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This review explores the dual landscape of PH, integrating recent insights into its complex pathogenesis with evolving clinical management strategies. The pathogenesis of PH involves a complex interplay of genetics, metabolism, immune dysfunction, biomechanical force and other factors, all contributing to vascular remodeling and presenting opportunities for novel therapies and biomarkers. Among these, certain pathogenic mechanisms are of particular relevance and may hold distinct importance in the pediatric population. Concurrently, notable progress has been made in clinical front including diagnosis, risk stratification, drug development, interventional or surgical procedures, and imaging technologies. Hence, this review integrates current evidence in both PH pathogenesis and clinical management to guide future research toward novel therapies and precision medicine.<\/jats:p>","DOI":"10.1515\/mr-2026-0002","type":"journal-article","created":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T21:06:18Z","timestamp":1773954378000},"page":"120-141","source":"Crossref","is-referenced-by-count":0,"title":["Advances in the pathogenesis and clinical management of pulmonary hypertension"],"prefix":"10.1515","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2860-3252","authenticated-orcid":false,"given":"Yi","family":"Yan","sequence":"first","affiliation":[{"name":"Heart Center and Shanghai Institute of Pediatric Congenital Heart Disease, Shanghai Children\u2019s Medical Center, National Children\u2019s Medical Center , 426116 Shanghai Jiao Tong University School of Medicine , Shanghai , China"},{"name":"Shanghai Clinical Research Center for Rare Pediatric Diseases, Shanghai Children\u2019s Medical Center, National Children\u2019s Medical Center , Shanghai Jiao Tong University School of Medicine , Shanghai , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhuoyuan","family":"Xu","sequence":"additional","affiliation":[{"name":"Heart Center and Shanghai Institute of Pediatric Congenital Heart Disease, Shanghai Children\u2019s Medical Center, National Children\u2019s Medical Center , 426116 Shanghai Jiao Tong University School of Medicine , Shanghai , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yanghanyue","family":"Zhou","sequence":"additional","affiliation":[{"name":"Heart Center and Shanghai Institute of Pediatric Congenital Heart Disease, Shanghai Children\u2019s Medical Center, National Children\u2019s Medical Center , 426116 Shanghai Jiao Tong University School of Medicine , Shanghai , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xike","family":"Wang","sequence":"additional","affiliation":[{"name":"Guizhou Branch of Shanghai Children's Medical Center , Guizhou Provincial People's Hospital , Guizhou Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hao","family":"Zhang","sequence":"additional","affiliation":[{"name":"Heart Center and Shanghai Institute of Pediatric Congenital Heart Disease, Shanghai Children\u2019s Medical Center, National Children\u2019s Medical Center , 426116 Shanghai Jiao Tong University School of Medicine , Shanghai , China"},{"name":"Shanghai Clinical Research Center for Rare Pediatric Diseases, Shanghai Children\u2019s Medical Center, National Children\u2019s Medical Center , Shanghai Jiao Tong University School of Medicine , Shanghai , China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","published-online":{"date-parts":[[2026,3,20]]},"reference":[{"key":"2026050115424109980_j_mr-2026-0002_ref_001","doi-asserted-by":"crossref","unstructured":"Humbert, M, Kovacs, G, Hoeper, MM, Badagliacca, R, Berger, RMF, Brida, M, et al.. 2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the european respiratory society (ERS). 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Arterioscler Thromb Vasc Biol 2023;43:1179\u201398. https:\/\/doi.org\/10.1161\/ATVBAHA.122.318842.","DOI":"10.1161\/ATVBAHA.122.318842"},{"key":"2026050115424109980_j_mr-2026-0002_ref_046","doi-asserted-by":"crossref","unstructured":"Cunningham, CM, Li, M, Ruffenach, G, Doshi, M, Aryan, L, Hong, J, et al.. Y-chromosome gene, uty, protects against pulmonary hypertension by reducing proinflammatory chemokines. Am J Respir Crit Care Med 2022;206:186\u201396. https:\/\/doi.org\/10.1164\/rccm.202110-2309OC.","DOI":"10.1164\/rccm.202110-2309OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_047","doi-asserted-by":"crossref","unstructured":"Umar, S, Cunningham, CM, Itoh, Y, Moazeni, S, Vaillancourt, M, Sarji, S, et al.. The Y chromosome plays a protective role in experimental hypoxic pulmonary hypertension. 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Circulation 2022;146:1243\u201358. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.122.059591.","DOI":"10.1161\/CIRCULATIONAHA.122.059591"},{"key":"2026050115424109980_j_mr-2026-0002_ref_054","doi-asserted-by":"crossref","unstructured":"Wu, Z, Zhu, L, Nie, X, Wei, L, Qi, Y. USP15 promotes pulmonary vascular remodeling in pulmonary hypertension in a YAP1\/TAZ-dependent manner. Exp Mol Med 2023;55:183\u201395. https:\/\/doi.org\/10.1038\/s12276-022-00920-y.","DOI":"10.1038\/s12276-022-00920-y"},{"key":"2026050115424109980_j_mr-2026-0002_ref_055","doi-asserted-by":"crossref","unstructured":"ShenH, Gao, Y, Ge, D, Tan, M, Yin, Q, Wei, TYW, et al.. BRCC3 regulation of ALK2 in vascular smooth muscle cells: implication in pulmonary hypertension. Circulation 2024;150:132\u201350. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.123.066430.","DOI":"10.1161\/CIRCULATIONAHA.123.066430"},{"key":"2026050115424109980_j_mr-2026-0002_ref_056","doi-asserted-by":"crossref","unstructured":"Rhodes, CJ. The cancer hypothesis of pulmonary arterial hypertension: are polyamines the new warburg. Eur Respir J 2020;56:2002350. https:\/\/doi.org\/10.1183\/13993003.02350-2020.","DOI":"10.1183\/13993003.02350-2020"},{"key":"2026050115424109980_j_mr-2026-0002_ref_057","doi-asserted-by":"crossref","unstructured":"He, YY, Yan, Y, Jiang, X, Zhao, JH, Wang, Z, Wu, T, et al.. Spermine promotes pulmonary vascular remodelling and its synthase is a therapeutic target for pulmonary arterial hypertension. Eur Respir J 2020;56:2000522. https:\/\/doi.org\/10.1183\/13993003.00522-2020.","DOI":"10.1183\/13993003.00522-2020"},{"key":"2026050115424109980_j_mr-2026-0002_ref_058","doi-asserted-by":"crossref","unstructured":"Luo, Y, Qi, X, Zhang, Z, Zhang, J, Li, B, Shu, T, et al.. Inactivation of malic enzyme 1 in endothelial cells alleviates pulmonary hypertension. 