Publication:

Clinical profiling of sickle cell disease patients with respect to pulmonary hypertension

Background Sickle cell disease (SCD) is the commonest heritable hematologic abnormality affecting humans. Pulmonary hypertension is documented in patients of SCD by both non-invasive methods and catheterization, with incidence reported to be as high as 20%-63%. However, pulmonary hypertension has not been studied as complication of patients of sickle cell disease in India.

Methods A total of 94 (54 SS and 40 AS) more than 12 years old cases diagnosed to have sickle cell disease by hemoglobin electrophoresis were recruited. All the participants were assessed with complete history, general and systemic examination, hematological and biochemical tests, X-ray chest, ECG, pulmonary function tests and 2D echocardiography and Doppler studies. Cases were observed in 2 groups: SS cases and AS cases. Sixty four age/sex matched healthy close relatives of the cases with ‘AA’ Hb electrophoresis pattern were the controls.

Results Five SS cases were detected to have pulmonary hypertension. Pulmonary hypertension was not detected in any AS case or control. Pulmonary hypertension was more common in females (3/23, 13%) than males (2/26, 7.7%). The mean age of the SS cases with PH (32±5.15) was found to be significantly higher than that of the SS cases without PH (24.2±6.21) (p<0.01).

Conclusions The incidence of PH was low in this study, as compared to western studies. This may be since most of the cases of SCD being in remote areas having no access to health care facilities.