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.