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

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.