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Doppler-defined pulmonary hypertension and the risk of death in children with sickle cell disease followed for a mean of three years
Article first published online: 25 JUN 2009
DOI: 10.1111/j.1365-2141.2009.07779.x
© 2009 Blackwell Publishing Ltd
Additional Information
How to Cite
Lee, M. T., Small, T., Khan, M. A., Rosenzweig, E. B., Barst, R. J. and Brittenham, G. M. (2009), Doppler-defined pulmonary hypertension and the risk of death in children with sickle cell disease followed for a mean of three years. British Journal of Haematology, 146: 437–441. doi: 10.1111/j.1365-2141.2009.07779.x
Publication History
- Issue published online: 27 JUL 2009
- Article first published online: 25 JUN 2009
- Received 3 March 2009; accepted for publication 14 May 2009
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Keywords:
- sickle cell disease;
- pulmonary hypertension;
- tricuspid regurgitant jet velocity;
- children;
- survival
Summary
Pulmonary hypertension (PH) is associated with increased mortality in adults with sickle cell disease (SCD), but its prognostic significance in children is unknown. Eighty-eight children with SCD were followed after echocardiographic screening for PH. After a mean follow-up of 3 years, all 18 subjects with PH were alive. In our children, as in adults with SCD, PH was associated with increased haemolysis. In contrast, our subjects with PH did not have overt systemic disease observed in adults. PH may be a manifestation of progressive organ damage from chronic haemolysis and systemic vasculopathy that ultimately leads to early death in adulthood.