Pulmonary function, CT and echocardiographic abnormalities in sickle cell disease

Area of Science:

• Pulmonary Medicine
• Cardiovascular Medicine
• Radiology

Background:

• Sickle cell disease (SCD) is associated with significant pulmonary complications.
• Lung function decline is a known issue in adults with SCD.

Purpose of the Study:

• To investigate the association between pulmonary vascular abnormalities on high-resolution CT (HRCT) and echocardiographic findings.
• To determine if these vascular changes correlate with lung function abnormalities and decline in SCD patients.

Main Methods:

• 35 adult SCD patients underwent HRCT, echocardiography, and lung function tests.
• Pulmonary artery dimensions were quantified using the artery/bronchus (A/B) ratio and cross-sectional area (CSA<5 mm%) on HRCT.
• 20 patients were reassessed after a median of 6.6 years.

Main Results:

• Pulmonary vascular abnormalities (A/B ratio and CSA<5 mm%) were negatively correlated with lung function parameters (FEV1, VC, FEF25/75, SpO2) and positively with measures of air trapping and resistance.
• Echocardiographic cardiac output correlated significantly with A/B ratio and CSA<5 mm%.
• Significant deterioration in lung function and pulmonary vascular measures was observed over the follow-up period.

Conclusions:

• Abnormalities in pulmonary vascular volumes identified by HRCT are linked to lung function impairments in SCD.
• These vascular changes may contribute to the progressive decline in lung function observed in adults with SCD.