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Primary pulmonary hypertension(PPH1)

MedGen UID:
57749
•Concept ID:
C0152171
•
Disease or Syndrome
Synonym: PPH1
 
Related genes: ATP13A3, SMAD9, KCNK3, CAV1, BMPR2
 
Monarch Initiative: MONDO:0001999

Definition

Increased blood pressure in the arteries of the lungs; the etiology is unknown. [from MONDO]

Professional guidelines

PubMed

Pulmonary hypertension: evaluation and management.
Heresi GA, Dweik RA
Compr Ther 2007 Fall;33(3):150-61. doi: 10.1007/s12019-007-0014-0. PMID: 18004030
Primary pulmonary hypertension.
Runo JR, Loyd JE
Lancet 2003 May 3;361(9368):1533-44. doi: 10.1016/S0140-6736(03)13167-4. PMID: 12737878
Primary pulmonary hypertension.
Gaine SP, Rubin LJ
Lancet 1998 Aug 29;352(9129):719-25. doi: 10.1016/S0140-6736(98)02111-4. PMID: 9729004

Recent clinical studies

Etiology

Disease characteristics, treatments, and outcomes of patients with pulmonary arterial hypertension treated with selexipag in real-world settings from the SPHERE registry (SelexiPag: tHe usErs dRug rEgistry).
McLaughlin V, Farber HW, Highland KB, Hemnes AR, Chakinala MM, Chin KM, Han M, Cho M, Tobore T, Rahman M, Kim NH
J Heart Lung Transplant 2024 Feb;43(2):272-283. Epub 2023 Sep 29 doi: 10.1016/j.healun.2023.09.016. PMID: 37778526
Portopulmonary Hypertension: Management and Liver Transplantation Evaluation.
DuBrock HM
Chest 2023 Jul;164(1):206-214. Epub 2023 Jan 14 doi: 10.1016/j.chest.2023.01.009. PMID: 36649754
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Primary pulmonary hypertension.
Runo JR, Loyd JE
Lancet 2003 May 3;361(9368):1533-44. doi: 10.1016/S0140-6736(03)13167-4. PMID: 12737878
Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.
D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT
Ann Intern Med 1991 Sep 1;115(5):343-9. doi: 10.7326/0003-4819-115-5-343. PMID: 1863023

Diagnosis

Managing Pulmonary Arterial Hypertension With Cardiopulmonary Comorbidities.
Provencher S, Mai V, Bonnet S
Chest 2024 Mar;165(3):682-691. doi: 10.1016/j.chest.2023.08.023. PMID: 38461018
Portopulmonary Hypertension: Management and Liver Transplantation Evaluation.
DuBrock HM
Chest 2023 Jul;164(1):206-214. Epub 2023 Jan 14 doi: 10.1016/j.chest.2023.01.009. PMID: 36649754
Portopulmonary hypertension.
Cartin-Ceba R, Krowka MJ
Clin Liver Dis 2014 May;18(2):421-38. Epub 2014 Feb 25 doi: 10.1016/j.cld.2014.01.004. PMID: 24679504
Primary pulmonary hypertension.
Runo JR, Loyd JE
Lancet 2003 May 3;361(9368):1533-44. doi: 10.1016/S0140-6736(03)13167-4. PMID: 12737878
Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.
D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT
Ann Intern Med 1991 Sep 1;115(5):343-9. doi: 10.7326/0003-4819-115-5-343. PMID: 1863023

Therapy

Drug-Drug Interactions in the Management of Patients With Pulmonary Arterial Hypertension.
Wu S, Hoang HB, Yang JZ, Papamatheakis DG, Poch DS, Alotaibi M, Lombardi S, Rodriguez C, Kim NH, Fernandes TM
Chest 2022 Dec;162(6):1360-1372. Epub 2022 Jul 14 doi: 10.1016/j.chest.2022.06.042. PMID: 35841932Free PMC Article
Immunity and inflammation in pulmonary arterial hypertension: From pathophysiology mechanisms to treatment perspective.
Wang RR, Yuan TY, Wang JM, Chen YC, Zhao JL, Li MT, Fang LH, Du GH
Pharmacol Res 2022 Jun;180:106238. Epub 2022 Apr 30 doi: 10.1016/j.phrs.2022.106238. PMID: 35504356
Portopulmonary hypertension.
Cartin-Ceba R, Krowka MJ
Clin Liver Dis 2014 May;18(2):421-38. Epub 2014 Feb 25 doi: 10.1016/j.cld.2014.01.004. PMID: 24679504
Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study.
Hoeper MM, Barst RJ, Bourge RC, Feldman J, Frost AE, Galié N, Gómez-Sánchez MA, Grimminger F, Grünig E, Hassoun PM, Morrell NW, Peacock AJ, Satoh T, Simonneau G, Tapson VF, Torres F, Lawrence D, Quinn DA, Ghofrani HA
Circulation 2013 Mar 12;127(10):1128-38. Epub 2013 Feb 12 doi: 10.1161/CIRCULATIONAHA.112.000765. PMID: 23403476
Endothelin.
Kawanabe Y, Nauli SM
Cell Mol Life Sci 2011 Jan;68(2):195-203. Epub 2010 Sep 17 doi: 10.1007/s00018-010-0518-0. PMID: 20848158Free PMC Article

