Under-recognized complications in patients with paroxysmal nocturnal haemoglobinuria: Raised pulmonary pressure and reduced right ventricular function

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Abstract

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 (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. © 2012 Blackwell Publishing Ltd.

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Hill, A., Sapsford, R. J., Scally, A., Kelly, R., Richards, S. J., Khurisgara, G., … 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(3), 409–414. https://doi.org/10.1111/j.1365-2141.2012.09166.x

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