Details of Disease

General Information of Disease (ID: DISP8ZX5)

Disease Name	Pulmonary arterial hypertension
Synonyms	PPH; idiopathic pulmonary hypertension; PAH with overt features of venous/capillaries involvement; PVOD/PCH; PAH; pulmonary arterial hypertension
Disease Class	BB01: Pulmonary hypertension
Definition	Pulmonary arterial hypertension (PAH) is a group of diseases characterized by mean pulmonary artery pressure >20 mmHg and elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, have overt features of venous/capillary involvement (pulmonary veno-occlusive disease, PVOD/pulmonary capillary hemangiomatosis, PCH), induced by drug or toxin (drug-or toxin-induced PAH), or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease).
Disease Hierarchy	DIS1RSP5: Pulmonary hypertension DISP8ZX5: Pulmonary arterial hypertension
ICD Code	ICD-11 ICD-11: BB01.0 ICD-9 ICD-9: 416 Expand ICD-11 'BB01.0 Expand ICD-10 'I26.9; 'I27; 'I27.0; 'I27.2; 'I78; 'I78.8 Expand ICD-9 416
Disease Identifiers	MONDO ID MONDO_0015924 UMLS CUI C2973725 MedGen ID 425404 HPO ID HP:0002092 Orphanet ID 182090 SNOMED CT ID 11399002

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 6 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Ambrisentan	DMD1QXW	Approved	Small molecular drug	[1]
Bosentan	DMIOGBU	Approved	Small molecular drug	[2]
Iloprost	DMVPZBE	Approved	Small molecular drug	[3]
Riociguat	DMXBLMP	Approved	Small molecular drug	[4]
Selexipag	DMAHSU0	Approved	Small molecular drug	[5]
Treprostinil	DMTIQF3	Approved	Small molecular drug	[6]
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⏷ Show the Full List of 6 Drug(s)

This Disease is Treated as An Indication in 33 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Bardoxolone methyl	DMODA2X	Phase 3	Small molecular drug	[7]
Beraprost 314d	DMU2G9T	Phase 3	NA	[8]
esuberaprost	DMXLGED	Phase 3	Small molecular drug	[7]
INOpulse	DMLLNGO	Phase 3	NA	[8]
LIQ861	DMFYKYG	Phase 3	NA	[8]
RemUnity	DMGE7J0	Phase 3	NA	[8]
Tysuberprost	DMFJHDC	Phase 3	NA	[8]
Treprostinil palmitil	DMSQ6U6	Phase 2/3	Small molecule	[9]
2-Methoxyestradiol	DMGBPME	Phase 2	Small molecular drug	[10]
ABI-009	DMQ6KZM	Phase 2	NA	[8]
BQ-123	DM0MO8I	Phase 2	Small molecular drug	[11]
Cicletanine	DMGTZLW	Phase 2	Small molecular drug	[12]
CXA10	DMM7Z6K	Phase 2	Small molecular drug	[13]
GeNOsyl	DMTMPSB	Phase 2	NA	[8]
GS-4997	DMNABMU	Phase 2	NA	[7]
GSK2586881	DMU5EVR	Phase 2	NA	[8]
H-1152	DM8XH3S	Phase 2	Small molecular drug	[14]
JTT-251	DM1945H	Phase 2	NA	[15]
LTP001	DMW1HI5	Phase 2	NA	[16]
MK-5475	DMIIZ4R	Phase 2	Small molecule	[17]
PB1046	DM8MJ3F	Phase 2	Recombinant protein	[18]
PRX-08066	DMR7H2V	Phase 2	Small molecular drug	[19]
Ralinepag	DM7G4Y9	Phase 2	NA	[8]
RemoSynch	DMKHWHE	Phase 2	NA	[8]
SPI-026	DMH9X10	Phase 2	NA	[7]
Terguride	DMZNFPG	Phase 2	Small molecular drug	[20]
Vasoactive intestinal peptide	DMRJ5LP	Phase 2	Small molecular drug	[21]
CAM2043	DMOSNCW	Phase 1/2	NA	[8]
Nitric oxide synthase gene therapy	DMQ0GOE	Phase 1/2	NA	[22]
Brilaroxazine	DM90J3J	Phase 1	Small molecule	[23]
GSK-2256098	DMR24MI	Phase 1	Small molecular drug	[8]
INS1009	DM1UDP3	Phase 1	NA	[8]
KAR5585	DMXYN1H	Phase 1	NA	[8]
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⏷ Show the Full List of 33 Drug(s)

This Disease is Treated as An Indication in 2 Patented Agent(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
PMID25623274-Compound-WO2014132220C1	DMA2IMQ	Patented	Small molecular drug	[24]
PMID25623274-Compound-WO2014132220C2	DMXEZWP	Patented	Small molecular drug	[24]
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This Disease is Treated as An Indication in 6 Discontinued Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Sitaxsentan	DMKM5HB	Withdrawn from market	Small molecular drug	[25]
TBC 11251 (TBC)	DMFR79T	Withdrawn from market	Small molecular drug	[26]
BMS-193884	DMODK2J	Discontinued in Phase 2	Small molecular drug	[27]
EDONENTAN HYDRATE	DMRPUEC	Discontinued in Phase 2	NA	[28]
ENRASENTAN	DM05F1Z	Discontinued in Phase 2	Small molecular drug	[29]
J-104132	DM5E6MK	Discontinued in Phase 2	Small molecular drug	[30]
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⏷ Show the Full List of 6 Drug(s)

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 46 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
AQP1	TTSF1KH	Limited	Autosomal dominant	[31]
BMP10	TTTG6H1	Limited	Autosomal dominant	[31]
CCN2	TTIL516	Limited	Altered Expression	[32]
ENG	TTB30LE	Limited	Genetic Variation	[33]
HLA-B	TTGS10J	Limited	Genetic Variation	[34]
KLK1	TT5T3P6	Limited	Autosomal dominant	[31]
PDE5A	TTJ0IQB	Limited	Biomarker	[35]
PDGFD	TTSN0GA	Limited	Autosomal dominant	[31]
NOTCH3	TTVX7IA	Disputed	Autosomal dominant	[31]
SMAD1	TT9GR53	Disputed	Autosomal dominant	[31]
GGCX	TT76OLR	Moderate	Autosomal dominant	[31]
ACE2	TTUI5H7	Strong	Biomarker	[36]
APLN	TT87D3J	Strong	Altered Expression	[37]
APLNR	TTJ8E43	Strong	Biomarker	[37]
ASIC1	TTRJYB6	Strong	Biomarker	[38]
BMP10	TTTG6H1	Strong	Biomarker	[39]
CAPN2	TTG5QB7	Strong	Altered Expression	[40]
CCL21	TTLZK1U	Strong	Biomarker	[41]
CES1	TTMF541	Strong	Biomarker	[42]
EIF2AK4	TT9U4EP	Strong	Genetic Variation	[43]
EPAS1	TTWPA54	Strong	Genetic Variation	[44]
FOSL2	TT689IR	Strong	Altered Expression	[45]
GDF15	TT4MXVG	Strong	Biomarker	[46]
GDF2	TTAP4T1	Strong	Altered Expression	[39]
HTR2B	TT0K1SC	Strong	Biomarker	[47]
ITPR2	TTK9OV3	Strong	Biomarker	[48]
KCNA5	TTW0CMT	Strong	Genetic Variation	[49]
LGALS3	TTFPQV7	Strong	Biomarker	[50]
LONP1	TTM1VPZ	Strong	Biomarker	[51]
MCAM	TTHRE05	Strong	Biomarker	[52]
METAP2	TTZL0OI	Strong	Altered Expression	[53]
NOX4	TTQRBSJ	Strong	Biomarker	[54]
NPPC	TTRK0B9	Strong	Biomarker	[55]
PAH	TTGSVH2	Strong	Genetic Variation	[56]
PTGIR	TTOFYT1	Strong	Biomarker	[57]
PTGIS	TTLXKZR	Strong	Genetic Variation	[58]
TPH1	TTZSJHV	Strong	Altered Expression	[59]
TPT1	TT3PTB6	Strong	Biomarker	[60]
UTRN	TTNO1VA	Strong	Biomarker	[61]
VHL	TTEMWSD	Strong	Biomarker	[62]
BMPR2	TTGKF90	Definitive	Autosomal dominant	[31]
CAV1	TTXUBN2	Definitive	Autosomal dominant	[31]
GDF2	TTAP4T1	Definitive	Autosomal dominant	[31]
KCNK3	TTGR91N	Definitive	Autosomal dominant	[31]
KDR	TTPFN62	Definitive	Autosomal dominant	[31]
SMAD9	TTX8EBV	Definitive	Autosomal dominant	[31]
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⏷ Show the Full List of 46 DTT(s)

