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Published on: March 17, 2022
Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control
Irene M Lang1, Gérald Simonneau, Joanna W Pepke-Zaba
1Irene Lang, Department of Cardiology, Medical University of Vienna, Währinger Gürtel 18 - 20, 1090 Vienna, Austria. irene.lang@meduniwien.ac.at
Insights
Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary hypertension (IPAH) share symptoms, but risk factors differ. History of venous thromboembolism suggests CTEPH, while diabetes and higher pulmonary artery pressure suggest IPAH. Operability depends on age, lesion location, and pulmonary vascular resistance.
Area of Science:
- Cardiology
- Pulmonary Medicine
- Vascular Medicine
Background:
- Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary hypertension (IPAH) present similarly, necessitating differential diagnosis.
- Identifying operable CTEPH patients is crucial for treatment selection.
Purpose of the Study:
- Investigate risk factors differentiating CTEPH from IPAH.
- Determine criteria for selecting operable CTEPH patients.
Main Methods:
- Case-control study involving 436 CTEPH and 158 IPAH patients across eight European centers (2006-2010).
- Analyzed risk factors for CTEPH and IPAH, and factors associated with CTEPH operability.
Main Results:
- CTEPH risk factors: history of acute venous thromboembolism, large pulmonary embolism, non-O blood groups, older age.
- IPAH risk factors: diabetes mellitus, female gender, higher mean pulmonary artery pressure.
- Operable CTEPH criteria: younger age, proximal lesions, pulmonary vascular resistance < 1200 dyn.s.cm⁻⁵.
- Non-operable CTEPH patients showed more overlap in risk factors with IPAH patients than operable CTEPH patients.
Conclusions:
- Confirmed CTEPH association with venous thromboembolism history and non-O blood groups.
- Identified diabetes mellitus and higher mean pulmonary artery pressure as indicators for IPAH diagnosis.
- Non-operable CTEPH shares more risk factor similarities with IPAH than operable CTEPH.
Abstract:
Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary hypertension (IPAH) share a similar clinical presentation, and a differential diagnosis requires a thorough workup. Once CTEPH is confirmed, patients who can be safely operated have to be identified. We investigated risk factors associated with CTEPH and IPAH, and the criteria for the selection of operable CTEPH patients. This case-control study included 436 consecutive patients with CTEPH and 158 with IPAH in eight European centres, between 2006 and 2010. Conditions identified as risk factors for CTEPH included history of acute venous thromboembolism (p < 0.0001), large size of previous pulmonary embolism (p = 0.0040 in univariate analysis), blood groups non-O (p < 0.0001 in univariate analysis), and older age (p = 0.0198), whereas diabetes mellitus (p = 0.0006), female gender (p = 0.0197) and higher mean pulmonary artery pressure (p = 0.0103) were associated with increased likelihood for an IPAH diagnosis. Operability of CTEPH patients was associated with younger age (p = 0.0108), proximal lesions (p ≤ 0.0001), and pulmonary vascular resistance below 1200 dyn.s.cm⁻⁵ (p = 0.0080). Non-operable CTEPH patients tended to be less differentiable from IPAH patients by risk factor analysis than operable patients. This study confirmed the association of CTEPH with history of acute venous thromboembolism and blood groups non-O, and identified diabetes mellitus and higher mean pulmonary artery pressure as factors suggesting an IPAH diagnosis. Non-operable CTEPH is more similar to IPAH than operable CTEPH regarding risk factors.
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