Idiopathic pulmonary arterial hypertension
ICD-10 Code
I27.0
Idiopathic pulmonary arterial hypertension
Definition
A sporadic form of pulmonary arterial hypertension (PAH) characterized by elevated pulmonary arterial resistance leading to right heart failure. IPAH is progressive and potentially fatal and not associated with an underlying condition or family history of PAH. The etiology is unknown.
Also known as idiopathic pulmonary arterial hypertension, IPAH, primary pulmonary arterial hypertension, primary pulmonary hypertension — per MONDO