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A case\u2013control study of thalassaemia patients at high <jats:italic>versus<\/jats:italic> low <jats:styled-content style=\"fixed-case\">PH<\/jats:styled-content>\u2010risk was performed. A single cross\u2010sectional measurement for variables reflecting cardiopulmonary status and biological pathophysiology were obtained, including Doppler\u2010echocardiography, 6\u2010min\u2010walk\u2010test, Borg Dyspnoea Score, New York Heart Association functional class, cardiac magnetic resonance imaging (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>), chest\u2010computerized tomography, pulmonary function testing and laboratory analyses targeting mechanisms of coagulation, inflammation, haemolysis, adhesion and the arginine\u2010nitric oxide pathway. Twenty\u2010seven thalassaemia patients were evaluated, 14 with an elevated tricuspid\u2010regurgitant\u2010jet\u2010velocity (<jats:styled-content style=\"fixed-case\">TRV<\/jats:styled-content>)\u00a0\u2265\u00a02\u00b75\u00a0m\/s. Patients with increased <jats:styled-content style=\"fixed-case\">TRV<\/jats:styled-content> had a higher frequency of splenectomy, and significantly larger right atrial size, left atrial volume and left septal\u2010wall thickness on echocardiography and\/or <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>, with elevated biomarkers of abnormal coagulation, lactate dehydrogenase (LDH) levels and arginase concentration, and lower arginine\u2010bioavailability compared to low\u2010risk patients. Arginase concentration correlated significantly to several echocardiography\/<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> parameters of cardiovascular function in addition to global\u2010arginine\u2010bioavailability and biomarkers of haemolytic rate, including LDH, haemoglobin and bilirubin. Thalassaemia patients with a <jats:styled-content style=\"fixed-case\">TRV<\/jats:styled-content>\u00a0\u2265\u00a02\u00b75\u00a0m\/s have additional echocardiography and cardiac\u2010<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> parameters suggestive of right and left\u2010sided cardiac dysfunction. In addition, low arginine bioavailability may contribute to cardiopulmonary dysfunction in \u03b2\u2010thalassaemia.<\/jats:p>","DOI":"10.1111\/bjh.13452","type":"journal-article","created":{"date-parts":[[2015,4,24]],"date-time":"2015-04-24T05:20:59Z","timestamp":1429852859000},"page":"887-898","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Dysregulated arginine metabolism and cardiopulmonary dysfunction in patients with thalassaemia"],"prefix":"10.1111","volume":"169","author":[{"given":"Claudia R.","family":"Morris","sequence":"first","affiliation":[{"name":"Department of Pediatrics Division of Emergency Medicine Emory University School of Medicine Emory Children's Centre for Cystic Fibrosis and Airways Disease Research Atlanta GA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hae\u2010Young","family":"Kim","sequence":"additional","affiliation":[{"name":"New England Research Institutes 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