This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
ABCC8	DTI58LU	Moderate	Autosomal dominant	[31]
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This Disease Is Related to 3 DME Molecule(s)

Gene Name	DME ID	Evidence Level	Mode of Inheritance	REF
CHST3	DEQIZP2	Limited	Genetic Variation	[63]
CHDH	DEAHED0	Strong	Genetic Variation	[64]
CRMP1	DE0EUXB	Strong	Biomarker	[61]
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This Disease Is Related to 68 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
TOPBP1	OT6UPZPD	No Known	Autosomal dominant	[31]
AQP1	OTX5MYX9	Limited	Autosomal dominant	[31]
BMP10	OTA3QKKG	Limited	Autosomal dominant	[31]
FBLN2	OTEHR7N7	Limited	Autosomal dominant	[31]
ITSN1	OT8YF3S5	Limited	Biomarker	[65]
KLF2	OTIP1UFX	Limited	Autosomal dominant	[31]
KLK1	OTFGP3UR	Limited	Autosomal dominant	[31]
PDGFD	OT5WRWRJ	Limited	Autosomal dominant	[31]
SAFB	OTGRV2LW	Limited	Biomarker	[66]
SNRPD1	OTWKZV4E	Limited	Biomarker	[67]
BMPR1A	OTQOA4ZH	Disputed	Unknown	[31]
BMPR1B	OTGFN0OD	Disputed	Unknown	[31]
NOTCH3	OTMVVA7F	Disputed	Autosomal dominant	[31]
SMAD1	OTQSHR25	Disputed	Autosomal dominant	[31]
SMAD4	OTWQWCKG	Disputed	Autosomal dominant	[31]
ABCC8	OTCWQ54I	Moderate	Autosomal dominant	[31]
GGCX	OTE0FNAP	Moderate	Autosomal dominant	[31]
TET2	OTKKT03T	Moderate	Autosomal dominant	[31]
ADAMTS8	OT2KFY1S	Strong	Biomarker	[68]
ADO	OTRLGQ7V	Strong	Genetic Variation	[69]
ATOH8	OT7SY3BN	Strong	Biomarker	[70]
BRAP	OTB7BAFQ	Strong	Genetic Variation	[71]
CHST13	OTO59SZQ	Strong	Genetic Variation	[63]
CLIC4	OT6KTPKD	Strong	Altered Expression	[72]
COX5A	OTP0961M	Strong	Biomarker	[73]
DAPK2	OTWODUQG	Strong	Biomarker	[61]
DECR1	OTCDIR6X	Strong	Biomarker	[74]
DENR	OTXP9HOY	Strong	Biomarker	[61]
EFNA1	OTU2NUA2	Strong	Altered Expression	[75]
EYA3	OTHLP1J3	Strong	Biomarker	[76]
GDF11	OTOSNMND	Strong	Biomarker	[77]
HLA-DPA1	OT7OG7Y2	Strong	Genetic Variation	[78]
IFIH1	OTZA2AHA	Strong	CausalMutation	[79]
JPH2	OTL9YH7V	Strong	Biomarker	[80]
KCNH4	OTHJ8WTU	Strong	Altered Expression	[81]
KCNH8	OT3I5FLB	Strong	Altered Expression	[81]
KIRREL1	OTOA7ON7	Strong	Biomarker	[82]
MCU	OTQZAYWQ	Strong	Biomarker	[83]
MIEF1	OTFSP3FS	Strong	Biomarker	[84]
MMACHC	OTX0TT3W	Strong	Genetic Variation	[85]
MUC6	OTPVL723	Strong	Genetic Variation	[86]
NFATC3	OTYOORME	Strong	Biomarker	[87]
NOX1	OTZPJQCC	Strong	Biomarker	[88]
PDK4	OTCMHMBZ	Strong	Biomarker	[89]
PIK3C3	OTLUM9L7	Strong	Biomarker	[90]
PLCD4	OT14EZVB	Strong	Genetic Variation	[91]
RNF213	OT4OVE9O	Strong	Genetic Variation	[92]
RNPC3	OTW5MKC1	Strong	Biomarker	[93]
SBNO1	OTNX3RL0	Strong	Biomarker	[94]
SBNO2	OT1C6J3K	Strong	Biomarker	[94]
SERPINA12	OTS1E6IP	Strong	Biomarker	[95]
SESN2	OT889IXY	Strong	Biomarker	[96]
SETD3	OTO5RAU2	Strong	Biomarker	[97]
SKIL	OTNBXH32	Strong	Biomarker	[94]
SMURF1	OT5UIZR8	Strong	Biomarker	[98]
SORL1	OTQ8FFNS	Strong	Biomarker	[99]
STIM2	OTYNXAW0	Strong	Biomarker	[100]
TMEM70	OTLTKYXG	Strong	Biomarker	[101]
TRAM1	OT3I0H8E	Strong	Biomarker	[102]
ATP13A3	OT5EGV3F	Definitive	Semidominant	[31]
BMPR2	OTM9W547	Definitive	Autosomal dominant	[31]
CAV1	OTEZUR1L	Definitive	Autosomal dominant	[31]
GDF2	OTRB5L04	Definitive	Autosomal dominant	[31]
KCNK3	OTWMAV6G	Definitive	Autosomal dominant	[31]
KDR	OT15797V	Definitive	Autosomal dominant	[31]
SMAD9	OTK4M1H8	Definitive	Autosomal dominant	[31]
SOX17	OT9H4WWE	Definitive	Autosomal dominant	[31]
TBX4	OTW58FG4	Definitive	Autosomal dominant	[31]
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⏷ Show the Full List of 68 DOT(s)

