Prevalence and Factors Associated with Pulmonary Arterial Hypertension (PAH) in Sickle Cell Children Residing in Yaoundé, Cameroon

Introduction: Sickle cell disease is the most widespread single gene disease in the world. Pulmonary arterial hypertension (PAH), a late complication, is one of its most serious causes of morbidity and mortality. In the absence of data on PAH in paediatric settings in Cameroon, we proposed to conduct a prevalence study and research on its determinants in a group of sickle cell patients. General Objective: Identify the determinants of pulmonary arterial hypertension in a group of children with major sickle cell disease. Methods: We conducted a cross-sectional and analytical study from November 1, 2017 to May 31, 2018 in a group of sickle cell children regularly monitored at the Mother and Child Centre of the Chantal Biya Foundation. For each patient recruited, we collected socio-demographic data, medical history, clinical, biological and echocardiographic data. PAH was defined as mean pulmonary arterial pressure (mPAP)>25mmHg, obtained by measuring the pressure gradient between the pulmonary artery and the right ventricle (Bernoulli equation), to which we added the pressure of the right atrium. The data were analyzed with IBM SPSS 21.0. The association between variables was assessed by the Chi-square or Fisher test for qualitative variables and the Student t-test or Pearson Rho correlation test for quantitative variables. Results: Overall, we enrolled 129 patients, 79 (61.2%) were female. That is to say a sex ratio Male/Female of 0.63. The average age was 11.6 ± 3.2 years. We found PAH (mPAP>25mmHg) in 27 (20.9%) patients. In univariate analysis, the determinants found were high age (p=0.001), presence of dyspnea (p<0.001), high number of blood transfusions (p=0.043), history of pneumonia (p=0.01) dilated LV (DLV) (p=0.031) and dilated aorta (DA) (p=0.05). In multivariate analysis, the independent determinants we found were: age (p=0.041); high number of blood transfusions (p=0.005); history of pneumonia (p<0.001) and dyspnea (p=0.001). Conclusion: PAH is a common complication of sickle cell disease in children and its independent determinants are: age, number of blood transfusions, history of pneumonia, and dyspnea.