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Hypertension 2023;80:1035\u201347. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.122.19857.","DOI":"10.1161\/HYPERTENSIONAHA.122.19857"},{"key":"2026050115424109980_j_mr-2026-0002_ref_061","doi-asserted-by":"crossref","unstructured":"Liu, B, Yi, D, Li, S, Ramirez, K, Xia, X, Cao, Y, et al.. Single-cell and spatial transcriptomics identified fatty acid-binding proteins controlling endothelial glycolytic and arterial programming in pulmonary hypertension. Arterioscler Thromb Vasc Biol 2025;45:1145\u201365. https:\/\/doi.org\/10.1161\/ATVBAHA.124.321173.","DOI":"10.1161\/ATVBAHA.124.321173"},{"key":"2026050115424109980_j_mr-2026-0002_ref_062","doi-asserted-by":"crossref","unstructured":"Ma, Q, Yang, Q, Xu, J, Sellers, HG, Brown, ZL, Liu, Z, et al.. Purine synthesis suppression reduces the development and progression of pulmonary hypertension in rodent models. 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Hypertension 2023;80:2372\u201385. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.123.20914.","DOI":"10.1161\/HYPERTENSIONAHA.123.20914"},{"key":"2026050115424109980_j_mr-2026-0002_ref_067","doi-asserted-by":"crossref","unstructured":"Zhu, MM, Dai, J, Dai, Z, Peng, Y, Zhao, YY. GCN2 kinase activation mediates pulmonary vascular remodeling and pulmonary arterial hypertension. JCI Insight 2024;9:e177926. https:\/\/doi.org\/10.1172\/jci.insight.177926.","DOI":"10.1172\/jci.insight.177926"},{"key":"2026050115424109980_j_mr-2026-0002_ref_068","doi-asserted-by":"crossref","unstructured":"Rachedi, NS, Tang, Y, Tai, YY, Zhao, J, Chauvet, C, Grynblat, J, et al.. Dietary intake and glutamine-serine metabolism control pathologic vascular stiffness. 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SMYD2-methylated PPAR\u03b3 facilitates hypoxia-induced pulmonary hypertension by activating mitophagy. Circ Res 2024;135:93\u2013109. https:\/\/doi.org\/10.1161\/CIRCRESAHA.124.323698.","DOI":"10.1161\/CIRCRESAHA.124.323698"},{"key":"2026050115424109980_j_mr-2026-0002_ref_074","doi-asserted-by":"crossref","unstructured":"Niihori, M, James, J, Varghese, MV, McClain, N, Lawal, OS, Philip, RC, et al.. Mitochondria as a primary determinant of angiogenic modality in pulmonary arterial hypertension. J\u00a0Exp Med 2024;221:e20231568. https:\/\/doi.org\/10.1084\/jem.20231568.","DOI":"10.1084\/jem.20231568"},{"key":"2026050115424109980_j_mr-2026-0002_ref_075","doi-asserted-by":"crossref","unstructured":"Culley, MK, Zhao, J, Tai, YY, Tang, Y, Perk, D, Negi, V, et al.. Frataxin deficiency promotes endothelial senescence in pulmonary hypertension. J\u00a0Clin Investig 2021;131:e136459. https:\/\/doi.org\/10.1172\/JCI136459.","DOI":"10.1172\/JCI136459"},{"key":"2026050115424109980_j_mr-2026-0002_ref_076","doi-asserted-by":"crossref","unstructured":"Piper, B, Bogamuwa, S, Hossain, T, Farkas, D, Rosas, L, Green, AC, et al.. RAB7 deficiency impairs pulmonary artery endothelial function and promotes pulmonary hypertension. J\u00a0Clin Investig 2024;134:e169441. https:\/\/doi.org\/10.1172\/JCI169441.","DOI":"10.1172\/JCI169441"},{"key":"2026050115424109980_j_mr-2026-0002_ref_077","doi-asserted-by":"crossref","unstructured":"Fujiwara, T, Takeda, N, Hara, H, Ishii, S, Numata, G, Tokiwa, H, et al.. PGC-1\u03b1-mediated angiogenesis prevents pulmonary hypertension in mice. 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Acta Pharm Sin B 2022;12:2315\u201329. https:\/\/doi.org\/10.1016\/j.apsb.2021.12.025.","DOI":"10.1016\/j.apsb.2021.12.025"},{"key":"2026050115424109980_j_mr-2026-0002_ref_080","doi-asserted-by":"crossref","unstructured":"Liu, B, Peng, Y, Yi, D, Machireddy, N, Dong, D, Ramirez, K, et al.. Endothelial PHD2 deficiency induces nitrative stress via suppression of caveolin-1 in pulmonary hypertension. Eur Respir J 2022;60:2102643. https:\/\/doi.org\/10.1183\/13993003.02643-2021.","DOI":"10.1183\/13993003.02643-2021"},{"key":"2026050115424109980_j_mr-2026-0002_ref_081","doi-asserted-by":"crossref","unstructured":"Knight, H, Abis, G, Kaur, M, Green, HLH, Krasemann, S, Hartmann, K, et al.. Cyclin D-CDK4 disulfide bond attenuates pulmonary vascular cell proliferation. Circ Res 2023;133:966\u201388. https:\/\/doi.org\/10.1161\/CIRCRESAHA.122.321836.","DOI":"10.1161\/CIRCRESAHA.122.321836"},{"key":"2026050115424109980_j_mr-2026-0002_ref_082","doi-asserted-by":"crossref","unstructured":"Zhang, Y, Tian, X, Chen, L, Zhao, S, Tang, X, Liu, X, et al.. Endogenous hydrogen sulfide persulfidates endothelin type A receptor to inhibit pulmonary arterial smooth muscle cell proliferation. Redox Biol 2025;80:103493. https:\/\/doi.org\/10.1016\/j.redox.2025.103493.","DOI":"10.1016\/j.redox.2025.103493"},{"key":"2026050115424109980_j_mr-2026-0002_ref_083","doi-asserted-by":"crossref","unstructured":"M\u00fcller, M, Bischof, C, Kapries, T, Wollnitza, S, Liechty, C, Gei\u00dfen, S, et al.. Right heart failure in mice upon pressure overload is promoted by mitochondrial oxidative stress. JACC Basic Transl Sci 2022;7:658\u201377. https:\/\/doi.org\/10.1016\/j.jacbts.2022.02.018.","DOI":"10.1016\/j.jacbts.2022.02.018"},{"key":"2026050115424109980_j_mr-2026-0002_ref_084","doi-asserted-by":"crossref","unstructured":"Lemay, SE, Boucherat, O, Bonnet, S. Reverse reaction of nicotinamide nucleotide transhydrogenase promotes ROS production and contributes to right ventricular failure. JACC Basic Transl Sci 2022;7:678\u201380. https:\/\/doi.org\/10.1016\/j.jacbts.2022.03.015.","DOI":"10.1016\/j.jacbts.2022.03.015"},{"key":"2026050115424109980_j_mr-2026-0002_ref_085","doi-asserted-by":"crossref","unstructured":"Peng, Z, Luo, XY, Li, X, Li, Y, Wu, Y, Tian, Y, et al.. Cathepsin L promotes pulmonary hypertension via BMPR2\/GSDME-Mediated pyroptosis. Hypertension 2024;81:2430\u201343. