Pulmonary artery pressure and the acute chest syndrome in homozygous sickle cell disease

ABSTRACT

OBJECTIVE; to investigate whether attacks of acute chest syndrome affected pulmonary artery pressure in patients homozygous for sickle cell disease. MAIN OUTCOME MEASURES: Pulmonary artery pressure, assessed by non-invasive echocardiographic techniques. PATIENTS; 20 patients with homozygous sickle cell disease with a history of at least six episodes of acute chest syndrome and in 20 age, sex, and height matched controls with homozygous sickle cell disease without a history of acute chest syndrome. RESULTS: There was no difference in any of the echocardiographic or Doppler indices between these two groups. CONCLUSIONS: Repeated attacks of acute chest syndrome by the mean age of 12 ( range eight to 16 ) years have not had a discernible effect upon pulmonary artery pressure (AU)