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P11 PULMONARY ARTERIAL HYPERTENSION IN CHILDREN WITH SICKLE CELL DISEASE: HOW COMMON IS IT?
R.. Chaudry, presenting, T. Karu, C. Hutchinson, S. Ball, G. Sutherland, A. Bush, M. Rosenthal, S. Crowley.Royal Brompton Hospital, London; St George’s Hospital, London, UK

Introduction: The prevalence of pulmonary arterial hypertension (PAH) in adults with sickle cell disease (SCD) is estimated to be 32%, 2-year mortality of 50% from diagnosis.

Aims: To prospectively determine the prevalence of PAH in children with SCD and define associated risk factors such as hyper-haemolytic state defined by haemoglobin 8.5 g/dl and number of acute chest syndrome events

Methods: Fifty (F = 26) patients with SCD, range 10–18 years, and 50 (F = 25) sex, age, race-matched healthy controls were recruited. Tricuspid regurgitant jet velocity was measured using detailed 2D-Doppler echo; all subjects were at baseline state of health. Patients were free of any vaso-occlusive crisis for 14 days. Hb was measured and a detailed medical history was taken with verification from hospital . . . [Full text of this article]