 88.3 (86.2–90.4) | 54.3 (47.7–60.8) | 96.5 (95.2–97.8) |
| 3rd algorithm | 128/120 | 21/753 | 85.9 (80.3–91.4) | 86.3 (83.4–88.5) | 51.6 (45.4–57.8) | 97.3 (96.1–98.4) |
| 4th algorithm | 117/99 | 32/774 | 78.5 (71.9–85.1) | 88.7 (86.6–90.7) | 54.2 (47.5–60.8) | 96.0 (94.7–97.4) |
| 5th algorithm | 101/78 | 48/795 | 67.8 (60.2–75.2) | 91.1 (89.1–93.0) | 56.4 (49.2–63.7) | 94.3 (92.7–95.9) |
| 6th algorithm | 129/75 | 20/798 | 86.6 (81.1–92.1) | 91.4 (89.6–93.3) | 63.2 (56.7–69.9) | 97.6 (96.5–98.1) |
CI, Confidence Interval; NPV, Negative Predictive Value; PPV, Positive Predictive Value; RHC, right heart catheterization
* First/preliminary algorithm: ≥1 PH diagnosis during one year (2015) and any of the following criteria over 2009–2015: (i) history of PEA (medical procedures-CCAM code) or BPA (ICD-10 code of PH and a CCAM code of BPA), (ii) hospital stay for PH and PE, (iii) history of ≥3 PE, (iv) CTEPH care pathway: hospital stay for PE, then for PH in competence centre, then for PH in a reference centre, (v) history of PE and 1 RHC.
Fig 2Selection using the final case ascertainment algorithm.
Annual number of hospitalized patients with CTEPH in France before and after adjustment according to the algorithm performance.
| Patients | Low CI | High CI | |
|---|---|---|---|
| 5,405 | — | — | |
| Number of false positives subtracted | 3,197 | 3,737 | 2,657 |
| Number of false negatives added | 930 | 1,199 | 581 |
| 3,138 | 2,867 | 3,330 | |
| 47 | 43 | 50 |
CI, Confidence Interval; CTEPH, Chronic thromboembolic pulmonary hypertension
aLow CI estimate: use the lower limit of the 95% confidence interval (CI) of the performance indicators (Se, PPV) for computing the number of false positives to subtract and the number of false negatives to add.
bHigh CI estimate: use the upper limit of the 95% confidence interval of the performance indicators (Se, PPV) for computing the number of false positives to subtract and the number of false negatives to add.
c with a denominator of 66.381 million for the French population in 2015