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Pulmonary hypertension, primary, 3(PPH3)

MedGen UID:: 815522
•Concept ID:: C3809192
•: Disease or Syndrome

Synonyms:	PPH3; PULMONARY HYPERTENSION, PRIMARY, 3

Gene (location): Gene(s) directly associated with this condition or phenotype.	CAV1 (7q31.2)

Monarch Initiative:	MONDO:0014135
OMIM®:	615343

Definition

Pulmonary arterial hypertension is a progressive disorder characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. Pulmonary arterial hypertension is one form of a broader condition known as pulmonary hypertension. Pulmonary hypertension occurs when most of the very small arteries throughout the lungs narrow in diameter, which increases the resistance to blood flow through the lungs. To overcome the increased resistance, blood pressure increases in the pulmonary artery and in the right ventricle of the heart, which is the chamber that pumps blood into the pulmonary artery. Ultimately, the increased blood pressure can damage the right ventricle of the heart.

Signs and symptoms of pulmonary arterial hypertension occur when increased blood pressure cannot fully overcome the elevated resistance. As a result, the flow of oxygenated blood from the lungs to the rest of the body is insufficient. Shortness of breath (dyspnea) during exertion and fainting spells are the most common symptoms of pulmonary arterial hypertension. People with this disorder may experience additional symptoms, particularly as the condition worsens. Other symptoms include dizziness, swelling (edema) of the ankles or legs, chest pain, and a rapid heart rate. [from MedlinePlus Genetics]

Clinical features

From HPO

Increased pulmonary vascular resistance

MedGen UID:: 356775
•Concept ID:: C1867423
•: Finding

Pulmonary vascular resistance (PVR) more than 3 wood units, as defined by the current definition of pulmonary hypertension. 95% of individuals have a PVR of less than 2.4 wood units.

See: Feature record | Search on this feature

Pulmonary arterial hypertension

MedGen UID:: 425404
•Concept ID:: C2973725
•: Disease or Syndrome

Pulmonary hypertension is defined mean pulmonary artery pressure of 25mmHg or more and pulmonary capillary wedge pressure of 15mmHg or less when measured by right heart catheterisation at rest and in a supine position.

See: Feature record | Search on this feature

Elevated pulmonary artery pressure

MedGen UID:: 812247
•Concept ID:: C3805917
•: Finding

An abnormally elevated blood pressure in the circulation of the pulmonary artery.

See: Feature record | Search on this feature

Dyspnea

MedGen UID:: 3938
•Concept ID:: C0013404
•: Sign or Symptom

Difficult or labored breathing. Dyspnea is a subjective feeling only the patient can rate, e.g., on a Borg scale.

See: Feature record | Search on this feature

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Abnormality of the cardiovascular system
Abnormality of the respiratory system
- Dyspnea

Term Hierarchy

Professional guidelines

PubMed

Abiraterone acetate and prednisolone with or without enzalutamide for high-risk non-metastatic prostate cancer: a meta-analysis of primary results from two randomised controlled phase 3 trials of the STAMPEDE platform protocol.

Attard G, Murphy L, Clarke NW, Cross W, Jones RJ, Parker CC, Gillessen S, Cook A, Brawley C, Amos CL, Atako N, Pugh C, Buckner M, Chowdhury S, Malik Z, Russell JM, Gilson C, Rush H, Bowen J, Lydon A, Pedley I, O'Sullivan JM, Birtle A, Gale J, Srihari N, Thomas C, Tanguay J, Wagstaff J, Das P, Gray E, Alzoueb M, Parikh O, Robinson A, Syndikus I, Wylie J, Zarkar A, Thalmann G, de Bono JS, Dearnaley DP, Mason MD, Gilbert D, Langley RE, Millman R, Matheson D, Sydes MR, Brown LC, Parmar MKB, James ND; Systemic Therapy in Advancing or Metastatic Prostate cancer: Evaluation of Drug Efficacy (STAMPEDE) investigators
Lancet 2022 Jan 29;399(10323):447-460. Epub 2021 Dec 23 doi: 10.1016/S0140-6736(21)02437-5. PMID: 34953525 Free PMC Article

Venous Ulcers: Diagnosis and Treatment.

Bonkemeyer Millan S, Gan R, Townsend PE
Am Fam Physician 2019 Sep 1;100(5):298-305. PMID: 31478635

Contemporary management of acute right ventricular failure: a statement from the Heart Failure Association and the Working Group on Pulmonary Circulation and Right Ventricular Function of the European Society of Cardiology.

Harjola VP, Mebazaa A, Čelutkienė J, Bettex D, Bueno H, Chioncel O, Crespo-Leiro MG, Falk V, Filippatos G, Gibbs S, Leite-Moreira A, Lassus J, Masip J, Mueller C, Mullens W, Naeije R, Nordegraaf AV, Parissis J, Riley JP, Ristic A, Rosano G, Rudiger A, Ruschitzka F, Seferovic P, Sztrymf B, Vieillard-Baron A, Yilmaz MB, Konstantinides S
Eur J Heart Fail 2016 Mar;18(3):226-41. doi: 10.1002/ejhf.478. PMID: 26995592

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Recent clinical studies

Etiology

Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension.

Humbert M, McLaughlin V, Gibbs JSR, Gomberg-Maitland M, Hoeper MM, Preston IR, Souza R, Waxman AB, Ghofrani HA, Escribano Subias P, Feldman J, Meyer G, Montani D, Olsson KM, Manimaran S, de Oliveira Pena J, Badesch DB
Eur Respir J 2023 Jan;61(1) Epub 2023 Jan 6 doi: 10.1183/13993003.01347-2022. PMID: 36041750 Free PMC Article

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.

Hoeper MM, Dwivedi K, Pausch C, Lewis RA, Olsson KM, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Opitz C, Gibbs JSR, Delcroix M, Park DH, Ghofrani HA, Ewert R, Kaemmerer H, Kabitz HJ, Skowasch D, Behr J, Milger K, Lange TJ, Wilkens H, Seyfarth HJ, Held M, Dumitrescu D, Tsangaris I, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Eisenmann S, Schmidt KH, Swift AJ, Thompson AAR, Elliot CA, Rosenkranz S, Condliffe R, Kiely DG, Halank M
Lancet Respir Med 2022 Oct;10(10):937-948. Epub 2022 Jun 28 doi: 10.1016/S2213-2600(22)00097-2. PMID: 35777416 Free PMC Article

Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension.

Toshner M, Church C, Harbaum L, Rhodes C, Villar Moreschi SS, Liley J, Jones R, Arora A, Batai K, Desai AA, Coghlan JG, Gibbs JSR, Gor D, Gräf S, Harlow L, Hernandez-Sanchez J, Howard LS, Humbert M, Karnes J, Kiely DG, Kittles R, Knightbridge E, Lam B, Lutz KA, Nichols WC, Pauciulo MW, Pepke-Zaba J, Suntharalingam J, Soubrier F, Trembath RC, Schwantes-An TL, Wort SJ, Wilkins MR, Gaine S, Morrell NW, Corris PA; Uniphy Clinical Trials Network
Eur Respir J 2022 Mar;59(3) Epub 2022 Mar 10 doi: 10.1183/13993003.02463-2020. PMID: 34588193 Free 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

Macitentan and morbidity and mortality in pulmonary arterial hypertension.

Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani HA, Jansa P, Jing ZC, Le Brun FO, Mehta S, Mittelholzer CM, Perchenet L, Sastry BK, Sitbon O, Souza R, Torbicki A, Zeng X, Rubin LJ, Simonneau G; SERAPHIN Investigators
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Diagnosis

Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis.

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J Heart Lung Transplant 2023 Jan;42(1):102-114. Epub 2022 Oct 13 doi: 10.1016/j.healun.2022.10.003. PMID: 36333206

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.

Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.

Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study.

Coghlan JG, Denton CP, Grünig E, Bonderman D, Distler O, Khanna D, Müller-Ladner U, Pope JE, Vonk MC, Doelberg M, Chadha-Boreham H, Heinzl H, Rosenberg DM, McLaughlin VV, Seibold JR; DETECT study group
Ann Rheum Dis 2014 Jul;73(7):1340-9. Epub 2013 May 18 doi: 10.1136/annrheumdis-2013-203301. PMID: 23687283 Free PMC Article

Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology.

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Therapy

Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension.

Hoeper MM, Badesch DB, Ghofrani HA, Gibbs JSR, Gomberg-Maitland M, McLaughlin VV, Preston IR, Souza R, Waxman AB, Grünig E, Kopeć G, Meyer G, Olsson KM, Rosenkranz S, Xu Y, Miller B, Fowler M, Butler J, Koglin J, de Oliveira Pena J, Humbert M; STELLAR Trial Investigators
N Engl J Med 2023 Apr 20;388(16):1478-1490. Epub 2023 Mar 6 doi: 10.1056/NEJMoa2213558. PMID: 36877098

Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension.

The evolving landscape of pulmonary arterial hypertension clinical trials.

Weatherald J, Boucly A, Peters A, Montani D, Prasad K, Psotka MA, Zannad F, Gomberg-Maitland M, McLaughlin V, Simonneau G, Humbert M; Evolving Landscape of Pulmonary Arterial Hypertension and Redesigning Pulmonary Arterial Hypertension Clinical Trials Task Force of the 18th Global CardioVascular Clinical Trialists Forum
Lancet 2022 Nov 26;400(10366):1884-1898. doi: 10.1016/S0140-6736(22)01601-4. PMID: 36436527

Macitentan and morbidity and mortality in pulmonary arterial hypertension.

