Pulmonary hypertension in Nigerian adults with sickle cell anemia

BACKGROUND Sickle cell anemia (SCA) is the commonest hemoglobinopathy and is associated with high morbidity and mortality. Pulmonary hypertension (PH) is reported to play a significant role in this regard. There is very limited literature on PH in SCA in Nigeria. OBJECTIVES The objectives of this study were to determine the prevalence of Doppler-derived PH in SCA, assess its influence on exercise capacity, and determine the correlates and predictors of measures of estimated pulmonary pressure. METHODS A total of 92 SCA subjects had echocardiography and 6-minute self-paced walking exercise. PH was diagnosed by Doppler echocardiography on finding a tricuspid regurgitant velocity (TRV) of ≥2.5 m/s. The pulmonary flow profile was also assessed to estimate mean pulmonary arterial pressure (MPAP). RESULTS Doppler-derived PH was detected in 23.9% of adults with SCA. The 6-minute walking distance (6MWD) was significantly lower in SCA adults with PH than in those without PH (380.33 ± 63.17 m vs 474.28 ± 76.74 m; p = 0.014). TRV and estimated MPAP had a significant inverse correlation with the 6MWD (r = -0.442; p < 0.001 and r = -0.571; p < 0.001, respectively). CONCLUSION PH as derived by Doppler is common in Nigerian adults with SCA and has a significantly negative influence on exercise capacity. Screening for PH should be encouraged to optimize management and thus improve their quality of life and life expectancy.