Pulmonary Hypertension in Adults with Sickle Cell Anaemia: A Prevalence Study in the Niger Delta Region of Nigeria

Abstract

Introduction: Sickle cell anaemia (SCA) is a lifelong multisystem disease endemic in sub Saharan Africa. As the population of patients living with SCA into adulthood continues to increase, pulmonary hypertension has emerged as one of the foremost complications with severe implications on the quality of life and a risk factor for premature mortality. Aims: The present investigation is a prevalence study in the Niger Delta region regarding pulmonary hypertension in adults with sickle cell anaemia. Methods: One hundred and six adults were recruite; fifty-three sickle cell anaemia patients recruited from the outpatient clinic of the haematology department of the University of Port Harcourt teaching hospital, Port Harcourt and 53 genotype AA adults recruited as control. Transthoracic echocardiography was performed on all 106 subjects. The prevalence of pulmonary hypertension was assessed based on tricuspid regurgitation jet of velocity ≥2.5 m/s (estimated SPAP≥30 mmHg).  Results: The mean age of the SCA patients was 25.94±6.47 years, (range 18-45 years) while the age group with the highest representation was those within 18–27 years. The proportion of males (52.8%) was slightly higher than that of the females. Pulmonary hypertension was observed in 5.7% while high normal pulmonary artery pressure was observed in 1.9% of the SCA patients studied. Conclusion: The prevalence of pulmonary hypertension in adults living with Sickle Cell Anaemia in this study is 5.7% and it is significantly influenced by the age of the study cohort and the method used in assessing this parameter.