References

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2	URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 3494).
3	URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 1966).
4	URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 5257).
5	URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 7552).
6	URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 5820).
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24	Evaluation of WO-2014132220, selective PDGFR inhibitors for the treatment of pulmonary arterial hypertension.Expert Opin Ther Pat. 2015 Apr;25(4):493-9.
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36	Angiotensin-converting enzyme 2 activation suppresses pulmonary vascular remodeling by inducing apoptosis through the Hippo signaling pathway in rats with pulmonary arterial hypertension.Clin Exp Hypertens. 2019;41(6):589-598. doi: 10.1080/10641963.2019.1583247. Epub 2019 Feb 26.
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38	Revisiting the Role of TRP, Orai, and ASIC Channels in the Pulmonary Arterial Response to Hypoxia.Front Physiol. 2018 May 7;9:486. doi: 10.3389/fphys.2018.00486. eCollection 2018.
39	Characterization of GDF2 Mutations and Levels of BMP9 and BMP10 in Pulmonary Arterial Hypertension.Am J Respir Crit Care Med. 2020 Mar 1;201(5):575-585. doi: 10.1164/rccm.201906-1141OC.
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42	Reduced carboxylesterase 1 is associated with endothelial injury in methamphetamine-induced pulmonary arterial hypertension.Am J Physiol Lung Cell Mol Physiol. 2017 Aug 1;313(2):L252-L266. doi: 10.1152/ajplung.00453.2016. Epub 2017 May 4.
43	Novel EIF2AK4 mutations in histologically proven pulmonary capillary hemangiomatosis and hereditary pulmonary arterial hypertension.BMC Med Genet. 2019 Nov 11;20(1):176. doi: 10.1186/s12881-019-0915-7.
44	Therapeutic Targeting of Vascular Remodeling and Right Heart Failure in Pulmonary Arterial Hypertension with a HIF-2 Inhibitor.Am J Respir Crit Care Med. 2018 Dec 1;198(11):1423-1434. doi: 10.1164/rccm.201710-2079OC.
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48	Type 2 inositol 1,4,5-trisphosphate receptor inhibits the progression of pulmonary arterial hypertension via calcium signaling and apoptosis.Heart Vessels. 2019 Apr;34(4):724-734. doi: 10.1007/s00380-018-1304-4. Epub 2018 Nov 20.
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53	Early treatment with fumagillin, an inhibitor of methionine aminopeptidase-2, prevents Pulmonary Hypertension in monocrotaline-injured rats.PLoS One. 2012;7(4):e35388. doi: 10.1371/journal.pone.0035388. Epub 2012 Apr 11.
54	Effect of Ambrisentan Therapy on the Expression of Endothelin Receptor, Endothelial Nitric Oxide Synthase and NADPH Oxidase 4 in Monocrotaline-induced Pulmonary Arterial Hypertension Rat Model.Korean Circ J. 2019 Sep;49(9):866-876. doi: 10.4070/kcj.2019.0006. Epub 2019 Apr 25.
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