Chronic thromboembolic pulmonary hypertension

Orpha code: 70591OMIM code: 612862

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.

Disease data
Classification

Disease

Synonyms
CTEPH
CTEPH
ORPHA code
70591
OMIM code
612862
ICD10 code
-
ICD11 code
BB01.3

No additional description.

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