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

Summary 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 (...

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Published in:British journal of haematology 2012-08, Vol.158 (3), p.409-414
Main Authors: Hill, Anita, Sapsford, Robert J., Scally, Andrew, Kelly, Richard, Richards, Stephen J., Khurisgara, Gus, Sivananthan, Mohan U., Hillmen, Peter
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Anemias. Hemoglobinopathies
Biological and medical sciences
Diseases of red blood cells
Doppler-estimated systolic pulmonary hypertension
Echocardiography, Doppler
endogenous nitrate vasodilator
haemolysis
Heart - physiopathology
Hematologic and hematopoietic diseases
Hemoglobinuria, Paroxysmal - blood
Hemoglobinuria, Paroxysmal - complications
Hemoglobinuria, Paroxysmal - physiopathology
Humans
Hypertension, Pulmonary - blood
Hypertension, Pulmonary - complications
Hypertension, Pulmonary - physiopathology
Medical sciences
Middle Aged
Natriuretic Peptide, Brain - blood
paroxysmal nocturnal haemoglobinuria
Peptide Fragments - blood
Pneumology
pulmonary hypertension
Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
Ventricular Function, Right - physiology
Young Adult
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Summary:Summary 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 
ISSN:0007-1048
1365-2141
DOI:10.1111/j.1365-2141.2012.09166.x