Publication:

Study of pulmonary hypertension as a complication of sickle cell disease

Background Sickle cell anaemia is the most common inherited hematological abnormality across human race, particularly prevalent in pockets of central India and pulmonary hypertension (PH) supposedly worsens prognosis. Data from central India is lacking though. Present study aims to study incidence of pulmonary hypertension as a complication in patients of sickle cell disease.

Methods Patients aged more than 12 years diagnosed to have sickle cell anaemia on hemoglobin electrophoresis during the study period (total- 94; 54 SS and 40 AS) were enrolled as cases. Sixty four age/sex matched healthy close relatives of the cases with ‘AA’ Hb electrophoresis pattern doubled up as controls. Participants were thoroughly assessed with complete history, examination, haematological and biochemical tests, X-ray chest, ECG, pulmonary function tests and 2D-echocardiography and Doppler studies and data analysed.

Results Pulmonary hypertension was observed in a total of five SS cases (5/54, 9.26%). No participant from the AS group or the control group developed pulmonary hypertension. It 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 years) was found to be significantly higher than that of the SS cases without PH (24.2±6.21 years) (p<0.01).

Conclusions The incidence of PH in sickle cell disease was relatively lower as compared to western countries and was found to affect females and older individuals more. Larger community-based studies are recommended for corroboration.