{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T23:03:50Z","timestamp":1790550230269,"version":"4.1.0"},"reference-count":9,"publisher":"BMJ","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Heart"],"accepted":{"date-parts":[[2000,4,19]]},"published-print":{"date-parts":[[2000,8,1]]},"abstract":"<jats:p>Primary pulmonary hypertension is a rare disease of childhood, which carries a poor prognosis. Patients often present with severe exercise limitation, and untreated life expectancy is less than 1 year. Pharmacological intervention is directed towards reduction of the raised pulmonary artery pressure with vasodilator treatment, initially with calcium antagonists, although more recently long term prostacyclin treatment has shown benefit in some patients. Heart\u2013lung transplantation remains an option for children with severe disease refractory to therapeutic treatment.  \u2003A 4 year old Bangladeshi girl with dyspnoea, cyanosis, and signs of a low cardiac output, is described. Initial treatment with prostacyclin was gradually reduced, and maintenance treatment with oral sildenafil (Viagra; Pfizer) instituted. At follow up 3 months later, her exercise capacity was greatly improved and she continues to enjoy a good quality of life without obvious side effects. In view of the encouraging initial results, this may become an acceptable adjunct in treating this patient group.<\/jats:p>","DOI":"10.1136\/heart.84.2.e4","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T06:37:59Z","timestamp":1027751879000},"page":"e4-e4","source":"Crossref","is-referenced-by-count":168,"title":["Sildenafil as a selective pulmonary vasodilator in childhood primary pulmonary hypertension"],"prefix":"10.1136","volume":"84","author":[{"given":"D","family":"Abrams","sequence":"first","affiliation":[{"name":"Department of Paediatric Cardiology, Royal Brompton & Harefield NHS Trust, Sydney Street, London SW3 6NP, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I","family":"Schulze-Neick","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Royal Brompton & Harefield NHS Trust, Sydney Street, London SW3 6NP, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A G","family":"Magee","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Royal Brompton & Harefield NHS Trust, Sydney Street, London SW3 6NP, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2000,8,1]]},"reference":[{"key":"2025101722462957000_84.2.e4.1","unstructured":"Barst RJ (1996) Primary pulmonary hypertension in children. in Primary pulmonary hypertension. eds Rubin LJ Rich S (Marcel Dekker, New York), pp 179\u2013225."},{"key":"2025101722462957000_84.2.e4.2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.99.9.1197"},{"key":"2025101722462957000_84.2.e4.3","first-page":"692","article-title":"Pediatric heart-lung transplantation: intermediate-term results.","volume":"15","author":"Conte","year":"1996","journal-title":"J Heart Lung Transplant"},{"key":"2025101722462957000_84.2.e4.4","first-page":"1189","article-title":"The Registry of the International Society of Heart and Lung Transplantation: first official pediatric report\u20141997.","volume":"16","author":"Boucek","year":"1997","journal-title":"J Heart Lung Transplant"},{"key":"2025101722462957000_84.2.e4.5","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"2025101722462957000_84.2.e4.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199801293380501"},{"key":"2025101722462957000_84.2.e4.7","doi-asserted-by":"publisher","DOI":"10.1124\/mol.56.1.124"},{"key":"2025101722462957000_84.2.e4.8","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199802000-00002"},{"key":"2025101722462957000_84.2.e4.9","doi-asserted-by":"publisher","DOI":"10.1097\/00000542-199907000-00041"}],"container-title":["Heart"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/heart.84.2.e4","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,18]],"date-time":"2025-10-18T05:46:39Z","timestamp":1760766399000},"score":1,"resource":{"primary":{"URL":"https:\/\/heart.bmj.com\/lookup\/doi\/10.1136\/heart.84.2.e4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2000,8,1]]},"references-count":9,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2000,8,1]]},"published-print":{"date-parts":[[2000,8,1]]}},"alternative-id":["10.1136\/heart.84.2.e4"],"URL":"https:\/\/doi.org\/10.1136\/heart.84.2.e4","relation":{},"ISSN":["1355-6037","1468-201X"],"issn-type":[{"value":"1355-6037","type":"print"},{"value":"1468-201X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2000,8,1]]}}}