Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia.

BACKGROUND: Pulmonary hypertension (PH), a complication of sickle cell anaemia (SCA), results in considerable morbidity. This study aims to determine the prevalence and associations of echocardiography-suggested PH in children with SCA. METHODS: We performed a cross-sectional comparative study involving 100 systematically sampled SCA subjects 3-14 y of age in their steady state with matched haemoglobin AA phenotype controls. Clinical, laboratory and echocardiography data (including tricuspid regurgitation velocity [TRV], mean pulmonary arterial pressure [mPAP] and tricuspid annular plane systolic excursion [TAPSE]) were obtained from all patients. Statistical analyses were performed using SPSS version 22 (IBM, Armonk, NY, USA). A p-value <0.05 was considered statistically significant. RESULTS: Of the 100 SCA subjects studied, 22 (22%) had echocardiographic findings suggestive of PH compared with none in the controls. The median TAPSE was significantly lower in the PH group (2.55 cm [interquartile range {IQR} 2.2-2.8]) compared with the no PH group (2.77 cm [IQR 2.4-3.2]) (p=0.03). No significant correlation existed between mPAP and age, nor any laboratory parameters studied. The odds ratio (OR) suggested PH significantly increased with an increase in the frequency of hospitalizations for vaso-occlusive crises within a 12-month period (OR 15.15 [95% CI 1.57 to 146.35], p=0.02) and a lifetime history of blood transfusion (OR 5.44 [95% CI 1.09 to 27.24], p=0.04). CONCLUSIONS: Echocardiography-suggested PH is common in children with SCA and is associated with poorer right ventricular function, frequent vaso-occlusive crises and blood transfusions.

Elevated Mean Pulmonary Artery Pressure and Right Ventricular Dysfunction in Children with Chronic Kidney Disease.

BACKGROUND: Elevated mean pulmonary artery pressure (mPAP) and right heart failure increase mortality in patients with chronic kidney disease (CKD). OBJECTIVES: The objective of this study is to determine the prevalence of elevated mPAP in children with CKD compared with matched controls and to ascertain the relationship between elevated mPAP with right ventricular dysfunction and history of hemodialysis. MATERIALS AND METHODS: A cross-sectional comparative study of mPAP and tricuspid annular plane systolic excursion of 21 children with CKD and age- and sex-matched controls asymptomatic for cardiac disease was conducted. RESULTS: Median mPAP was 27.69 (18.3-36.1) mmHg in CKD patients compared with 14.55 (13.5-17.1) mmHg in controls (P = 0.002). Elevated mPAP was present in 42.9% of CKD group and 0% in controls (P < 0.001). The prevalence of right ventricle (RV) dysfunction in CKD was 9.5% and 0% in controls (P = 0.49). Right ventricular dysfunction was significantly more common in patients with elevated mPAP compared with those with normal mPAP (P < 0.001). Children with CKD who had a history of having been dialyzed were less likely to have elevated mPAP (P < 0.001). CONCLUSION: Elevated mPAP is significantly more common in children with CKD compared with controls. CKD population with mPAP elevation is more likely to have impaired RV function. The occurrence of elevated mPAP was more common in those who were never dialyzed.