{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,16]],"date-time":"2023-10-16T08:43:52Z","timestamp":1697445832307},"reference-count":21,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2013,6,9]],"date-time":"2013-06-09T00:00:00Z","timestamp":1370736000000},"content-version":"vor","delay-in-days":8,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2013,6]]},"abstract":"Abstract<\/jats:title>Background and Aims<\/jats:title>Pulmonary arterial hypertension (PAH<\/jats:styled-content>) is associated with significant morbidity and mortality. In N<\/jats:styled-content>ew Z<\/jats:styled-content>ealand, access to PAH<\/jats:styled-content>\u2010specific pharmacotherapy has been restricted for economic reasons. Since 2009, access to medication has been provided by a S<\/jats:styled-content>pecial A<\/jats:styled-content>uthority scheme that ensures equity of access for all patients. Management of patients with PAH<\/jats:styled-content> in this environment poses unique challenges. This study describes outcomes of patients managed in the A<\/jats:styled-content>uckland PAH<\/jats:styled-content> clinic over a 12\u2010year period.<\/jats:p><\/jats:sec>Methods<\/jats:title>The clinic database was interrogated. Patients were eligible for this study if they had PAH<\/jats:styled-content> (W<\/jats:styled-content>orld H<\/jats:styled-content>ealth O<\/jats:styled-content>rganization (WHO<\/jats:styled-content>) C<\/jats:styled-content>lass 1, 1\u2032 and 4) and had been managed in the clinic from the year 2000.<\/jats:p><\/jats:sec>Results<\/jats:title>One hundred and twenty\u2010six patients (75% female, mean age at diagnosis 50) were included. Most had idiopathic PAH<\/jats:styled-content> (37%) or PAH<\/jats:styled-content> because of connective tissue disease (29%). At diagnosis, patients had advanced disease (median WHO C<\/jats:styled-content>lass III<\/jats:styled-content>, 6\u2010min walk distance 367\u2009m and pulmonary vascular resistance of 10.6 W<\/jats:styled-content>ood U<\/jats:styled-content>nits). Initial therapy was sildenafil in most cases. PAH<\/jats:styled-content>\u2010specific therapy was associated with improved WHO<\/jats:styled-content> class and longer 6\u2010min walk distance (P<\/jats:italic> < 0.01 for both). Thirty per cent of patients were escalated to combination therapy. Survival was 91% at 1 year and 67% at 5 years.<\/jats:p><\/jats:sec>Conclusions<\/jats:title>Despite historic difficulties with access to PAH<\/jats:styled-content>\u2010specific therapy, these data confirm benefits of therapy (primarily sildenafil as first\u2010line treatment) for patients with PAH<\/jats:styled-content> managed within the A<\/jats:styled-content>uckland PAH<\/jats:styled-content> clinic. Survival data are comparable with other reported cohorts.<\/jats:p><\/jats:sec>","DOI":"10.1111\/imj.12069","type":"journal-article","created":{"date-parts":[[2012,12,24]],"date-time":"2012-12-24T11:23:00Z","timestamp":1356348180000},"page":"635-642","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Building a pulmonary vascular service: the 12\u2010year experience and outcomes of the A<\/scp>uckland pulmonary arterial hypertension clinic"],"prefix":"10.1111","volume":"43","author":[{"given":"C. A.","family":"Wasywich","sequence":"first","affiliation":[{"name":"Green Lane Cardiovascular Service and New Zealand Heart and Lung Transplant Program Auckland City Hospital Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Cicovic","sequence":"additional","affiliation":[{"name":"General Medicine Auckland City Hospital Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"McWilliams","sequence":"additional","affiliation":[{"name":"New Zealand Heart and Lung Transplant Program Auckland City Hospital Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H. A.","family":"Coverdale","sequence":"additional","affiliation":[{"name":"New Zealand Heart and Lung Transplant Program Auckland City Hospital Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Stewart","sequence":"additional","affiliation":[{"name":"New Zealand Heart and Lung Transplant Program Auckland City Hospital Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Whyte","sequence":"additional","affiliation":[{"name":"New Zealand Heart and Lung Transplant Program Auckland City Hospital Auckland New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,6,9]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199207093270203"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.104.488486"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa020204"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.165.6.2106079"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(01)06250-X"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa012212"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.04.00028404"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.106.630715"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)60919-8"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa050010"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.839274"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp022"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2009.11.028"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp297"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-1166"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00078411"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.11-1460"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.911818"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1445-5994.2010.02403.x"}],"container-title":["Internal Medicine Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fimj.12069","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/imj.12069","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,15]],"date-time":"2023-10-15T06:55:48Z","timestamp":1697352948000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/imj.12069"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,6]]},"references-count":21,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2013,6]]}},"alternative-id":["10.1111\/imj.12069"],"URL":"https:\/\/doi.org\/10.1111\/imj.12069","archive":["Portico"],"relation":{},"ISSN":["1444-0903","1445-5994"],"issn-type":[{"value":"1444-0903","type":"print"},{"value":"1445-5994","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,6]]},"assertion":[{"value":"2012-09-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2012-12-03","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-06-09","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}