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.124.22903.","DOI":"10.1161\/HYPERTENSIONAHA.124.22903"},{"key":"2026050115424109980_j_mr-2026-0002_ref_086","doi-asserted-by":"crossref","unstructured":"Kazmirczak, F, Vogel, NT, Prisco, SZ, Patterson, MT, Annis, J, Moon, RT, et al.. Ferroptosis mediated inflammation promotes pulmonary hypertension. Circ Res 2024;135:1067\u201383. https:\/\/doi.org\/10.1161\/CIRCRESAHA.123.324138.","DOI":"10.1161\/CIRCRESAHA.123.324138"},{"key":"2026050115424109980_j_mr-2026-0002_ref_087","doi-asserted-by":"crossref","unstructured":"Li, M, Hao, Y, Song, X, Liu, H, Zhang, C, Zhang, J, et al.. ca-circSCN8A promotes HPASMCs ferroptosis via LLPS initiated R-Loop. Hypertension 2025;82:e114\u201328. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.125.25036.","DOI":"10.1161\/HYPERTENSIONAHA.125.25036"},{"key":"2026050115424109980_j_mr-2026-0002_ref_088","doi-asserted-by":"crossref","unstructured":"Born, E, Lipskaia, L, Breau, M, Houssaini, A, Beaulieu, D, Marcos, E, et al.. Eliminating senescent cells can promote pulmonary hypertension development and progression. Circulation 2023;147:650\u201366. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.122.058794.","DOI":"10.1161\/CIRCULATIONAHA.122.058794"},{"key":"2026050115424109980_j_mr-2026-0002_ref_089","doi-asserted-by":"crossref","unstructured":"He, Y, Zuo, C, Jia, D, Bai, P, Kong, D, Chen, D, et al.. Loss of DP1 aggravates vascular remodeling in pulmonary arterial hypertension via mTORC1 signaling. Am J Respir Crit Care Med 2020;201:1263\u201376. https:\/\/doi.org\/10.1164\/rccm.201911-2137OC.","DOI":"10.1164\/rccm.201911-2137OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_090","doi-asserted-by":"crossref","unstructured":"Xu, H, Ye, L, Du, C, Tang, H, Zheng, Q, Zhu, C, et al.. EP4\/ANXA2 axis in pulmonary arterial hypertension: therapeutic implications. Eur Heart J Published online September 25, 2025:ehaf763. https:\/\/doi.org\/10.1093\/eurheartj\/ehaf763.","DOI":"10.1093\/eurheartj\/ehaf763"},{"key":"2026050115424109980_j_mr-2026-0002_ref_091","doi-asserted-by":"crossref","unstructured":"Xu, H, Fang, B, Bao, C, Mao, X, Zhu, C, Ye, L, et al.. The prostaglandin E2 receptor EP4 promotes vascular neointimal hyperplasia through translational control of tenascin C via the cAPM\/PKA\/mTORC1\/rpS6 pathway. 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NEDD9, a hypoxia-upregulated mediator for pathogenic platelet-endothelial cell interaction in pulmonary hypertension. Am J Respir Crit Care Med 2021;203:1455\u20138. https:\/\/doi.org\/10.1164\/rccm.202101-0007ED.","DOI":"10.1164\/rccm.202101-0007ED"},{"key":"2026050115424109980_j_mr-2026-0002_ref_095","doi-asserted-by":"crossref","unstructured":"Florentin, J, Zhao, J, Tai, YY, Sun, W, Ohayon, LL, O\u2019Neil, SP, et al.. Loss of amphiregulin drives inflammation and endothelial apoptosis in pulmonary hypertension. Life Sci Alliance 2022;5:e202101264. https:\/\/doi.org\/10.26508\/lsa.202101264.","DOI":"10.26508\/lsa.202101264"},{"key":"2026050115424109980_j_mr-2026-0002_ref_096","doi-asserted-by":"crossref","unstructured":"Harvey, LD, Alotaibi, M, Tai, YY, Tang, Y, Kim, HJ, Kelly, NJ, et al.. Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension. 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Circ Res 2025;137:46\u201366. https:\/\/doi.org\/10.1161\/CIRCRESAHA.125.326173.","DOI":"10.1161\/CIRCRESAHA.125.326173"},{"key":"2026050115424109980_j_mr-2026-0002_ref_103","doi-asserted-by":"crossref","unstructured":"Rong, W, Liu, C, Li, X, Wan, N, Wei, L, Zhu, W, et al.. Caspase-8 promotes pulmonary hypertension by activating macrophage-associated inflammation and IL-1\u03b2 (interleukin 1\u03b2) production. Arterioscler Thromb Vasc Biol 2022;42:613\u201331. https:\/\/doi.org\/10.1161\/ATVBAHA.121.317168.","DOI":"10.1161\/ATVBAHA.121.317168"},{"key":"2026050115424109980_j_mr-2026-0002_ref_104","doi-asserted-by":"crossref","unstructured":"Agrawal, V, Kropski, JA, Gokey, JJ, Kobeck, E, Murphy, MB, Murray, KT, et al.. Myeloid cell derived IL1\u03b2 contributes to pulmonary hypertension in HFpEF. 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Cardiovasc Diabetol 2025;24:268. https:\/\/doi.org\/10.1186\/s12933-025-02772-y.","DOI":"10.1186\/s12933-025-02772-y"},{"key":"2026050115424109980_j_mr-2026-0002_ref_107","doi-asserted-by":"crossref","unstructured":"Pham, AT, Oliveira, AC, Albanna, M, Alvarez-Castanon, J, Dupee, Z, Patel, D, et al.. Non-interferon-dependent role of STING signaling in pulmonary hypertension. Arterioscler Thromb Vasc Biol 2024;44:124\u201342. https:\/\/doi.org\/10.1161\/ATVBAHA.123.320121.","DOI":"10.1161\/ATVBAHA.123.320121"},{"key":"2026050115424109980_j_mr-2026-0002_ref_108","doi-asserted-by":"crossref","unstructured":"Huang, L, Zhang, H, Liu, Y, Long, Y. The role of gut and airway microbiota in pulmonary arterial hypertension. Front Microbiol 2022;13:929752. https:\/\/doi.org\/10.3389\/fmicb.2022.929752.","DOI":"10.3389\/fmicb.2022.929752"},{"key":"2026050115424109980_j_mr-2026-0002_ref_109","doi-asserted-by":"crossref","unstructured":"Huang, Y, Lin, F, Tang, R, Bao, C, Zhou, Q, Ye, K, et al.. Gut microbial metabolite trimethylamine N-oxide aggravates pulmonary hypertension. Am J Respir Cell Mol Biol 2022;66:452\u201360. https:\/\/doi.org\/10.1165\/rcmb.2021-0414OC.","DOI":"10.1165\/rcmb.2021-0414OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_220","doi-asserted-by":"crossref","unstructured":"Son, KH, Oh, S, Jang, AY, Byun, K, Chung, WJ. Pyridoxamine attenuates PAH by inhibiting the receptor for advanced glycation end products in macrophages. Right Heart J 2026;1:e5. https:\/\/doi.org\/10.65721\/rhj.2026.1.e52.","DOI":"10.65721\/rhj.2026.1.e5"},{"key":"2026050115424109980_j_mr-2026-0002_ref_221","doi-asserted-by":"crossref","unstructured":"Kim, HJ. Macrophage-AGE-RAGE axis, a promising therapeutic target in pulmonary arterial hypertension. 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Hypertension 2023;80:1784\u201394. