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.