BACKGROUND: Pulmonary artery hypertension (PAH) is a known complication of patients with sickle cell disease (SCD). The prevalence of PAH has been reported to be high in children with SCD in developed countries. The mortality rate of patients with SCD who develop PAH is said to be 40% at 24 to 40 months after diagnosis. In Sub-Saharan Africa, particularly Nigeria, where the prevalence of SCD is high, there is a dearth of data on the prevalence of PAH in children with SCD. PAH may be a likely contributor to the unacceptably high mortality rate of children with SCD in Africa. The present study aimed to determine the pattern of pulmonary artery pressure in children with sickle cell anaemia (SCA) aged 1 to 12 years in their steady state using Doppler echocardiography in our environment. METHODS: Analytical, comparative and cross-sectional study. It was carried out at Lagos State University Teaching Hospital (LASUTH) over a period of seven months (31st August 2015 through 31st March 2016). A total of 400 children, aged 1 to 12 years were recruited following parental consent and assent. Two hundred children with SCA in steady state and 200 age and sex matched children with haemoglobin genotype AA who served as controls were studied. All subjects and controls had Doppler echocardiography performed on them by the author. PAH was defined as a pulmonary artery systolic pressure (PASP) of ≥30 mmHg at rest derived from the tricuspid regurgitant velocity (TRV) measured from Doppler echocardiography. This corresponds with TRV value of ≥2.5 m/s. RESULTS: The prevalence of PAH in children with SCA was 8% (n=16). None of the controls had PAH. The youngest subject with PAH was aged 2 years. The overall mean pulmonary artery pressure of children with SCA was significantly higher than that of controls (18.54±5.81 vs. 13.76±5.71 mmHg, P=0.000). The prevalence of PAH in children with SCA demonstrated an increase in trend with age. CONCLUSIONS: The prevalence of PAH in children with SCA in steady state is high. This complication was noticed as early as the second year of life. It is recommended that all children with SCA should have periodic Doppler echocardiography for early detection of PAH.
BACKGROUND: Pulmonary artery hypertension (PAH) is a known complication of patients with sickle cell disease (SCD). The prevalence of PAH has been reported to be high in children with SCD in developed countries. The mortality rate of patients with SCD who develop PAH is said to be 40% at 24 to 40 months after diagnosis. In Sub-Saharan Africa, particularly Nigeria, where the prevalence of SCD is high, there is a dearth of data on the prevalence of PAH in children with SCD. PAH may be a likely contributor to the unacceptably high mortality rate of children with SCD in Africa. The present study aimed to determine the pattern of pulmonary artery pressure in children with sickle cell anaemia (SCA) aged 1 to 12 years in their steady state using Doppler echocardiography in our environment. METHODS: Analytical, comparative and cross-sectional study. It was carried out at Lagos State University Teaching Hospital (LASUTH) over a period of seven months (31st August 2015 through 31st March 2016). A total of 400 children, aged 1 to 12 years were recruited following parental consent and assent. Two hundred children with SCA in steady state and 200 age and sex matched children with haemoglobin genotype AA who served as controls were studied. All subjects and controls had Doppler echocardiography performed on them by the author. PAH was defined as a pulmonary artery systolic pressure (PASP) of ≥30 mmHg at rest derived from the tricuspid regurgitant velocity (TRV) measured from Doppler echocardiography. This corresponds with TRV value of ≥2.5 m/s. RESULTS: The prevalence of PAH in children with SCA was 8% (n=16). None of the controls had PAH. The youngest subject with PAH was aged 2 years. The overall mean pulmonary artery pressure of children with SCA was significantly higher than that of controls (18.54±5.81 vs. 13.76±5.71 mmHg, P=0.000). The prevalence of PAH in children with SCA demonstrated an increase in trend with age. CONCLUSIONS: The prevalence of PAH in children with SCA in steady state is high. This complication was noticed as early as the second year of life. It is recommended that all children with SCA should have periodic Doppler echocardiography for early detection of PAH.
Authors: Wyman W Lai; Tal Geva; Girish S Shirali; Peter C Frommelt; Richard A Humes; Michael M Brook; Ricardo H Pignatelli; Jack Rychik Journal: J Am Soc Echocardiogr Date: 2006-12 Impact factor: 5.251
Authors: Mary Nell Suell; Louis I Bezold; M Fatih Okcu; Donald H Mahoney; Felix Shardonofsky; Brigitta U Mueller Journal: J Pediatr Hematol Oncol Date: 2005-12 Impact factor: 1.289
Authors: Kenneth I Ataga; Namita Sood; Guy De Gent; Eileen Kelly; Ashley G Henderson; Susan Jones; Dell Strayhorn; Alice Lail; Susan Lieff; Eugene P Orringer Journal: Am J Med Date: 2004-11-01 Impact factor: 4.965
Authors: Mark T Gladwin; Vandana Sachdev; Maria L Jison; Yukitaka Shizukuda; Jonathan F Plehn; Karin Minter; Bernice Brown; Wynona A Coles; James S Nichols; Inez Ernst; Lori A Hunter; William C Blackwelder; Alan N Schechter; Griffin P Rodgers; Oswaldo Castro; Frederick P Ognibene Journal: N Engl J Med Date: 2004-02-26 Impact factor: 91.245