Effect of off-label targeted drugs on long-term survival in chronic thromboembolic pulmonary hypertension: Insights from a national multicentre prospective registry
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View abstract on PubMed
Summary
This summary is machine-generated.Off-label pulmonary arterial hypertension (PAH)-targeted drugs significantly improve long-term survival in chronic thromboembolic pulmonary hypertension (CTEPH) patients. Early initiation of these PAH drugs is associated with better outcomes compared to no treatment.
Area Of Science
- Cardiology
- Pulmonology
- Pharmacology
Background
- Pulmonary arterial hypertension (PAH)-targeted drugs are frequently used off-label for non-operated chronic thromboembolic pulmonary hypertension (CTEPH).
- The long-term prognostic impact of these off-label drug regimens in CTEPH patients remains largely uncharacterized.
Purpose Of The Study
- To investigate the effect of off-label PAH-targeted drugs on the long-term survival of CTEPH patients.
- To compare survival rates between CTEPH patients receiving off-label PAH drugs and those who do not.
Main Methods
- A prospective, multicentre national registry enrolled CTEPH patients.
- Survival analysis was performed comparing patients receiving off-label PAH drugs (initial or subsequent treatment) versus untreated controls.
- Propensity score-matching (PSM) was utilized to control for confounding factors.
Main Results
- Patients treated with off-label PAH drugs demonstrated significantly higher 1-, 2-, 3-, and 5-year survival rates compared to controls (71.1% vs. 55.3% at 5 years, p=0.005).
- Initial treatment with off-label drugs correlated with better 5-year survival (HR: 0.611, p=0.025) after accounting for immortal-time bias.
- In PSM samples, initial treatment significantly improved 5-year survival versus untreated patients (68.9% vs. 49.3%, p=0.008).
Conclusions
- Off-label PAH-targeted drugs contribute to improved long-term survival in CTEPH patients undergoing pharmacotherapy.
- Early initiation of these therapies appears to be a key factor in enhancing patient prognosis.
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