Idiopathic/heritable pulmonary arterial hypertension

Orpha code: 422OMIM code: 615344

Definition

A form of pulmonary arterial hypertension (PAH) characterized by elevated pulmonary arterial resistance leading to right heart failure; it is progressive and potentially fatal. The majority cases have an identifiable genetic cause, but a significant proportion are idiopathic.

Disease data
Classification

Disease

Synonyms
Idiopathic and/or familial pulmonary arterial hypertension
ORPHA code
422
OMIM code
615344
ICD10 code
I27.0
ICD11 code
BB01.0

No additional description.

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