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Prognosis

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.

Novel Mechanisms Targeted by Drug Trials in Pulmonary Arterial Hypertension.

Condon DF, Agarwal S, Chakraborty A, Auer N, Vazquez R, Patel H, Zamanian RT, de Jesus Perez VA
Chest 2022 Apr;161(4):1060-1072. Epub 2021 Oct 13 doi: 10.1016/j.chest.2021.10.010. PMID: 34655569 Free PMC Article

Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension.

Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.

Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology.

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Clinical prediction guides

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.

Selexipag for the treatment of chronic thromboembolic pulmonary hypertension.

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Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.

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Circulation 2018 Oct 16;138(16):1623-1635. doi: 10.1161/CIRCULATIONAHA.118.034338. PMID: 30354459 Free PMC Article

Macitentan and morbidity and mortality in pulmonary arterial hypertension.

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Recent systematic reviews

Cancer Incidence, Mortality, Years of Life Lost, Years Lived With Disability, and Disability-Adjusted Life Years for 29 Cancer Groups From 2010 to 2019: A Systematic Analysis for the Global Burden of Disease Study 2019.

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El-Abid H, Elbarazi I, Elhabashy HR, Elhadi M, El-Jaafary SI, Eshrati B, Eskandarieh S, Esmaeilzadeh F, Etemadi A, Ezzikouri S, Faisaluddin M, Faraon EJA, Fares J, Farzadfar F, Feroze AH, Ferrero S, Ferro Desideri L, Filip I, Fischer F, Fisher JL, Foroutan M, Fukumoto T, Gaal PA, Gad MM, Gadanya MA, Gallus S, Gaspar Fonseca M, Getachew Obsa A, Ghafourifard M, Ghashghaee A, Ghith N, Gholamalizadeh M, Gilani SA, Ginindza TG, Gizaw ATT, Glasbey JC, Golechha M, Goleij P, Gomez RS, Gopalani SV, Gorini G, Goudarzi H, Grosso G, Gubari MIM, Guerra MR, Guha A, Gunasekera DS, Gupta B, Gupta VB, Gupta VK, Gutiérrez RA, Hafezi-Nejad N, Haider MR, Haj-Mirzaian A, Halwani R, Hamadeh RR, Hameed S, Hamidi S, Hanif A, Haque S, Harlianto NI, Haro JM, Hasaballah AI, Hassanipour S, Hay RJ, Hay SI, Hayat K, Heidari G, Heidari M, Herrera-Serna BY, Herteliu C, Hezam K, Holla R, Hossain MM, Hossain MBH, Hosseini MS, Hosseini M, Hosseinzadeh M, Hostiuc M, Hostiuc S, Househ M, Hsairi M, Huang J, Hugo FN, 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A, Shetty JK, Shetty PH, Shibuya K, Shirkoohi R, Shivakumar KM, Shivarov V, Siabani S, Siddappa Malleshappa SK, Silva DAS, Singh JA, Sintayehu Y, Skryabin VY, Skryabina AA, Soeberg MJ, Sofi-Mahmudi A, Sotoudeh H, Steiropoulos P, Straif K, Subedi R, Sufiyan MB, Sultan I, Sultana S, Sur D, Szerencsés V, Szócska M, Tabarés-Seisdedos R, Tabuchi T, Tadbiri H, Taherkhani A, Takahashi K, Talaat IM, Tan KK, Tat VY, Tedla BAA, Tefera YG, Tehrani-Banihashemi A, Temsah MH, Tesfay FH, Tessema GA, Thapar R, Thavamani A, Thoguluva Chandrasekar V, Thomas N, Tohidinik HR, Touvier M, Tovani-Palone MR, Traini E, Tran BX, Tran KB, Tran MTN, Tripathy JP, Tusa BS, Ullah I, Ullah S, Umapathi KK, Unnikrishnan B, Upadhyay E, Vacante M, Vaezi M, Valadan Tahbaz S, Velazquez DZ, Veroux M, Violante FS, Vlassov V, Vo B, Volovici V, Vu GT, Waheed Y, Wamai RG, Ward P, Wen YF, Westerman R, Winkler AS, Yadav L, Yahyazadeh Jabbari SH, Yang L, Yaya S, Yazie TSY, Yeshaw Y, Yonemoto N, Younis MZ, Yousefi Z, Yu C, Yuce D, 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Pulmonary hypertension, primary, 3(PPH3)

Definition

Clinical features

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Genetic Testing Registry

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