{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T00:55:35Z","timestamp":1759971335034,"version":"build-2065373602"},"reference-count":17,"publisher":"Springer Science and Business Media LLC","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,10,8]]},"DOI":"10.7759\/cureus.94152","type":"journal-article","created":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T23:30:08Z","timestamp":1759966208000},"source":"Crossref","is-referenced-by-count":0,"title":["A Case Report of Pulmonary Arterial Hypertension Associated With Hereditary Hemorrhagic Telangiectasia Successfully Treated With Riociguat"],"prefix":"10.7759","author":[{"given":"Toshiki","family":"Sakuma","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshihiko","family":"Sugiura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keiko","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akira","family":"Naito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayumi","family":"Sekine","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayako","family":"Shigeta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takuji","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199510053331407","article-title":"Hereditary hemorrhagic telangiectasia","volume":"333","author":"Guttmacher AE","year":"1995","unstructured":"Guttmacher AE, Marchuk DA, White RI Jr. Hereditary hemorrhagic telangiectasia. N Engl J Med. 1995, 333:918-24. 10.1056\/NEJM199510053331407","journal-title":"N Engl J Med"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.31.5.559","article-title":"Pulmonary hypertension in patients with pulmonary arteriovenous fistulae","volume":"31","author":"Sapru RP","year":"1969","unstructured":"Sapru RP, Hutchison DC, Hall JI. Pulmonary hypertension in patients with pulmonary arteriovenous fistulae. Br Heart J. 1969, 31:559-69. 10.1136\/hrt.31.5.559","journal-title":"Br Heart J"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201702-0267LE","article-title":"Pulmonary hypertension prevalence and prognosis in a cohort of patients with hereditary hemorrhagic telangiectasia undergoing embolization of pulmonary arteriovenous malformations","volume":"196","author":"Chizinga M","year":"2017","unstructured":"Chizinga M, Rudkovskaia AA, Henderson K, Pollak J, Garcia-Tsao G, Young LH, Fares WH. Pulmonary hypertension prevalence and prognosis in a cohort of patients with hereditary hemorrhagic telangiectasia undergoing embolization of pulmonary arteriovenous malformations. Am J Respir Crit Care Med. 2017, 196:1353-6. 10.1164\/rccm.201702-0267LE","journal-title":"Am J Respir Crit Care Med"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00061308","article-title":"The pulmonary vascular complications of hereditary haemorrhagic telangiectasia","volume":"33","author":"Faughnan ME","year":"2009","unstructured":"Faughnan ME, Granton JT, Young LH. The pulmonary vascular complications of hereditary haemorrhagic telangiectasia. Eur Respir J. 2009, 33:1186-94. 10.1183\/09031936.00061308","journal-title":"Eur Respir J"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2019.02.004","article-title":"Predicting survival in patients with pulmonary arterial hypertension: the REVEAL risk score calculator 2.0 and comparison with ESC\/ERS-based risk assessment strategies","volume":"156","author":"Benza RL","year":"2019","unstructured":"Benza RL, Gomberg-Maitland M, Elliott CG, et al.. Predicting survival in patients with pulmonary arterial hypertension: the REVEAL risk score calculator 2.0 and comparison with ESC\/ERS-based risk assessment strategies. Chest. 2019, 156:323-37. 10.1016\/j.chest.2019.02.004","journal-title":"Chest"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.04.00085504","article-title":"Primary pulmonary hypertension in families with hereditary haemorrhagic telangiectasia","volume":"23","author":"Abdalla SA","year":"2004","unstructured":"Abdalla SA, Gallione CJ, Barst RJ, et al.. Primary pulmonary hypertension in families with hereditary haemorrhagic telangiectasia. Eur Respir J. 2004, 23:373-7. 10.1183\/09031936.04.00085504","journal-title":"Eur Respir J"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1002\/pul2.12301","article-title":"Pulmonary hypertension in hereditary hemorrhagic telangiectasia: a clinical review","volume":"13","author":"Mathavan A","year":"2023","unstructured":"Mathavan A, Mathavan A, Reddy R, Jones K, Eagan C, Alnuaimat H, Ataya A. Pulmonary hypertension in hereditary hemorrhagic telangiectasia: a clinical review. Pulm Circ. 2023, 13:e12301. 10.1002\/pul2.12301","journal-title":"Pulm Circ"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(16)30438-6","article-title":"Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study","volume":"5","author":"Montani D","year":"2017","unstructured":"Montani D, Girerd B, Ja\u00efs X, et al.. Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study. Lancet Respir Med. 2017, 5:125-34. 