DISEASE SCANNER
Global Incurable Diseases Tracker
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Pulmonary Disease
Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
HIGH SEVERITY
A form of pulmonary hypertension caused by chronic obstruction of pulmonary arteries due to organized thrombi. Develops in 2-4% of patients after acute pulmonary embolism. Potentially curable with pulmonary endarterectomy.
Global Affected
500.0K
Countries
16
Symptoms
Progressive dyspnea
Exercise intolerance
Fatigue
Syncope
Chest pain
Edema
Hemoptysis
Right heart failure
Treatment Options
Pulmonary endarterectomy (surgical cure)
Balloon pulmonary angioplasty
Riociguat
Anticoagulation (lifelong)
Oxygen therapy
Diuretics
Lung transplantation
Risk Factors
1History of acute pulmonary embolism
2Recurrent DVT/PE
3Hypercoagulable states
4Asplenia
5Chronic inflammatory conditions
6Thyroid replacement therapy
Diagnostic Methods
- 1Echocardiogram
- 2V/Q scan (screening)
- 3CT pulmonary angiography
- 4Right heart catheterization
- 5Pulmonary angiography
- 6Pulmonary function tests
Prognosis
Good with surgical treatment; inoperable disease has 5-year survival ~70%. Medical therapy and BPA improving outcomes.
Prevention
- Anticoagulation after PE
- Early diagnosis
- Referral to CTEPH center
- Prophylaxis in high-risk patients
Research Status
Pulmonary endarterectomy (surgical cure) and Balloon pulmonary angioplasty are primary treatments. Research focuses on improved therapies and outcomes.
Affected Countries
Sources
- https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-hypertension
- https://www.ncbi.nlm.nih.gov/books/NBK540989
- https://www.hematology.org/education/patients
- https://www.ncbi.nlm.nih.gov/books
- https://rarediseases.org/rare-diseases
Medical Disclaimer
This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.