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.123.21142.","DOI":"10.1161\/HYPERTENSIONAHA.123.21142"},{"key":"2026050115424109980_j_mr-2026-0002_ref_116","doi-asserted-by":"crossref","unstructured":"Shu, T, Zhang, J, Zhou, Y, Chen, Z, Li, J, Tang, Q, et al.. Eosinophils protect against pulmonary hypertension through 14-HDHA and 17-HDHA. Eur Respir J 2023;61:2200582. https:\/\/doi.org\/10.1183\/13993003.00582-2022.","DOI":"10.1183\/13993003.00582-2022"},{"key":"2026050115424109980_j_mr-2026-0002_ref_117","doi-asserted-by":"crossref","unstructured":"Zhakeer, G, Zeng, Y, Maimaitiaili, N, Ju, P, Yao, H, Shi, Y, et al.. Treg cells attenuate pulmonary venous remodeling in PH-LHD via NLRC3 signaling. 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Hypertension 2025;82:2040\u201354. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.125.25284.","DOI":"10.1161\/HYPERTENSIONAHA.125.25284"},{"key":"2026050115424109980_j_mr-2026-0002_ref_120","doi-asserted-by":"crossref","unstructured":"Ferrian, S, Cao, A, McCaffrey, EF, Saito, T, Greenwald, NF, Nicolls, MR, et al.. Single-cell imaging maps inflammatory cell subsets to pulmonary arterial hypertension vasculopathy. Am J Respir Crit Care Med 2024;209:206\u201318. https:\/\/doi.org\/10.1164\/rccm.202209-1761OC.","DOI":"10.1164\/rccm.202209-1761OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_121","doi-asserted-by":"crossref","unstructured":"Guo, Y, He, Z, Chen, Z, Chen, F, Wang, C, Zhou, W, et al.. Inhibition of Th17 cells by donepezil ameliorates experimental lung fibrosis and pulmonary hypertension. Theranostics 2023;13:1826\u201342. https:\/\/doi.org\/10.7150\/thno.82069.","DOI":"10.7150\/thno.82069"},{"key":"2026050115424109980_j_mr-2026-0002_ref_122","doi-asserted-by":"crossref","unstructured":"Wang, L, Mu, M, Guo, Y, Huang, J, Zhang, R, Zhang, M, et al.. PD-1\/PD-L1 provides protective role in hypoxia-induced pulmonary vascular remodeling. Hypertension 2024;81:1822\u201336. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.123.22393.","DOI":"10.1161\/HYPERTENSIONAHA.123.22393"},{"key":"2026050115424109980_j_mr-2026-0002_ref_123","doi-asserted-by":"crossref","unstructured":"Wu, LW, Chen, M, Jiang, DJ, Jiang, CY, Liu, YW, Feng, B, et al.. TCF7 enhances pulmonary hypertension by boosting stressed natural killer cells and their interaction with pulmonary arterial smooth muscle cells. Respir Res 2025;26:202. https:\/\/doi.org\/10.1186\/s12931-025-03276-9.","DOI":"10.1186\/s12931-025-03276-9"},{"key":"2026050115424109980_j_mr-2026-0002_ref_124","doi-asserted-by":"crossref","unstructured":"Jones, RJ, De Bie, EMDD, Groves, E, Zalewska, KI, Swietlik, EM, Treacy, CM, et al.. Autoimmunity is a significant feature of idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med 2022;206:81\u201393. https:\/\/doi.org\/10.1164\/rccm.202108-1919OC.","DOI":"10.1164\/rccm.202108-1919OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_125","doi-asserted-by":"crossref","unstructured":"Kariotis, S, Jammeh, E, Swietlik, EM, Pickworth, JA, Rhodes, CJ, Otero, P, et al.. Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood. Nat Commun 2021;12:7104. https:\/\/doi.org\/10.1038\/s41467-021-27326-0.","DOI":"10.1038\/s41467-021-27326-0"},{"key":"2026050115424109980_j_mr-2026-0002_ref_126","doi-asserted-by":"crossref","unstructured":"Sanges, S, Guerrier, T, Duhamel, A, Guilbert, L, Hauspie, C, Largy, A, et al.. Soluble markers of B\u00a0cell activation suggest a role of B\u00a0cells in the pathogenesis of systemic sclerosis-associated pulmonary arterial hypertension. Front Immunol 2022;13:954007. https:\/\/doi.org\/10.3389\/fimmu.2022.954007.","DOI":"10.3389\/fimmu.2022.954007"},{"key":"2026050115424109980_j_mr-2026-0002_ref_127","doi-asserted-by":"crossref","unstructured":"Shu, T, Liu, Y, Zhou, Y, Zhou, Z, Li, B, Xing, Y, et al.. Inhibition of immunoglobulin E attenuates pulmonary hypertension. Nat Cardiovasc Res 2022;1:665\u201378. https:\/\/doi.org\/10.1038\/s44161-022-00095-9.","DOI":"10.1038\/s44161-022-00095-9"},{"key":"2026050115424109980_j_mr-2026-0002_ref_128","doi-asserted-by":"crossref","unstructured":"Yeh, FC, Chen, CN, Xie, CY, Baxan, N, Zhao, L, Ashek, A, et al.. TLR7\/8 activation induces autoimmune vasculopathy and causes severe pulmonary arterial hypertension. Eur Respir J 2023;62:2300204. https:\/\/doi.org\/10.1183\/13993003.00204-2023.","DOI":"10.1183\/13993003.00204-2023"},{"key":"2026050115424109980_j_mr-2026-0002_ref_129","doi-asserted-by":"crossref","unstructured":"Frid, MG, McKeon, BA, Thurman, JM, Maron, BA, Li, M, Zhang, H, et al.. Immunoglobulin-driven complement activation regulates proinflammatory remodeling in pulmonary hypertension. Am J Respir Crit Care Med 2020;201:224\u201339. https:\/\/doi.org\/10.1164\/rccm.201903-0591OC.","DOI":"10.1164\/rccm.201903-0591OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_130","doi-asserted-by":"crossref","unstructured":"Prasad, RR, Kumar, S, Zhang, H, Li, M, Hu, CJ, Riddle, S, et al.. An intracellular complement system drives metabolic and proinflammatory reprogramming of vascular fibroblasts in pulmonary hypertension. JCI Insight 2025;10:e184141. https:\/\/doi.org\/10.1172\/jci.insight.184141.","DOI":"10.1172\/jci.insight.184141"},{"key":"2026050115424109980_j_mr-2026-0002_ref_131","doi-asserted-by":"crossref","unstructured":"Zhang, ZJ, Wang, HF, Lian, TY, Zhou, YP, Xu, XQ, Guo, F, et al.. Human plasma IgG N-glycome profiles reveal a proinflammatory phenotype in chronic thromboembolic pulmonary hypertension. Hypertension 2023;80:1929\u201339. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.123.21408.","DOI":"10.1161\/HYPERTENSIONAHA.123.21408"},{"key":"2026050115424109980_j_mr-2026-0002_ref_132","doi-asserted-by":"crossref","unstructured":"Wang, Z, Chen, J, Babicheva, A, Jain, PP, Rodriguez, M, Ayon, RJ, et al.. Endothelial upregulation of mechanosensitive channel Piezo1 in pulmonary hypertension. Am J Physiol Cell Physiol 2021;321:C1010\u201327. https:\/\/doi.org\/10.1152\/ajpcell.00147.2021.","DOI":"10.1152\/ajpcell.00147.2021"},{"key":"2026050115424109980_j_mr-2026-0002_ref_133","doi-asserted-by":"crossref","unstructured":"Knoepp, F, Abid, S, Houssaini, A, Lipskaia, L, G\u00f6kyildirim, MY, Born, E, et al.. Piezo1 in PASMCs: critical for hypoxia-induced pulmonary hypertension development. Circ Res 2025;136:1031\u201348. https:\/\/doi.org\/10.1161\/CIRCRESAHA.124.325475.","DOI":"10.1161\/CIRCRESAHA.124.325475"},{"key":"2026050115424109980_j_mr-2026-0002_ref_134","doi-asserted-by":"crossref","unstructured":"Wei, F, Lin, Z, Lu, W, Luo, H, Feng, H, Liu, S, et al.. Deficiency of endothelial Piezo2 impairs pulmonary vascular angiogenesis and predisposes pulmonary hypertension. Hypertension 2025;82:583\u201397. