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

Hill, A.
; 
Spasford, R.J.
; 
Scally, Andy J.
; 
Kelly, R.J.
; 
Richards, S.J.
; 
Khurisgara, G.
; 
Sivananthan, M.U.
; 
Hillmen, P.
Publication Date
2012
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Keywords
Paroxysmal nocturnal haemoglobinuria, Endogenous nitrate vasodilator, Pulmonary hypertension, Haemolysis, Doppler-estimated systolic pulmonary hypertension
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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.
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http://hdl.handle.net/10454/6364
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Citation
Hill A, Sapsford RJ, Scally A et al (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.
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https://doi.org/10.1111/j.1365-2141.2012.09166.x
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