Pulmonary arterial hypertension WHO functional class I (DBCOND0160108)

Identifiers

Synonyms

PAH WHO Group I / Pulmonary Arterial Hypertension (PAH) (WHO Group 1 PH) / WHO Group 1 Pulmonary arterial hypertension / Pulmonary Arterial Hypertension WHO Group I / Pulmonary Arterial Hypertension / Pulmonary hypertensive arterial disease (disorder)

Associated Data

Indicated Drugs and Targets

Drug	Description	Targets
Ambrisentan	A selective type A endothelin receptor antagonist used to treat primary pulmonary arterial hypertension and pulmonary arterial hypertension based on diagnostic classifications.	Endothelin receptor type Btarget Endothelin-1 receptortarget Cytochrome P450 2C19enzyme Cytochrome P450 3A4enzyme UDP-glucuronosyltransferase 1A9enzyme UDP-glucuronosyltransferase 1A3enzyme UDP-glucuronosyltransferase 2B7enzyme ATP-dependent translocase ABCB1transporter Solute carrier organic anion transporter family member 1B1transporter Solute carrier organic anion transporter family member 1B3transporter Cytochrome P450 3A5enzyme
Macitentan	An endothelin receptor antagonist used to manage pulmonary arterial hypertension to delay disease progression.	Cytochrome P450 3A4enzyme Cytochrome P450 2C19enzyme Endothelin-1 receptortarget Endothelin receptor type Btarget Albumincarrier Cytochrome P450 2C9enzyme Cytochrome P450 2C8enzyme alpha1-acid glycoproteincarrier
Selexipag	A non prostanoid IP prostacyclin receptor agonist used to treat pulmonary arterial hypertension.	Prostacyclin receptortarget Liver carboxylesterase 1enzyme Cytochrome P450 2C8enzyme Cytochrome P450 3A4enzyme Solute carrier organic anion transporter family member 1B1transporter Solute carrier organic anion transporter family member 1B3transporter ATP-dependent translocase ABCB1transporter
Sotatercept	A recombinant activin receptor fusion protein used to treat pulmonary arterial hypertension.	Activin receptor type-2Atarget Activin receptor type-1Btarget Growth/differentiation factor 8target Growth/differentiation factor 11target

Clinical Trials

Identifier	Title	Drug(s)	Purpose	Phase	Status
NCT00593905	Pharmacogenomics in Pulmonary Arterial Hypertension	Ambrisentan Bosentan Sitaxentan	Not Available	Not Available	withdrawn
NCT06643143	A Phase 2 Study of IkT-001Pro in Pulmonary Arterial Hypertension (PAH)	No drug interventions	treatment	2	not_yet_recruiting
NCT03904693	Clinical Study to Compare the Efficacy and Safety of Macitentan and Tadalafil Monotherapies With the Corresponding Fixed-dose Combination Therapy in Subjects With Pulmonary Arterial Hypertension (PAH)	Macitentan Tadalafil	treatment	3	active_not_recruiting
NCT02198456	Imaging Control Study, 3D Echo, MR and RHC.	No drug interventions	Not Available	Not Available	unknown_status