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.124.22948.","DOI":"10.1161\/HYPERTENSIONAHA.124.22948"},{"key":"2026050115424109980_j_mr-2026-0002_ref_135","doi-asserted-by":"crossref","unstructured":"Shinohara, T, Moonen, JR, Chun, YH, Lee-Yow, YC, Okamura, K, Szafron, JM, et al.. High shear stress reduces ERG causing endothelial-mesenchymal transition and pulmonary arterial hypertension. 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J\u00a0Nanobiotechnol 2024;22:492. https:\/\/doi.org\/10.1186\/s12951-024-02738-4.","DOI":"10.1186\/s12951-024-02738-4"},{"key":"2026050115424109980_j_mr-2026-0002_ref_138","doi-asserted-by":"crossref","unstructured":"Xiao, R, Luo, S, Zhang, T, Lv, Y, Wang, T, Zhang, J, et al.. Peptide blocking self-polymerization of extracellular calcium-sensing receptor attenuates hypoxia-induced pulmonary hypertension. Hypertension 2021;78:1605\u201316. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.120.16712.","DOI":"10.1161\/HYPERTENSIONAHA.120.16712"},{"key":"2026050115424109980_j_mr-2026-0002_ref_139","doi-asserted-by":"crossref","unstructured":"Liu, B, Wei, YP, Fan, X, Hu, X, Chen, Z, Liu, X, et al.. Calcium sensing receptor variants increase pulmonary hypertension susceptibility. 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Transl Res J Lab Clin Med 2021;237:63\u201381. https:\/\/doi.org\/10.1016\/j.trsl.2021.05.007.","DOI":"10.1016\/j.trsl.2021.05.007"},{"key":"2026050115424109980_j_mr-2026-0002_ref_142","doi-asserted-by":"crossref","unstructured":"Fayyaz, AU, Sabbah, MS, Dasari, S, Griffiths, LG, DuBrock, HM, Wang, Y, et al.. Histologic and proteomic remodeling of the pulmonary veins and arteries in a porcine model of chronic pulmonary venous hypertension. Cardiovasc Res 2023;119:268\u201382. https:\/\/doi.org\/10.1093\/cvr\/cvac005.","DOI":"10.1093\/cvr\/cvac005"},{"key":"2026050115424109980_j_mr-2026-0002_ref_143","doi-asserted-by":"crossref","unstructured":"Quarck, R, Willems, L, Tielemans, B, Stoian, L, Ronisz, A, Wagenaar, A, et al.. Impairment of angiogenesis-driven clot resolution is a key event in the progression to chronic thromboembolic pulmonary hypertension: validation in a novel rabbit model. Arterioscler Thromb Vasc Biol 2023;43:1308\u201321. https:\/\/doi.org\/10.1161\/ATVBAHA.122.317262.","DOI":"10.1161\/ATVBAHA.122.317262"},{"key":"2026050115424109980_j_mr-2026-0002_ref_144","doi-asserted-by":"crossref","unstructured":"Hadzic, S, Wu, CY, Gredic, M, Pak, O, Loku, E, Kraut, S, et al.. Fibroblast growth factor 10 reverses cigarette smoke- and elastase-induced emphysema and pulmonary hypertension in mice. Eur Respir J 2023;62:2201606. https:\/\/doi.org\/10.1183\/13993003.01606-2022.","DOI":"10.1183\/13993003.01606-2022"},{"key":"2026050115424109980_j_mr-2026-0002_ref_145","doi-asserted-by":"crossref","unstructured":"Jani, V, Aslam, MI, Fenwick, AJ, Ma, W, Gong, H, Milburn, G, et al.. Right ventricular sarcomere contractile depression and the role of thick filament activation in human heart failure with pulmonary hypertension. Circulation 2023;147:1919\u201332. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.123.064717.","DOI":"10.1161\/CIRCULATIONAHA.123.064717"},{"key":"2026050115424109980_j_mr-2026-0002_ref_146","doi-asserted-by":"crossref","unstructured":"Janowski, AM, Ravellette, KS, Insel, M, Garcia, JGN, Rischard, FP, Vanderpool, RR. Advanced hemodynamic and cluster analysis for identifying novel RV function subphenotypes in patients with pulmonary hypertension. J\u00a0Heart Lung Transplant Off Publ Int Soc Heart Transplant 2024;43:755\u201370. https:\/\/doi.org\/10.1016\/j.healun.2023.12.009.","DOI":"10.1016\/j.healun.2023.12.009"},{"key":"2026050115424109980_j_mr-2026-0002_ref_147","doi-asserted-by":"crossref","unstructured":"Ichimura, K, Boehm, M, Andruska, AM, Zhang, F, Schimmel, K, Bonham, S, et al.. 3D imaging reveals complex microvascular remodeling in the right ventricle in pulmonary hypertension. Circ Res 2024;135:60\u201375. https:\/\/doi.org\/10.1161\/CIRCRESAHA.123.323546.","DOI":"10.1161\/CIRCRESAHA.123.323546"},{"key":"2026050115424109980_j_mr-2026-0002_ref_148","doi-asserted-by":"crossref","unstructured":"Wu, LW, Chen, M, Jiang, CY, Jiang, DJ, Zhang, X, Xu, XH, et al.. Inactivation of AXL in cardiac fibroblasts alleviates right ventricular remodeling in pulmonary hypertension. Adv Sci Weinh Baden-Wurtt Ger 2026;13:e08995. https:\/\/doi.org\/10.1002\/advs.202508995.","DOI":"10.1002\/advs.202508995"},{"key":"2026050115424109980_j_mr-2026-0002_ref_149","doi-asserted-by":"crossref","unstructured":"Savale, L, Akagi, S, Tu, L, Cumont, A, Thuillet, R, Phan, C, et al.. Serum and pulmonary uric acid in pulmonary arterial hypertension. Eur Respir J 2021;58:2000332. https:\/\/doi.org\/10.1183\/13993003.00332-2020.","DOI":"10.1183\/13993003.00332-2020"},{"key":"2026050115424109980_j_mr-2026-0002_ref_150","doi-asserted-by":"crossref","unstructured":"Lluci\u00e0-Valldeperas, A, van Wezenbeek, J, Groeneveldt, JA, Smal, R, S\u00e1nchez-Duffhues, G, Becher, C, et al.. Bone morphogenetic protein 10 is increased in pre-capillary pulmonary hypertension patients. Cardiovasc Res 2025;121:1254\u201368. https:\/\/doi.org\/10.1093\/cvr\/cvaf028.","DOI":"10.1093\/cvr\/cvaf028"},{"key":"2026050115424109980_j_mr-2026-0002_ref_151","doi-asserted-by":"crossref","unstructured":"Guignabert, C, Savale, L, Boucly, A, Thuillet, R, Tu, L, Ottaviani, M, et al.. Serum and pulmonary expression profiles of the activin signaling system in pulmonary arterial hypertension. Circulation 2023;147:1809\u201322. