
Familial Primary Pulmonary Hypertension
📋Overview
Familial Primary Pulmonary Hypertension is a legacy term referring to heritable pulmonary arterial hypertension (PAH), a rare disorder characterized by elevated blood pressure in the pulmonary arteries due to genetic mutations. The modern preferred term is Heritable Pulmonary Arterial Hypertension (HPAH), which is a subset of pulmonary arterial hypertension with a genetic basis, often involving mutations in genes such as BMPR2.
Medical codes (for reference)
UMLS CUI: C0340543ICD-10-CM
I27.0
MeSH
D065627
SNOMED CT (US)
233944003697897003
Codes are provided for reference and interoperability. They are not a diagnosis.
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