Show simple item record

dc.contributor.authorHill, A.*
dc.contributor.authorSpasford, R.J.*
dc.contributor.authorScally, Andy J.*
dc.contributor.authorKelly, R.J.*
dc.contributor.authorRichards, S.J.*
dc.contributor.authorKhurisgara, G.*
dc.contributor.authorSivananthan, M.U.*
dc.contributor.authorHillmen, P.*
dc.date.accessioned2014-05-30T16:23:11Z
dc.date.available2014-05-30T16:23:11Z
dc.date.issued2012
dc.identifier.citationHill, A., Sapsford, R. J., Scally, A., Kelly, R., Richards, S. J., Khurisgara, G., Sivananthan, M. U. and Hillmen, P. (2012). Under-recognized complications in patients with paroxysmal nocturnal haemoglobinuria: raised pulmonary pressure and reduced right ventricular function. British Journal of Haematology, 158: 409¿414.en_US
dc.identifier.urihttp://hdl.handle.net/10454/6364
dc.description.abstractPulmonary 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 (PNH) have high levels of N-terminal pro-brain natriuretic peptide (NT-proBNP), a biomarker associated with both right and left ventricular dysfunction and cardiac dysfunction. In the current study we evaluated a cohort of patients (N = 29) with haemolytic PNH for elevated pulmonary artery systolic pressure and cardiac function by Doppler-echocardiography. Of the 29 patients, eight were further studied using cardiac magnetic resonance imaging (MRI), as well as two additional patients (number of patients studied using cardiac MRI = 10). Plasma from the first cohort (N = 29) demonstrated intravascular haemolysis associated with a 12-fold increase in median nitric oxide (NO) consumption when compared with healthy volunteers (P < 0·001). Doppler echocardiography demonstrated normal left ventricular function and elevated pulmonary artery systolic pressure in 41% of patients. Cardiac MRI from the second cohort (N = 10) demonstrated depressed right ventricular function in 80% of PNH patients tested, and 60% had findings suggestive of subclinical small pulmonary emboli. Together, these data suggest a high prevalence of haemolysis-associated NO scavenging, Doppler-estimated systolic pulmonary hypertension, and depressed right ventricular function in patients with PNH.en_US
dc.language.isoenen_US
dc.subjectParoxysmal nocturnal haemoglobinuria; Endogenous nitrate vasodilator; Pulmonary hypertension; Haemolysis; Doppler-estimated systolic pulmonary hypertensionen_US
dc.titleUnder-recognized complications in patients with paroxysmal nocturnal haemoglobinuria: raised pulmonary pressure and reduced right ventricular function.en_US
dc.status.refereedyesen_US
dc.typeArticleen_US
dc.type.versionpublished version paperen_US
dc.identifier.doihttps://doi.org/10.1111/j.1365-2141.2012.09166.x


This item appears in the following Collection(s)

Show simple item record