High Prevalence of Pulmonary Hypertension in Children With Sickle Cell Disease

Abstract

Pulmonary hypertension (PHT) affects approximately 30% of adults with sickle cell disease. Adults with PHT have a significantly higher mortality rate. We report the results of a prospective study of the prevalence of PHT among children with sickle cell disease. In our cohort, 31% of children>or=10 years of age have evidence of PHT by Doppler echocardiography. Factors associated with the presence of PHT are male sex and elevated reticulocyte count. We recommend screening all children for PHT starting at the age of 10 years.

Pulmonary hypertension in sickle cell disease children under 10 years of age

“…Prevalence of PH was 21AE6%. This value is in the range of other prospective studies involving children during steady state, which observed a prevalence of PH ranging from 11% to 30% (Nelson et al, 2007;Pashankar et al, 2008;Minniti et al, 2009). Our data are the first to show a high prevalence of elevated TRV in very young children (see Table V).…”

“…On the contrary, besides the reticulocytes, other parameters of haemolysis, including degree of anaemia, lactate dehydrogenase and bilirubin levels were not different between the two groups, and other parameters of inflammation, besides the platelets, were also not different. Correlation of higher reticulocyte and platelet counts was demonstrated by other studies (Nelson et al, 2007;Pashankar et al, 2008;Lee et al, 2009), but these also failed to determine correlation with other determinants of haemolysis. In our study, the relatively small sample population could explain the lack of significance of the other markers of haemolysis or inflammation.…”

“…Our data confirmed recent reports (Hagar et al, 2008;Minniti et al, 2009) that also revealed an association of ACS and elevated TRV. Other studies failed to confirm this association both in children and adults (Gladwin et al, 2004;Nelson et al, 2007;Pashankar et al, 2008), but they outlined instead the importance of steady state hypoxia in the development of PH. In our study, patients with elevated TRV did not display steady state hypoxia and steady state SatO 2 was similar in the two groups.…”

“…In children, the reported prevalence of PH, defined as an elevated Tricuspid Regurgitant Velocity (TRV) ‡2AE5 m/s on echocardiographic measurement, varied from 16-30% in retrospective series (Ambrusko et al, 2006;Qureshi et al, 2006;Hagar et al, 2008) to 8AE3-33% in recent prospective series (Liem et al, 2007(Liem et al, , 2009Nelson et al, 2007;Onyekwere et al, 2008;Lee et al, 2009;Minniti et al, 2009). The mean age of the children in all these series was above 10 years and a great proportion of children was receiving treatment due to other manifestations of SCD (chronic transfusion or hydroxycarbamide).…”

Relationship of erythropoietin, fetal hemoglobin, and hydroxyurea treatment to tricuspid regurgitation velocity in children with sickle cell disease

“…Some studies have found an association of high hemoglobin F with lower pulmonary hypertension risk, [11][12][13][14] but several others have detected no such association. 1,2,[15][16][17][18] We recently reported a prospective, multicenter study of 310 children and adolescents with sickle cell disease at steady state in which tricuspid regurgitation velocity of 2.6 m/s or higher occurred in 11% of participants and had independent associations with hemolysis and hemoglobin oxygen desaturation. 3 In addition, we have found that an elevated screening tricuspid regurgitation velocity in children and adolescents with sickle cell disease predicts functional impairment over 2 years of follow-up (V.R.G., unpublished observations, April 2009).…”