10-year survival of pulmonary arterial hypertension associated with connective tissue disease: insights from a multicentre PAH registry
Authors' conclusion
The 10-year survival rate of CTD-PAH patients was reported for the first time. The 10-year prognosis was poor, but there was a tendency for more standardized treatment and better survival in patients enrolled after 2015. The recommended four-strata model at follow-up can effectively predict 10-year survival in CTD-PAH patients.
Study context
- Population
- Chinese patients with PAH associated with connective tissue disease (CTD-PAH), national multicentre registry, 2009 to 2019median follow-up 4.85 years
- Participants
- 266 patients
- Exposure
- Patients enrolled 2009-2014 vs 2015-2019
- Outcome
- 10-year survival rate
- Follow-up
- 10 years
Methodological detailsmulticentre, national registry
- Multicentre
- National registry
Main finding
As reported in the abstract
10-year survival rate, all CTD-PAH patients
59.9%
Observational data: associations, not effects.
Further findings
PAH-targeted combination therapy
Binary therapy (both CTD and PAH-targeted therapy)
1-year survival rate
P > 0.05
3-year survival rate
P > 0.05
Four-strata model at follow-up predicting 10-year survival
- C-index
- 0.742
Original publication
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