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Authors: Voskaridou E, Tsetsos G, Tsoutsias A, Spyropoulou E, Christoulas D and Terpos E – Haematologica, 2007; 92(6); 738-743
Summarised by Dr. Michael Angastiniotis, TIF Medical Advisor

In a second study presented at the same Conference, Voskaridou et al. also studied the phenomenon of pulmonary hypertension, mainly from the point of view of diagnosis and monitoring in patients with sickle cell/b-thalassaemia.

They confirm the role of haemolysis in the aetiology of pulmonary hypertension, and that markers of haemolysis such as low haemoglobin concentration, raised reticulocyte counts and bilirubin are associated with this condition.

In their study, they also confirm that echocardiography is the cornerstone screening test for diagnosis. Like the previous authors, they point out that systolic pulmonary artery pressure equal or above 35mmHg is diagnostic, as well as a tricuspid valve regurgitant velocity (TRV) equal to or above 2.5m/sec. Such patients have a nine- to ten-fold risk of early mortality compared to those with a normal TRV.

In this study, 33.3% of the 91 patients with sickle cell/thalassaemia examined had pulmonary hypertension. They also investigated the diagnostic role of NT-pro BNP in this condition. This is a stable form of Pro BNP in the serum, which is a hormone released in response to stretching of the heart muscle fibres. In their study, an elevated NT-proBNP level reflected the severity of pulmonary hypertension.

They conclude that NT-proBNP levels along with echocardiographic measurements, especially TRV, provide a useful tool for recognising patients with significant pulmonary hypertension.

Treatment of SCD with hydroxyurea and increased foetal haemoglobin levels are not associated with lower TRV in the study, confirming other studies which showed that hydroxyurea does not appear to protect against the development of pulmonary hypertension.