Pulmonary Hypertension, Primary, 1 (PPH1)

Alias:

Pulmonary Arterial Hypertension

Pah

Pulmonary Hypertension, Primary, Fenfluramine or Dexfenfluramine-Associated

Pulmonary Hypertension, Familial Primary, 1, with or Without Hht

Familial Primary Pulmonary Hypertension

Sporadic Primary Pulmonary Hypertension

Idiopathic Pulmonary Hypertension

Primary Pulmonary Hypertension

Pph1

Idiopathic Pulmonary Arterial Hypertension

Pulmonary Hypertension, Familial Primary

Hypertension, Pulmonary, Primary, Type 1

Pah - [pulmonary Arterial Hypertension]

Arrillaga Ayerza Syndrome

Ayerza's Syndrome

Ayerza Syndrome

Fpph

Ppht

Pht

Pph

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Basic Information

Medical Symptom

Gene & Mutation

Related Drugs

Disease Model

References Literature

Pulmonary Hypertension, Primary, 1, also known as pulmonary arterial hypertension, is related to pulmonary arterial hypertension associated with congenital heart disease and heritable pulmonary arterial hypertension, and has symptoms including dyspnea An important gene associated with Pulmonary Hypertension, Primary, 1 is BMPR2 (Bone Morphogenetic Protein Receptor Type 2), and among its related pathways/superpathways are Signaling by BMP and ALK1 signaling events. The drugs Oxymetazoline and Phenylephrine have been mentioned in the context of this disorder. Affiliated tissues include heart and lung, and related phenotypes are right ventricular hypertrophy and increased pulmonary vascular resistance

Related ID：

MALACARDS：PLM164

OMIM：178600

MESH：D006976

ICD11：1931148955