Chronic thromboembolic pulmonary hypertension
ORPHA:70591· ICD-10 I26.0
Definition
A rare complication of acute pulmonary embolism (PE), either symptomatic or not, that is characterized by fibrotic intravascular material occlusion of pulmonary arteries in combination with a secondary microvasculopathy of vessels less than 500 µm. The consequence is an increase in pulmonary vascular resistance (PVR) and progressive right heart failure.
- Prevalence
- Unknown
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly