 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.
Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.
| Descriptor ID |
D065627
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| MeSH Number(s) |
C08.381.423.847
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| Concept/Terms |
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
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Below are MeSH descriptors whose meaning is more general than "Familial Primary Pulmonary Hypertension".
Below are MeSH descriptors whose meaning is more specific than "Familial Primary Pulmonary Hypertension".
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.
To see the data from this visualization as text, click here.
| Year | Major Topic | Minor Topic | Total |
|---|
| 2010 | 0 | 2 | 2 | | 2011 | 0 | 14 | 14 | | 2012 | 0 | 14 | 14 | | 2013 | 0 | 13 | 13 | | 2014 | 0 | 9 | 9 | | 2015 | 2 | 0 | 2 | | 2016 | 5 | 1 | 6 | | 2017 | 1 | 2 | 3 | | 2018 | 3 | 2 | 5 | | 2019 | 1 | 2 | 3 | | 2020 | 0 | 2 | 2 | | 2021 | 1 | 6 | 7 | | 2022 | 0 | 12 | 12 | | 2023 | 0 | 10 | 10 | | 2024 | 0 | 4 | 4 | | 2025 | 1 | 0 | 1 |
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Below are the most recent publications written about "Familial Primary Pulmonary Hypertension" by people in Profiles.
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Crnkovic S, Thekkekara Puthenparampil H, Mulch S, Biasin V, Radic N, Wilhelm J, Bartkuhn M, Bonyadi Rad E, Wawrzen A, Matzer I, Mitra A, Leib RD, Nagy BM, Sahu-Osen A, Valzano F, Bordag N, Evermann M, Hoetzenecker K, Olschewski A, Ljubojevic-Holzer S, Wygrecka M, Stenmark K, Marsh LM, de Jesus Perez V, Kwapiszewska G. Adventitial fibroblasts direct smooth muscle cell-state transition in pulmonary vascular disease. Elife. 2025 Apr 10; 13.
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Kovacs G, Bartolome S, Denton CP, Gatzoulis MA, Gu S, Khanna D, Badesch D, Montani D. Definition, classification and diagnosis of pulmonary hypertension. Eur Respir J. 2024 Oct; 64(4).
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Tuder RM, Gandjeva A, Williams S, Kumar S, Kheyfets VO, Hatton-Jones KM, Starr JR, Yun J, Hong J, West NP, Stenmark KR. Digital Spatial Profiling Identifies Distinct Molecular Signatures of Vascular Lesions in Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. 2024 08 01; 210(3):329-342.
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Ploegstra MJ, Ivy DD, Beghetti M, Bonnet D, Alehan D, Ablonczy L, Mattos S, Bowers D, Humpl T, Berger RMF. Long-term outcome of children with newly diagnosed pulmonary arterial hypertension: results from the global TOPP registry. Eur Heart J Qual Care Clin Outcomes. 2024 Jan 12; 10(1):66-76.
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Neves da Silva HV, Weinman JP, Englund EK, Deterding RR, Ivy DD, Browne LP. Computed tomographic findings in TBX4 mutation: a common cause of severe pulmonary artery hypertension in children. Pediatr Radiol. 2024 02; 54(2):199-207.
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Doughty ES, Norvik C, Levin A, Bodmer J, Tran-Lundmark K, Abman SH, Galambos C. Long-Term Effect of TBX4 Germline Mutation on Pulmonary Clinico-Histopathologic Phenotype. Pediatr Dev Pathol. 2024 Jan-Feb; 27(1):83-89.
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Souza R, Badesch DB, Ghofrani HA, Gibbs JSR, Gomberg-Maitland M, McLaughlin VV, Preston IR, Waxman AB, Gr?nig E, Kopec G, Meyer G, Olsson KM, Rosenkranz S, Lin J, Johnson-Levonas AO, de Oliveira Pena J, Humbert M, Hoeper MM. Effects of sotatercept on haemodynamics and right heart function: analysis of the STELLAR trial. Eur Respir J. 2023 09; 62(3).
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Norlander AE, Abney M, Cephus JY, Roe CE, Irish JM, Shelburne NJ, Newcomb DC, Hemnes AR, Peebles RS. Prostaglandin I2 Therapy Promotes Regulatory T Cell Generation in Patients with Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. 2023 09 15; 208(6):737-739.
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Avitabile CM, Yung D, Handler S, Hopper RK, Fineman J, Freire G, Varghese N, Mullen MP, Krishnan US, Austin E, Silveira L, Ivy DD. Measurement of Physical Activity by Actigraphy in Infants and Young Children with Pulmonary Arterial Hypertension. J Pediatr. 2023 11; 262:113639.
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McCormick A, Krishnan A, Badesch D, Benza RL, Bull TM, De Marco T, Feldman J, Hemnes AR, Hirsch R, Horn E, Kennedy J, Mathai SC, McConnell W, Pugliese SC, Sager JS, Shlobin OA, Simon MA, Lammi MR. Pulmonary artery compliance in different forms of pulmonary hypertension. Heart. 2023 06 26; 109(14):1098-1105.
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