10.1016\/S2213-2600(16)30438-6","journal-title":"Lancet Respir Med"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1159\/000103205","article-title":"Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (Rendu-Osler disease)","volume":"74","author":"Cottin V","year":"2007","unstructured":"Cottin V, Dupuis-Girod S, Lesca G, Cordier JF. Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (Rendu-Osler disease). Respiration. 2007, 74:361-78. 10.1159\/000103205","journal-title":"Respiration"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1186\/s13256-022-03296-9","article-title":"Pulmonary arterial hypertension in hereditary hemorrhagic telangiectasia associated with ACVRL1 mutation: a case report","volume":"16","author":"Walsh LJ","year":"2022","unstructured":"Walsh LJ, Collins C, Ibrahim H, Kerins DM, Brady AP, O Connor TM. Pulmonary arterial hypertension in hereditary hemorrhagic telangiectasia associated with ACVRL1 mutation: a case report. J Med Case Rep. 2022, 16:99. 10.1186\/s13256-022-03296-9","journal-title":"J Med Case Rep"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1007\/s00380-010-0079-z","article-title":"Successful bosentan therapy for pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia","volume":"26","author":"Chang SA","year":"2011","unstructured":"Chang SA, Jang SY, Ki CS, Kang IS, Kim DK. Successful bosentan therapy for pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia. Heart Vessels. 2011, 26:231-4. 10.1007\/s00380-010-0079-z","journal-title":"Heart Vessels"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2016.03.211","article-title":"Pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia successfully treated with sildenafil","volume":"214","author":"Miyake R","year":"2016","unstructured":"Miyake R, Fujino T, Abe K, et al.. Pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia successfully treated with sildenafil. Int J Cardiol. 2016, 214:275-6. 10.1016\/j.ijcard.2016.03.211","journal-title":"Int J Cardiol"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1016\/j.pupt.2021.102033","article-title":"Treatment of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia - a case series and systematic review","volume":"68","author":"Abston E","year":"2021","unstructured":"Abston E, Hon S, Rodriguez-Lopez J, Moll M, Lanuti M, Farber HW, Wilson KC. Treatment of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia - a case series and systematic review. Pulm Pharmacol Ther. 2021, 68:3. 10.1016\/j.pupt.2021.102033","journal-title":"Pulm Pharmacol Ther"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.110.944603","article-title":"Drug interactions with phosphodiesterase-5 inhibitors used for the treatment of erectile dysfunction or pulmonary hypertension","volume":"122","author":"Schwartz BG","year":"2010","unstructured":"Schwartz BG, Kloner RA. Drug interactions with phosphodiesterase-5 inhibitors used for the treatment of erectile dysfunction or pulmonary hypertension. Circulation. 2010, 122:88-95. 10.1161\/CIRCULATIONAHA.110.944603","journal-title":"Circulation"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"43","author":"Humbert M","year":"2022","unstructured":"Humbert M, Kovacs G, Hoeper MM, et al.. 2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022, 43:3618-731. 10.1093\/eurheartj\/ehac237","journal-title":"Eur Heart J"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.116.003896","article-title":"Clinical adverse effects of endothelin receptor antagonists: insights from the meta-analysis of 4894 patients from 24 randomized double-blind placebo-controlled clinical trials","volume":"5","author":"Wei A","year":"2016","unstructured":"Wei A, Gu Z, Li J, Liu X, Wu X, Han Y, Pu J. Clinical adverse effects of endothelin receptor antagonists: insights from the meta-analysis of 4894 patients from 24 randomized double-blind placebo-controlled clinical trials. J Am Heart Assoc. 2016, 5:6. 10.1161\/JAHA.116.003896","journal-title":"J Am Heart Assoc"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1378\/chest.15-0535","article-title":"Pulmonary hypertension in hereditary hemorrhagic telangiectasia","volume":"149","author":"Lyle MA","year":"2016","unstructured":"Lyle MA, Fenstad ER, McGoon MD, Frantz RP, Krowka MJ, Kane GC, Swanson KL. Pulmonary hypertension in hereditary hemorrhagic telangiectasia. Chest. 2016, 149:362-71. 10.1378\/chest.15-0535","journal-title":"Chest"}],"container-title":["Cureus"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cureus.com\/articles\/410775-a-case-report-of-pulmonary-arterial-hypertension-associated-with-hereditary-hemorrhagic-telangiectasia-successfully-treated-with-riociguat","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T23:30:12Z","timestamp":1759966212000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cureus.com\/articles\/410775-a-case-report-of-pulmonary-arterial-hypertension-associated-with-hereditary-hemorrhagic-telangiectasia-successfully-treated-with-riociguat"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,8]]},"references-count":17,"URL":"https:\/\/doi.org\/10.7759\/cureus.94152","relation":{},"ISSN":["2168-8184"],"issn-type":[{"value":"2168-8184","type":"print"}],"subject":[],"published":{"date-parts":[[2025,10,8]]}}}