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.122.061501.","DOI":"10.1161\/CIRCULATIONAHA.122.061501"},{"key":"2026050115424109980_j_mr-2026-0002_ref_152","doi-asserted-by":"crossref","unstructured":"Harbaum, L, Rhodes, CJ, Wharton, J, Lawrie, A, Karnes, JH, Desai, AA, et al.. Mining the plasma proteome for insights into the molecular pathology of pulmonary arterial hypertension. Am J Respir Crit Care Med 2022;205:1449\u201360. https:\/\/doi.org\/10.1164\/rccm.202109-2106OC.","DOI":"10.1164\/rccm.202109-2106OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_153","doi-asserted-by":"crossref","unstructured":"Le Ribeuz, H, Masson, B, Capuano, V, Dutheil, M, Gooroochurn, H, Bo\u00ebt, A, et al.. SUR1 as a new therapeutic target for pulmonary arterial hypertension. Am J Respir Cell Mol Biol 2022;66:539\u201354. https:\/\/doi.org\/10.1165\/rcmb.2021-0180OC.","DOI":"10.1165\/rcmb.2021-0180OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_154","doi-asserted-by":"crossref","unstructured":"Bian, JS, Chen, J, Zhang, J, Tan, J, Chen, Y, Yang, X, et al.. ErbB3 governs endothelial dysfunction in hypoxia-induced pulmonary hypertension. Circulation 2024;150:1533\u201353. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.123.067005.","DOI":"10.1161\/CIRCULATIONAHA.123.067005"},{"key":"2026050115424109980_j_mr-2026-0002_ref_155","doi-asserted-by":"crossref","unstructured":"Boucly, A, Tu, L, Guignabert, C, Rhodes, C, De Groote, P, Pr\u00e9vot, G, et al.. Cytokines as prognostic biomarkers in pulmonary arterial hypertension. Eur Respir J 2023;61:2201232. https:\/\/doi.org\/10.1183\/13993003.01232-2022.","DOI":"10.1183\/13993003.01232-2022"},{"key":"2026050115424109980_j_mr-2026-0002_ref_156","doi-asserted-by":"crossref","unstructured":"Jiang, CY, Wu, LW, Liu, YW, Feng, B, Ye, LC, Huang, X, et al.. Identification of ACKR4 as an immune checkpoint in pulmonary arterial hypertension. Front Immunol 2023;14:1153573. https:\/\/doi.org\/10.3389\/fimmu.2023.1153573.","DOI":"10.3389\/fimmu.2023.1153573"},{"key":"2026050115424109980_j_mr-2026-0002_ref_157","doi-asserted-by":"crossref","unstructured":"Kong, D, Liu, J, Lu, J, Zeng, C, Chen, H, Duan, Z, et al.. HMGB2 release promotes pulmonary hypertension and predicts severity and mortality of patients with pulmonary arterial hypertension. Arterioscler Thromb Vasc Biol 2024;44:e172\u201395. https:\/\/doi.org\/10.1161\/ATVBAHA.123.319916.","DOI":"10.1161\/ATVBAHA.123.319916"},{"key":"2026050115424109980_j_mr-2026-0002_ref_158","doi-asserted-by":"crossref","unstructured":"He, YY, Xie, XM, Zhang, HD, Ye, J, Gencer, S, van der Vorst, EPC, et al.. Identification of hypoxia induced metabolism associated genes in pulmonary hypertension. Front Pharmacol 2021;12:753727. https:\/\/doi.org\/10.3389\/fphar.2021.753727.","DOI":"10.3389\/fphar.2021.753727"},{"key":"2026050115424109980_j_mr-2026-0002_ref_159","doi-asserted-by":"crossref","unstructured":"Smits, AJ, Arkani, M, In \u2019t Veld, SGJG, Huis In \u2019t Veld, AE, Sol, N, Groeneveldt, JA, et al.. Distinct platelet ribonucleic acid signatures in patients with pulmonary hypertension. Ann Am Thorac Soc 2022;19:1650\u201360. https:\/\/doi.org\/10.1513\/AnnalsATS.202201-085OC.","DOI":"10.1513\/AnnalsATS.202201-085OC"},{"key":"2026050115424109980_j_mr-2026-0002_ref_160","doi-asserted-by":"crossref","unstructured":"Brusca, SB, Elinoff, JM, Zou, Y, Jang, MK, Kong, H, Demirkale, CY, et al.. Plasma cell-free DNA predicts survival and maps specific sources of injury in pulmonary arterial hypertension. Circulation 2022;146:1033\u201345. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.121.056719.","DOI":"10.1161\/CIRCULATIONAHA.121.056719"},{"key":"2026050115424109980_j_mr-2026-0002_ref_161","doi-asserted-by":"crossref","unstructured":"Khassafi, F, Chelladurai, P, Valasarajan, C, Nayakanti, SR, Martineau, S, Sommer, N, et al.. Transcriptional profiling unveils molecular subgroups of adaptive and maladaptive right ventricular remodeling in pulmonary hypertension. Nat Cardiovasc Res 2023;2:917\u201336. https:\/\/doi.org\/10.1038\/s44161-023-00338-3.","DOI":"10.1038\/s44161-023-00338-3"},{"key":"2026050115424109980_j_mr-2026-0002_ref_162","doi-asserted-by":"crossref","unstructured":"Klouda, T, Kim, Y, Baek, SH, Bhaumik, M, Li, Y, Liu, Y, et al.. Specialized pericyte subtypes in the pulmonary capillaries. EMBO J 2025;44:1074\u2013106. https:\/\/doi.org\/10.1038\/s44318-024-00349-1.","DOI":"10.1038\/s44318-024-00349-1"},{"key":"2026050115424109980_j_mr-2026-0002_ref_163","doi-asserted-by":"crossref","unstructured":"He, YY, Yan, Y, Chen, JW, Liu, S, Hua, L, Jiang, X, et al.. Plasma metabolomics in the perioperative period of defect repair in patients with pulmonary arterial hypertension associated with congenital heart disease. Acta Pharmacol Sin 2022;43:1710\u201320. https:\/\/doi.org\/10.1038\/s41401-021-00804-3.","DOI":"10.1038\/s41401-021-00804-3"},{"key":"2026050115424109980_j_mr-2026-0002_ref_164","doi-asserted-by":"crossref","unstructured":"Evans, JDW, Girerd, B, Montani, D, Wang, XJ, Gali\u00e8, N, Austin, ED, et al.. BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis. Lancet Respir Med 2016;4:129\u201337. https:\/\/doi.org\/10.1016\/S2213-2600(15)00544-5.","DOI":"10.1016\/S2213-2600(15)00544-5"},{"key":"2026050115424109980_j_mr-2026-0002_ref_165","doi-asserted-by":"crossref","unstructured":"Gr\u00fcnig, E, Koehler, R, Miltenberger-Miltenyi, G, Zimmermann, R, Gorenflo, M, Mereles, D, et al.. Primary pulmonary hypertension in children may have a different genetic background than in adults. Pediatr Res 2004;56:571\u20138. https:\/\/doi.org\/10.1203\/01.PDR.0000139481.20847.D0.","DOI":"10.1203\/01.PDR.0000139481.20847.D0"},{"key":"2026050115424109980_j_mr-2026-0002_ref_166","doi-asserted-by":"crossref","unstructured":"Welch, CL, Austin, ED, Chung, WK. Genes that drive the pathobiology of pediatric pulmonary arterial hypertension. Pediatr Pulmonol 2021;56:614\u201320. https:\/\/doi.org\/10.1002\/ppul.24637.","DOI":"10.1002\/ppul.24637"},{"key":"2026050115424109980_j_mr-2026-0002_ref_167","doi-asserted-by":"crossref","unstructured":"Zhu, N, Gonzaga-Jauregui, C, Welch, CL, Ma, L, Qi, H, King, AK, et al.. Exome sequencing