Prognosis

Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis.
Rosenkranz S, Pausch C, Coghlan JG, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Delcroix M, Ghofrani HA, Ewert R, Kabitz HJ, Skowasch D, Behr J, Milger K, Halank M, Wilkens H, Seyfarth HJ, Held M, Scelsi L, Neurohr C, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Eisenmann S, Schmidt KH, Remppis BA, Skride A, Jureviciene E, Gumbiene L, Miliauskas S, Löffler-Ragg J, Lange TJ, Olsson KM, Hoeper MM, Opitz C
J Heart Lung Transplant 2023 Jan;42(1):102-114. Epub 2022 Oct 13 doi: 10.1016/j.healun.2022.10.003. PMID: 36333206
Incidence and outcomes of pulmonary hypertension in the Ireland.
Cullivan S, Lennon D, Meghani S, Minnock C, McCullagh B, Gaine S
BMJ Open Respir Res 2022 Jun;9(1) doi: 10.1136/bmjresp-2022-001272. PMID: 35768152Free PMC Article
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Primary pulmonary hypertension.
Rubin LJ
N Engl J Med 1997 Jan 9;336(2):111-7. doi: 10.1056/NEJM199701093360207. PMID: 8988890
Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.
D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT
Ann Intern Med 1991 Sep 1;115(5):343-9. doi: 10.7326/0003-4819-115-5-343. PMID: 1863023

Clinical prediction guides

Pulmonary Artery Strain Predicts Prognosis in Pulmonary Arterial Hypertension.
Zhong L, Leng S, Alabed S, Chai P, Teo L, Ruan W, Low TT, Wild JM, Allen JC, Lim ST, Tan JL, Yip JW, Swift AJ, Kiely DG, Tan RS
JACC Cardiovasc Imaging 2023 Aug;16(8):1022-1034. Epub 2023 Apr 12 doi: 10.1016/j.jcmg.2023.02.007. PMID: 37052561
Combined pre- and post-capillary pulmonary hypertension in left heart disease.
Riccardi M, Pagnesi M, Sciatti E, Lombardi CM, Inciardi RM, Metra M, Vizzardi E
Heart Fail Rev 2023 Jan;28(1):137-148. Epub 2022 Jun 1 doi: 10.1007/s10741-022-10251-9. PMID: 35650331
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Portopulmonary hypertension.
Mukhtar NA, Fix OK
J Clin Gastroenterol 2011 Sep;45(8):703-10. doi: 10.1097/MCG.0b013e31820656bd. PMID: 21325952
Primary pulmonary hypertension.
Gaine SP, Rubin LJ
Lancet 1998 Aug 29;352(9129):719-25. doi: 10.1016/S0140-6736(98)02111-4. PMID: 9729004

Recent systematic reviews

Pulmonary hypertension survival and hospitalisations in people living with HIV: a systematic review and meta-analysis.
Youssef M, Boutros Salama M, Rehman N, Hanna C, Waniss MR, Mbuagbaw L
BMJ Open Respir Res 2024 Apr 11;11(1) doi: 10.1136/bmjresp-2024-002318. PMID: 38604738Free PMC Article
A Systematic Review of Novel Therapies of Pulmonary Arterial Hypertension.
Nabeh OA, Saud AI, Amin B, Khedr AS, Amr A, Faoosa AM, Esmat E, Mahmoud YM, Hatem A, Mohamed M, Osama A, Soliman YMA, Elkorashy RI, Elmorsy SA
Am J Cardiovasc Drugs 2024 Jan;24(1):39-54. Epub 2023 Nov 9 doi: 10.1007/s40256-023-00613-5. PMID: 37945977Free PMC Article
The Role of Aryl Hydrocarbon Receptor in the Endothelium: A Systematic Review.
Guerra-Ojeda S, Suarez A, Valls A, Verdú D, Pereda J, Ortiz-Zapater E, Carretero J, Mauricio MD, Serna E
Int J Mol Sci 2023 Aug 31;24(17) doi: 10.3390/ijms241713537. PMID: 37686342Free PMC Article
Pulmonary vasodilator therapies in pulmonary arterial hypertension associated with CHD: a systematic review and network meta-analysis.
Yasuhara J, Watanabe K, Watanabe A, Shirasu T, Matsuzaki Y, Watanabe H, Takagi H, Sumitomo N, Kuno T
Cardiol Young 2023 Nov;33(11):2297-2311. Epub 2023 Feb 1 doi: 10.1017/S1047951123000124. PMID: 36721907
New progress in diagnosis and treatment of pulmonary arterial hypertension.
Zhang ZQ, Zhu SK, Wang M, Wang XA, Tong XH, Wan JQ, Ding JW
J Cardiothorac Surg 2022 Aug 29;17(1):216. doi: 10.1186/s13019-022-01947-y. PMID: 36038916Free PMC Article

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