{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T01:32:12Z","timestamp":1790645532070,"version":"4.1.0"},"reference-count":30,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2012,8,1]],"date-time":"2012-08-01T00:00:00Z","timestamp":1343779200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The American Journal of Cardiology"],"published-print":{"date-parts":[[2012,8]]},"DOI":"10.1016\/j.amjcard.2012.04.035","type":"journal-article","created":{"date-parts":[[2012,5,25]],"date-time":"2012-05-25T16:00:56Z","timestamp":1337961656000},"page":"586-593","source":"Crossref","is-referenced-by-count":72,"title":["Outcomes of Childhood Pulmonary Arterial Hypertension in BMPR2 and ALK1 Mutation Carriers"],"prefix":"10.1016","volume":"110","author":[{"given":"Ayako","family":"Chida","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Shintani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hisato","family":"Yagi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maya","family":"Fujiwara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuko","family":"Kojima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroki","family":"Sato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichiro","family":"Imamura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masato","family":"Yokozawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norio","family":"Onodera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hitoshi","family":"Horigome","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomio","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiho","family":"Hatai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomotaka","family":"Nakayama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Fukushima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsunori","family":"Nishiyama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shouzaburo","family":"Doi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuo","family":"Ono","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Yasukouchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fukiko","family":"Ichida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuto","family":"Fujimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichi","family":"Ohtsuki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidetaka","family":"Teshima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuya","family":"Kawano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuichi","family":"Nomura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong","family":"Gu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takahiro","family":"Ishiwata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiyuki","family":"Furutani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kei","family":"Inai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsutomu","family":"Saji","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rumiko","family":"Matsuoka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shigeaki","family":"Nonoyama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshio","family":"Nakanishi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.amjcard.2012.04.035_bib1","doi-asserted-by":"crossref","first-page":"343","DOI":"10.7326\/0003-4819-115-5-343","article-title":"Survival in patients with primary pulmonary hypertension: results from a natural prospective registry","volume":"115","author":"D'Alonzo","year":"1991","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.amjcard.2012.04.035_bib2","doi-asserted-by":"crossref","first-page":"1533","DOI":"10.1016\/S0140-6736(03)13167-4","article-title":"Primary pulmonary hypertension","volume":"361","author":"Runo","year":"2003","journal-title":"Lancet"},{"key":"10.1016\/j.amjcard.2012.04.035_bib3","series-title":"Lung Biology in Health and Disease","first-page":"179","article-title":"Primary pulmonary hypertension in children in primary pulmonary hypertension","author":"Barst","year":"1997"},{"key":"10.1016\/j.amjcard.2012.04.035_bib4","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1161\/01.CIR.99.9.1197","article-title":"Vasodilator therapy for primary pulmonary hypertension in children","volume":"99","author":"Barst","year":"1999","journal-title":"Circulation"},{"key":"10.1016\/j.amjcard.2012.04.035_bib5","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1038\/79226","article-title":"Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension","volume":"26","author":"Lane","year":"2000","journal-title":"Nat Genet"},{"key":"10.1016\/j.amjcard.2012.04.035_bib6","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1086\/303059","article-title":"Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene","volume":"67","author":"Deng","year":"2000","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.amjcard.2012.04.035_bib7","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1056\/NEJM200108023450503","article-title":"Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia","volume":"345","author":"Trembath","year":"2001","journal-title":"N Engl J Med"},{"key":"10.1016\/j.amjcard.2012.04.035_bib8","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1253\/circj.72.127","article-title":"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","volume":"72","author":"Fujiwara","year":"2008","journal-title":"Circ J"},{"key":"10.1016\/j.amjcard.2012.04.035_bib9","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1136\/jmg.40.12.865","article-title":"Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia","volume":"40","author":"Harrison","year":"2003","journal-title":"J Med Genet"},{"key":"10.1016\/j.amjcard.2012.04.035_bib10","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1136\/jmg.2008.062703","article-title":"A new nonsense mutation of SMAD8 associated with pulmonary arterial hypertension","volume":"46","author":"Shintani","year":"2009","journal-title":"J Med Genet"},{"key":"10.1016\/j.amjcard.2012.04.035_bib11","doi-asserted-by":"crossref","DOI":"10.1253\/circj.CJ-11-1281","article-title":"Missense mutations of BMPR1B (ALK6) gene in childhood idiopathic pulmonary arterial hypertension (IPAH)","author":"Chida","year":"2012","journal-title":"Circ J"},{"key":"10.1016\/j.amjcard.2012.04.035_bib12","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1164\/rccm.200908-1284OC","article-title":"Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1(ALK1) mutation","volume":"181","author":"Girerd","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.amjcard.2012.04.035_bib13","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1186\/1465-9921-11-73","article-title":"Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension","volume":"11","author":"Girerd","year":"2010","journal-title":"Respir Res"},{"key":"10.1016\/j.amjcard.2012.04.035_bib14","doi-asserted-by":"crossref","first-page":"1377","DOI":"10.1164\/rccm.200712-1807OC","article-title":"Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation","volume":"177","author":"Sztrymf","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.amjcard.2012.04.035_bib15","doi-asserted-by":"crossref","first-page":"216","DOI":"10.7326\/0003-4819-107-2-216","article-title":"Primary pulmonary hypertension","volume":"107","author":"Rich","year":"1987","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.amjcard.2012.04.035_bib16","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1378\/chest.89.4.497","article-title":"Pharmacologically induced pulmonary vasodilation in children and young adults with primary pulmonary hypertension","volume":"89","author":"Barst","year":"1986","journal-title":"Chest"},{"key":"10.1016\/j.amjcard.2012.04.035_bib17","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1086\/316947","article-title":"BMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension","volume":"68","author":"Machado","year":"2001","journal-title":"Am J Hum Genet"},{"issue":"1 Suppl","key":"10.1016\/j.amjcard.2012.04.035_bib18","doi-asserted-by":"crossref","first-page":"S32","DOI":"10.1016\/j.jacc.2009.04.015","article-title":"Genetics and genomics of pulmonary arterial hypertension","volume":"54","author":"Machado","year":"2009","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.amjcard.2012.04.035_bib19","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1136\/jmg.37.10.741","article-title":"Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family","volume":"37","author":"Thomson","year":"2000","journal-title":"J Med Genet"},{"key":"10.1016\/j.amjcard.2012.04.035_bib20","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1186\/1465-9921-12-99","article-title":"Hemodynamic and clinical onset in patients with hereditary pulmonary arterial hypertension and BMPR2 mutations","volume":"12","author":"Pfarr","year":"2011","journal-title":"Respir Res"},{"key":"10.1016\/j.amjcard.2012.04.035_bib21","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1046\/j.1442-200X.2002.01567.x","article-title":"Novel nonsense mutation of the BMPR-II gene in a Japanese patient with familial primary pulmonary hypertension","volume":"44","author":"Uehara","year":"2002","journal-title":"Pediatr Int"},{"key":"10.1016\/j.amjcard.2012.04.035_bib22","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1002\/humu.9398","article-title":"BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension","volume":"27","author":"Aldred","year":"2006","journal-title":"Hum Mutat"},{"key":"10.1016\/j.amjcard.2012.04.035_bib23","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1002\/humu.20200","article-title":"BMPR2 mutations have short lifetime expectancy in primary pulmonary hypertension","volume":"26","author":"Sankelo","year":"2005","journal-title":"Hum Mutat"},{"key":"10.1016\/j.amjcard.2012.04.035_bib24","doi-asserted-by":"crossref","first-page":"e127","DOI":"10.1136\/jmg.2004.023101","article-title":"Low frequency of BMPR2 mutations in a German cohort of patients with sporadic idiopathic pulmonary arterial hypertension","volume":"41","author":"Koehler","year":"2004","journal-title":"J Med Genet"},{"key":"10.1016\/j.amjcard.2012.04.035_bib25","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1161\/01.CIR.0000153798.78540.87","article-title":"Transforming growth factor-\u03b2, receptor mutations and pulmonary arterial hypertension in childhood","volume":"111","author":"Harrison","year":"2005","journal-title":"Circulation"},{"key":"10.1016\/j.amjcard.2012.04.035_bib26","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1161\/CIRCULATIONAHA.111.026591","article-title":"Survival in childhood pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term PAH Disease Management","volume":"125","author":"Barst","year":"2012","journal-title":"Circulation"},{"key":"10.1016\/j.amjcard.2012.04.035_bib27","doi-asserted-by":"crossref","first-page":"1401","DOI":"10.1136\/hrt.2009.182378","article-title":"Childhood idiopathic pulmonary arterial hypertension: a national cohort study","volume":"96","author":"Moledina","year":"2010","journal-title":"Heart"},{"key":"10.1016\/j.amjcard.2012.04.035_bib28","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1161\/01.CIR.42.6.1163","article-title":"Primary pulmonary hypertension: a pathologic study of the lung vessels in 156 clinically diagnosed cases","volume":"42","author":"Wagenvoort","year":"1970","journal-title":"Circulation"},{"key":"10.1016\/j.amjcard.2012.04.035_bib29","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1183\/09031936.00056110","article-title":"Pulmonary arterial hypertension: a comparison between children and adults","volume":"37","author":"Barst","year":"2011","journal-title":"Eur Respir J"},{"key":"10.1016\/j.amjcard.2012.04.035_bib30","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1186\/1465-9921-10-87","article-title":"Truncating and missense BMPR2 mutations differentially affect the severity of heritable pulmonary arterial hypertension","volume":"10","author":"Austin","year":"2009","journal-title":"Respir Res"}],"container-title":["The American Journal of Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002914912011976?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002914912011976?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,11,23]],"date-time":"2018-11-23T00:55:49Z","timestamp":1542934549000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0002914912011976"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,8]]},"references-count":30,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2012,8]]}},"alternative-id":["S0002914912011976"],"URL":"https:\/\/doi.org\/10.1016\/j.amjcard.2012.04.035","relation":{},"ISSN":["0002-9149"],"issn-type":[{"value":"0002-9149","type":"print"}],"subject":[],"published":{"date-parts":[[2012,8]]}}}