in children with pulmonary arterial hypertension demonstrates differences compared with adults. Circ Genomic Precis Med 2018;11:e001887. https:\/\/doi.org\/10.1161\/CIRCGEN.117.001887.","DOI":"10.1161\/CIRCGEN.117.001887"},{"key":"2026050115424109980_j_mr-2026-0002_ref_168","doi-asserted-by":"crossref","unstructured":"Kerstjens-Frederikse, WS, Bongers, EMHF, Roofthooft, MTR, Leter, EM, Douwes, JM, Van Dijk, A, et al.. TBX4 mutations (small patella syndrome) are associated with childhood-onset pulmonary arterial hypertension. J\u00a0Med Genet 2013;50:500\u20136. https:\/\/doi.org\/10.1136\/jmedgenet-2012-101152.","DOI":"10.1136\/jmedgenet-2012-101152"},{"key":"2026050115424109980_j_mr-2026-0002_ref_169","doi-asserted-by":"crossref","unstructured":"Haarman, MG, Kerstjens-Frederikse, WS, Berger, RMF. The ever-expanding phenotypical spectrum of human TBX4 mutations: from toe to lung. Eur Respir J 2019;54:1901504. https:\/\/doi.org\/10.1183\/13993003.01504-2019.","DOI":"10.1183\/13993003.01504-2019"},{"key":"2026050115424109980_j_mr-2026-0002_ref_170","doi-asserted-by":"crossref","unstructured":"Jiang, DJ, Yang, YJ, Wang, YZ, Zhang, X, Chan, WX, Yu, TT, et al.. Whole exome sequencing unravels genetic architecture and its clinical implications in pediatric pulmonary arterial hypertension. Int J Cardiol 2025;437:133515. https:\/\/doi.org\/10.1016\/j.ijcard.2025.133515.","DOI":"10.1016\/j.ijcard.2025.133515"},{"key":"2026050115424109980_j_mr-2026-0002_ref_171","doi-asserted-by":"crossref","unstructured":"Zhu, N, Welch, CL, Wang, J, Allen, PM, Gonzaga-Jauregui, C, Ma, L, et al.. Rare variants in SOX17 are associated with pulmonary arterial hypertension with congenital heart disease. Genome Med 2018;10:56. https:\/\/doi.org\/10.1186\/s13073-018-0566-x.","DOI":"10.1186\/s13073-018-0566-x"},{"key":"2026050115424109980_j_mr-2026-0002_ref_172","doi-asserted-by":"crossref","unstructured":"Levy, M, Eyries, M, Szezepanski, I, Ladouceur, M, Nadaud, S, Bonnet, D, et al.. Genetic analyses in a cohort of children with pulmonary hypertension. Eur Respir J 2016;48:1118\u201326. https:\/\/doi.org\/10.1183\/13993003.00211-2016.","DOI":"10.1183\/13993003.00211-2016"},{"key":"2026050115424109980_j_mr-2026-0002_ref_173","doi-asserted-by":"crossref","unstructured":"Fujiwara, M, Yagi, H, Matsuoka, R, Akimoto, K, Furutani, M, Imamura, S, et al.. Implications of mutations of activin receptor-like kinase 1 gene (ALK1) in addition to bone morphogenetic protein receptor II gene (BMPR2) in children with pulmonary arterial hypertension. Circ J Off J Jpn Circ Soc 2008;72:127\u201333. https:\/\/doi.org\/10.1253\/circj.72.127.","DOI":"10.1253\/circj.72.127"},{"key":"2026050115424109980_j_mr-2026-0002_ref_174","doi-asserted-by":"crossref","unstructured":"Harrison, RE, Berger, R, Haworth, SG, Tulloh, R, Mache, CJ, Morrell, NW, et al.. Transforming growth factor-beta receptor mutations and pulmonary arterial hypertension in childhood. Circulation 2005;111:435\u201341. https:\/\/doi.org\/10.1161\/01.CIR.0000153798.78540.87.","DOI":"10.1161\/01.CIR.0000153798.78540.87"},{"key":"2026050115424109980_j_mr-2026-0002_ref_175","doi-asserted-by":"crossref","unstructured":"Ma, L, Roman-Campos, D, Austin, ED, Eyries, M, Sampson, KS, Soubrier, F, et al.. A novel channelopathy in pulmonary arterial hypertension. N\u00a0Engl J Med 2013;369:351\u201361. https:\/\/doi.org\/10.1056\/NEJMoa1211097.","DOI":"10.1056\/NEJMoa1211097"},{"key":"2026050115424109980_j_mr-2026-0002_ref_176","doi-asserted-by":"crossref","unstructured":"Afolayan, AJ, Eis, A, Alexander, M, Michalkiewicz, T, Teng, RJ, Lakshminrusimha, S, et al.. Decreased endothelial nitric oxide synthase expression and function contribute to impaired mitochondrial biogenesis and oxidative stress in fetal lambs with persistent pulmonary hypertension. Am J Physiol Lung Cell Mol Physiol 2016;310:L40\u20139. https:\/\/doi.org\/10.1152\/ajplung.00392.2014.","DOI":"10.1152\/ajplung.00392.2014"},{"key":"2026050115424109980_j_mr-2026-0002_ref_177","doi-asserted-by":"crossref","unstructured":"Ambalavanan, N, Mariani, G, Bulger, A, Philips, IIIJB. Role of nitric oxide in regulating neonatal porcine pulmonary artery smooth muscle cell proliferation. Biol Neonate 1999;76:291\u2013300. https:\/\/doi.org\/10.1159\/000014171.","DOI":"10.1159\/000014171"},{"key":"2026050115424109980_j_mr-2026-0002_ref_178","doi-asserted-by":"crossref","unstructured":"Lassus, P, Turanlahti, M, Heikkil\u00e4, P, Andersson, LC, Nupponen, I, Sarnesto, A, et al.. Pulmonary vascular endothelial growth factor and Flt-1 in fetuses, in acute and chronic lung disease, and in persistent pulmonary hypertension of the newborn. Am J Respir Crit Care Med 2001;164:1981\u20137. https:\/\/doi.org\/10.1164\/ajrccm.164.10.2012036.","DOI":"10.1164\/ajrccm.164.10.2012036"},{"key":"2026050115424109980_j_mr-2026-0002_ref_179","doi-asserted-by":"crossref","unstructured":"Li, X, Hegarty, K, Lin, F, Chang, JL, Abdalla, A, Dhanabalan, K, et al.. Endothelial Cpt1a inhibits neonatal hyperoxia-induced pulmonary vascular remodeling by repressing endothelial-mesenchymal transition. Adv Sci Weinh Baden-Wurtt Ger 2025;12:e2415824. https:\/\/doi.org\/10.1002\/advs.202415824.","DOI":"10.1002\/advs.202415824"},{"key":"2026050115424109980_j_mr-2026-0002_ref_180","doi-asserted-by":"crossref","unstructured":"Wang, T, Xing, Y, Peng, B, Yang, K, Zhang, C, Chen, Y, et al.. Respiratory microbiome profile of pediatric pulmonary hypertension patients associated with congenital heart disease. Hypertension 2023;80:214\u201326. https:\/\/doi.org\/10.1161\/HYPERTENSIONAHA.122.19182.","DOI":"10.1161\/HYPERTENSIONAHA.122.19182"},{"key":"2026050115424109980_j_mr-2026-0002_ref_181","doi-asserted-by":"crossref","unstructured":"Ma, R, Cheng, L, Song, Y, Sun, Y, Gui, W, Deng, Y, et al.. Altered lung microbiome and metabolome profile in children with pulmonary arterial hypertension associated with congenital heart disease. Front Med 2022;9:940784. https:\/\/doi.org\/10.3389\/fmed.2022.940784.","DOI":"10.3389\/fmed.2022.940784"},{"key":"2026050115424109980_j_mr-2026-0002_ref_182","doi-asserted-by":"crossref","unstructured":"Sun, HX, Li, GJ, Du, ZH, Bing, Z, Ji, ZX, Luo, G, et