Familial Primary Pulmonary Hypertension

"Familial Primary Pulmonary Hypertension" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity.

MeSH information

Definition | Details | More General Concepts | Related Concepts | More Specific Concepts

Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.

Descriptor ID	D065627
MeSH Number(s)	C08.381.423.847
Concept/Terms	Familial Primary Pulmonary Hypertension Familial Primary Pulmonary Hypertension Heritable Pulmonary Arterial Hypertension Pulmonary Hypertension, Primary, Fenfluramine-Associated Pulmonary Hypertension, Primary, Fenfluramine-Associated Pulmonary Hypertension, Primary, Dexfenfluramine-Associated Pulmonary Hypertension, Primary, Dexfenfluramine-Associated Idiopathic Pulmonary Arterial Hypertension Idiopathic Pulmonary Arterial Hypertension Primary Pulmonary Hypertension Hypertension, Primary Pulmonary Hypertensions, Primary Pulmonary Primary Pulmonary Hypertensions Pulmonary Hypertension, Primary Pulmonary Hypertensions, Primary Pulmonary Hypertension, Primary, 1 Idiopathic Pulmonary Hypertension Hypertension, Idiopathic Pulmonary Hypertensions, Idiopathic Pulmonary Idiopathic Pulmonary Hypertensions Pulmonary Hypertension, Idiopathic Pulmonary Hypertensions, Idiopathic Pulmonary Hypertension, Primary, 1, With Hereditary Hemorrhagic Telangiectasia Pulmonary Hypertension, Primary, 1, With Hereditary Hemorrhagic Telangiectasia Pph1 With Hht Hht, Pph1 With Hhts, Pph1 With Pph1 With Hhts With Hht, Pph1 With Hhts, Pph1

Below are MeSH descriptors whose meaning is more general than "Familial Primary Pulmonary Hypertension".

Diseases [C]
Respiratory Tract Diseases [C08]
Lung Diseases [C08.381]
Hypertension, Pulmonary [C08.381.423]
Familial Primary Pulmonary Hypertension [C08.381.423.847]

Below are MeSH descriptors whose meaning is related to "Familial Primary Pulmonary Hypertension".

Below are MeSH descriptors whose meaning is more specific than "Familial Primary Pulmonary Hypertension".

publications

Timeline | Most Recent

This graph shows the total number of publications written about "Familial Primary Pulmonary Hypertension" by people in this website by year, and whether "Familial Primary Pulmonary Hypertension" was a major or minor topic of these publications.

Bar chart showing 7 publications over 6 distinct years, with a maximum of 2 publications in 2013

To see the data from this visualization as text, click here.

Year	Minor Topic	Total
2011	1	1
2012	1	1
2013	2	2
2014	1	1
2018	1	1
2023	1	1

Below are the most recent publications written about "Familial Primary Pulmonary Hypertension" by people in Profiles.

Katz MG, Hadas Y, Vincek A, Freage-Kahn L, Shtraizent N, Madjarov JM, Pastuszko P, Eliyahu E. Acid ceramidase gene therapy ameliorates pulmonary arterial hypertension with right heart dysfunction. Respir Res. 2023 Aug 11; 24(1):197.

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Cohen JL, Nees SN, Valencia GA, Rosenzweig EB, Krishnan US. Sildenafil Use in Children with Pulmonary Hypertension. J Pediatr. 2019 02; 205:29-34.e1.

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Khan MA, Pitcher JD, Kawut SM, Ho AC. Bilateral cotton wool spots after use of an endothelin receptor antagonist. Ophthalmic Surg Lasers Imaging Retina. 2014 Mar-Apr; 45(2):156-9.

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van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, M?ller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov; 72(11):1747-55.

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Derrett-Smith EC, Dooley A, Gilbane AJ, Trinder SL, Khan K, Baliga R, Holmes AM, Hobbs AJ, Abraham D, Denton CP. Endothelial injury in a transforming growth factor ?-dependent mouse model of scleroderma induces pulmonary arterial hypertension. Arthritis Rheum. 2013 Nov; 65(11):2928-39.

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Wojciak-Stothard B, Zhao L, Oliver E, Dubois O, Wu Y, Kardassis D, Vasilaki E, Huang M, Mitchell JA, Harrington LS, Louise H, Prendergast GC, Wilkins MR. Role of RhoB in the regulation of pulmonary endothelial and smooth muscle cell responses to hypoxia. Circ Res. 2012 May 25; 110(11):1423-34.

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Sachdev V, Kato GJ, Gibbs JS, Barst RJ, Machado RF, Nouraie M, Hassell KL, Little JA, Schraufnagel DE, Krishnamurti L, Novelli EM, Girgis RE, Morris CR, Rosenzweig EB, Badesch DB, Lanzkron S, Castro OL, Taylor JG, Hannoush H, Goldsmith JC, Gladwin MT, Gordeuk VR. Echocardiographic markers of elevated pulmonary pressure and left ventricular diastolic dysfunction are associated with exercise intolerance in adults and adolescents with homozygous sickle cell anemia in the United States and United Kingdom. Circulation. 2011 Sep 27; 124(13):1452-60.

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