{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T04:38:24Z","timestamp":1792643904841,"version":"4.5.0"},"reference-count":23,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2012,5,29]],"date-time":"2012-05-29T00:00:00Z","timestamp":1338249600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2012,8]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Pulmonary hypertension is becoming a recognized complication of the hereditary and acquired haemolytic anaemias, associated with a poor prognosis. Recently we reported that patients with paroxysmal nocturnal haemoglobinuria (\n                    <jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>\n                    ) have high levels of N\u2010terminal pro\u2010brain natriuretic peptide (\n                    <jats:styled-content style=\"fixed-case\">NT<\/jats:styled-content>\n                    \u2010pro\n                    <jats:styled-content style=\"fixed-case\">BNP<\/jats:styled-content>\n                    ), a biomarker associated with both right and left ventricular dysfunction and cardiac dysfunction. In the current study we evaluated a cohort of patients (\n                    <jats:italic>N<\/jats:italic>\n                    \u00a0=\u00a029) with haemolytic\n                    <jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>\n                    for elevated pulmonary artery systolic pressure and cardiac function by\n                    <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>\n                    oppler\u2010echocardiography. Of the 29 patients, eight were further studied using cardiac magnetic resonance imaging (\n                    <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>\n                    ), as well as two additional patients (number of patients studied using cardiac\n                    <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>\n                    \u00a0=\u00a010). Plasma from the first cohort (\n                    <jats:italic>N<\/jats:italic>\n                    \u00a0=\u00a029) demonstrated intravascular haemolysis associated with a 12\u2010fold increase in median nitric oxide (\n                    <jats:styled-content style=\"fixed-case\">NO<\/jats:styled-content>\n                    ) consumption when compared with healthy volunteers (\n                    <jats:italic>P<\/jats:italic>\n                    \u00a0&lt;\u00a00\u00b7001). Doppler echocardiography demonstrated normal left ventricular function and elevated pulmonary artery systolic pressure in 41% of patients. Cardiac\n                    <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>\n                    from the second cohort (\n                    <jats:italic>N<\/jats:italic>\n                    \u00a0=\u00a010) demonstrated depressed right ventricular function in 80% of\n                    <jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>\n                    patients tested, and 60% had findings suggestive of subclinical small pulmonary emboli. Together, these data suggest a high prevalence of haemolysis\u2010associated\n                    <jats:styled-content style=\"fixed-case\">NO<\/jats:styled-content>\n                    scavenging,\n                    <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>\n                    oppler\u2010estimated systolic pulmonary hypertension, and depressed right ventricular function in patients with\n                    <jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>\n                    .\n                  <\/jats:p>","DOI":"10.1111\/j.1365-2141.2012.09166.x","type":"journal-article","created":{"date-parts":[[2012,5,29]],"date-time":"2012-05-29T02:35:56Z","timestamp":1338258956000},"page":"409-414","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":53,"title":["Under\u2010recognized complications in patients with paroxysmal nocturnal haemoglobinuria: raised pulmonary pressure and reduced right ventricular function"],"prefix":"10.1111","volume":"158","author":[{"given":"Anita","family":"Hill","sequence":"first","affiliation":[{"name":"Department of Haematology St. James's Institute of Oncology Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J.","family":"Sapsford","sequence":"additional","affiliation":[{"name":"Department of Cardiology Leeds General Infirmary Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Scally","sequence":"additional","affiliation":[{"name":"University of Bradford Bradford UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Kelly","sequence":"additional","affiliation":[{"name":"Department of Haematology St. James's Institute of Oncology Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen J.","family":"Richards","sequence":"additional","affiliation":[{"name":"Department of Haematology St. James's Institute of Oncology Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gus","family":"Khurisgara","sequence":"additional","affiliation":[{"name":"Alexion Pharmaceuticals, Inc Cheshire CT USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohan U.","family":"Sivananthan","sequence":"additional","affiliation":[{"name":"LIGHT, University of Leeds Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Hillmen","sequence":"additional","affiliation":[{"name":"Department of Haematology St. James's Institute of Oncology Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,5,29]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V97.11.3411"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmri.10262"},{"key":"e_1_2_6_4_1","first-page":"419","volume-title":"Hematology: Basic Principles and 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