al.. The relationship between endothelial progenitor cells and pulmonary arterial hypertension in children with congenital heart disease. BMC Pediatr 2019;19:502. https:\/\/doi.org\/10.1186\/s12887-019-1884-x.","DOI":"10.1186\/s12887-019-1884-x"},{"key":"2026050115424109980_j_mr-2026-0002_ref_183","doi-asserted-by":"crossref","unstructured":"Smadja, DM, Mauge, L, Gaussem, P, d\u2019Audigier, C, Israel-Biet, D, Celermajer, DS, et al.. Treprostinil increases the number and angiogenic potential of endothelial progenitor cells in children with pulmonary hypertension. Angiogenesis 2011;14:17\u201327. https:\/\/doi.org\/10.1007\/s10456-010-9192-y.","DOI":"10.1007\/s10456-010-9192-y"},{"key":"2026050115424109980_j_mr-2026-0002_ref_184","doi-asserted-by":"crossref","unstructured":"Alkon, J, Humpl, T, Manlhiot, C, McCrindle, BW, Reyes, JT, Friedberg, MK. Usefulness of the right ventricular systolic to diastolic duration ratio to predict functional capacity and survival in children with pulmonary arterial hypertension. Am J Cardiol 2010;106:430\u20136. https:\/\/doi.org\/10.1016\/j.amjcard.2010.03.048.","DOI":"10.1016\/j.amjcard.2010.03.048"},{"key":"2026050115424109980_j_mr-2026-0002_ref_185","doi-asserted-by":"crossref","unstructured":"Wang, S, Ye, L, Hong, H, Tang, C, Li, M, Zhang, Z, et al.. A neonatal rat model of increased right ventricular afterload by pulmonary artery banding. J\u00a0Thorac Cardiovasc Surg 2017;154:1734\u20139. https:\/\/doi.org\/10.1016\/j.jtcvs.2017.06.016.","DOI":"10.1016\/j.jtcvs.2017.06.016"},{"key":"2026050115424109980_j_mr-2026-0002_ref_186","doi-asserted-by":"crossref","unstructured":"Li, D, Hong, H, Li, M, Xu, X, Wang, S, Xiao, Y, et al.. A surgical mouse model of neonatal right ventricular outflow tract obstruction by pulmonary artery banding. J\u00a0Heart Lung Transplant Off Publ Int Soc Heart Transplant 2024;43:496\u2013507. https:\/\/doi.org\/10.1016\/j.healun.2023.10.009.","DOI":"10.1016\/j.healun.2023.10.009"},{"key":"2026050115424109980_j_mr-2026-0002_ref_187","doi-asserted-by":"crossref","unstructured":"Li, D, Qiu, L, Hong, H, Zhao, P, Xiao, Y, Zhang, H, et al.. A neonatal rat model of pulmonary vein stenosis. Cell Biosci 2023;13:112. https:\/\/doi.org\/10.1186\/s13578-023-01058-8.","DOI":"10.1186\/s13578-023-01058-8"},{"key":"2026050115424109980_j_mr-2026-0002_ref_188","doi-asserted-by":"crossref","unstructured":"Li, D, Xiao, Y, Ruan, P, Wan, Z, Hu, Y, Sun, S, et al.. Evaluation of treatment effect of sirolimus on pediatric pulmonary vein stenosis using a neonatal rat model. JACC, Basic Transl Sci 2025;10:101229. https:\/\/doi.org\/10.1016\/j.jacbts.2025.01.010.","DOI":"10.1016\/j.jacbts.2025.01.010"},{"key":"2026050115424109980_j_mr-2026-0002_ref_189","doi-asserted-by":"crossref","unstructured":"Daly, CM, Griffiths, M, Simpson, CE, Yang, J, Damico, RL, Vaidya, RD, et al.. Angiostatic peptide, endostatin, predicts severity in pediatric congenital heart disease-associated pulmonary hypertension. J\u00a0Am Heart Assoc 2021;10:e021409. https:\/\/doi.org\/10.1161\/JAHA.120.021409.","DOI":"10.1161\/JAHA.120.021409"},{"key":"2026050115424109980_j_mr-2026-0002_ref_190","doi-asserted-by":"crossref","unstructured":"Zeng, H, Lan, B, Li, B, Xie, H, Zhao, E, Liu, X, et al.. The role and mechanism of thrombospondin-4 in pulmonary arterial hypertension associated with congenital heart disease. Respir Res 2024;25:313. https:\/\/doi.org\/10.1186\/s12931-024-02932-w.","DOI":"10.1186\/s12931-024-02932-w"},{"key":"2026050115424109980_j_mr-2026-0002_ref_191","doi-asserted-by":"crossref","unstructured":"Zhou, Jjing, Jing-Li, Luo, Clei, He, Yhua, Qian, Yling. NEK2 inhibition reverses vascular remodeling in pulmonary arterial hypertension associated with congenital heart disease. 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Society for Cardiovascular Magnetic Resonance\/European Society of Cardiovascular Imaging\/American Society of Echocardiography\/Society for Pediatric Radiology\/North American Society for Cardiovascular Imaging Guidelines for the use of cardiac magnetic resonance in Pediatric congenital and acquired heart disease: endorsed by the American heart Association. Circ, Cardiovasc Imaging 2022;15:e014415. https:\/\/doi.org\/10.1161\/CIRCIMAGING.122.014415.","DOI":"10.1161\/CIRCIMAGING.122.014415"},{"key":"2026050115424109980_j_mr-2026-0002_ref_214","doi-asserted-by":"crossref","unstructured":"Valdeolmillos, E, Sakhi, H, Tortigue, M, Audi\u00e9, M, Isorni, MA, Lecerf, F, et al.. 4D flow cardiac MRI to assess pulmonary blood flow in patients with pulmonary arterial hypertension associated with congenital heart disease. Diagn Interv Imaging 2024;105:266\u201372. https:\/\/doi.org\/10.1016\/j.diii.2024.01.009.","DOI":"10.1016\/j.diii.2024.01.009"},{"key":"2026050115424109980_j_mr-2026-0002_ref_215","doi-asserted-by":"crossref","unstructured":"Broncano, J, Bhalla, S, Gutierrez, FR, Vargas, D, Williamson, EE, Makan, M, et al.. Cardiac MRI in pulmonary hypertension: from magnet to bedside. Radiogr Rev Publ Radiol Soc N Am Inc 2020;40:982\u20131002. https:\/\/doi.org\/10.1148\/rg.2020190179.","DOI":"10.1148\/rg.2020190179"},{"key":"2026050115424109980_j_mr-2026-0002_ref_216","doi-asserted-by":"crossref","unstructured":"Ka\u010dar, P, Prok\u0161elj, K, Ghonim, S, Semple, T, Babu-Narayan, SV, Nashat, H, et al.. Advances in the imaging of pulmonary hypertension. Int J Cardiol Congenit Heart Dis 2025;22:100619. https:\/\/doi.org\/10.1016\/j.ijcchd.2025.100619.","DOI":"10.1016\/j.ijcchd.2025.100619"},{"key":"2026050115424109980_j_mr-2026-0002_ref_217","doi-asserted-by":"crossref","unstructured":"Rajagopal, S, Bogaard, HJ, Elbaz, MSM, Freed, BH, Remy-Jardin, M, van Beek, EJR, et al.. Emerging multimodality imaging techniques for the pulmonary circulation. Eur Respir J 2024;64:2401128. https:\/\/doi.org\/10.1183\/13993003.01128-2024.","DOI":"10.1183\/13993003.01128-2024"},{"key":"2026050115424109980_j_mr-2026-0002_ref_218","doi-asserted-by":"crossref","unstructured":"Reddy, C, Yan, Y, Qiu, M, Tang, Y, Jin, B, Han, Z, et al.. 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