TY - JOUR
T1 - Prospective Echocardiography Assessment of Pulmonary Hypertension and Its Potential Etiologies in Children With Sickle Cell Disease
AU - Dham, Niti
AU - Ensing, Gregory
AU - Minniti, Caterina
AU - Campbell, Andrew
AU - Arteta, Manuel
AU - Rana, Sohail
AU - Darbari, Deepika
AU - Nouraie, Mehdi
AU - Onyekwere, Onyinye
AU - Lasota, Malgorzata
AU - Kato, Gregory J.
AU - Gladwin, Mark T.
AU - Castro, Oswaldo
AU - Gordeuk, Victor
AU - Sable, Craig
N1 - Funding Information:
This study was supported in part by Grants 2 R25 HL003679-08 and 1 R01 HL079912-02 from the National Heart, Lung, and Blood Institute, Bethesda, Maryland; by Howard University General Clinical Research Center Grant 2MOI RR10284-10 from the National Center for Research Resources, National Institutes of Health, Bethesda, Maryland; and by the Intramural Research Program of the National Institutes of Health.
Funding Information:
This observational, prospective, and multicenter study of children and adolescents with SCD and matched controls was supported by the National Institutes of Health, Bethesda, Maryland (Grant 1 R01 HL079912-01). The centers included were the Children's National Medical Center, Washington, District of Columbia; Howard University, Washington, District of Columbia; the National Institutes of Health; and the University of Michigan, Ann Arbor, Michigan ( ClinicalTrials.gov identifier NCT00495638 ). The study was approved by the institutional review boards of all participating institutions. Assent and consent were obtained from each patient and a family member of each patient (for patients aged <18 years).
PY - 2009/9/1
Y1 - 2009/9/1
N2 - Pulmonary hypertension (PH) is associated with adverse outcomes in adults with sickle-cell disease (SCD), but its importance in children is less clear. The aim of this study was to define the incidence and causes of PH in pediatric patients with SCD. Children with SCD (n = 310) and matched controls (n = 54) were prospectively enrolled under basal conditions. Participants underwent echocardiography, pulse oximetry, 6-minute walk tests, and hematologic testing. Echocardiographic measures were compared between patients with SCD and control subjects before and after adjusting for hemoglobin. Correlations of echocardiographic and clinical parameters were determined. Tricuspid regurgitation velocity (TRV) was elevated compared to controls (2.28 vs 2.10 m/s, p <0.0001). Increased TRV was associated with left ventricular diastolic diameter, hemoglobin, and estimated left atrial pressure. TRV remained elevated when controlling for left ventricular diameter and left atrial pressure. Echocardiographically derived pulmonary resistance was not significantly different between patients with SCD and controls, although it was elevated in the SCD subgroup with elevated TRV. When controlling for hemoglobin, TRV was no longer statistically different, but pulmonary insufficiency velocity, septal wall thickness, and estimated pulmonary resistance were statistically higher. TRV, pulmonary insufficiency end-diastolic velocity, and markers of increased cardiac output were correlated with indicators of adverse functional status, including history of acute chest syndrome, stroke, transfusions, and 6-minute walk distance. In conclusion, children with SCD had mildly increased TRV that was correlated with increased cardiac output and left ventricular filling pressures. Hemoglobin-adjusted analysis also suggested a contribution of primary vascular changes.
AB - Pulmonary hypertension (PH) is associated with adverse outcomes in adults with sickle-cell disease (SCD), but its importance in children is less clear. The aim of this study was to define the incidence and causes of PH in pediatric patients with SCD. Children with SCD (n = 310) and matched controls (n = 54) were prospectively enrolled under basal conditions. Participants underwent echocardiography, pulse oximetry, 6-minute walk tests, and hematologic testing. Echocardiographic measures were compared between patients with SCD and control subjects before and after adjusting for hemoglobin. Correlations of echocardiographic and clinical parameters were determined. Tricuspid regurgitation velocity (TRV) was elevated compared to controls (2.28 vs 2.10 m/s, p <0.0001). Increased TRV was associated with left ventricular diastolic diameter, hemoglobin, and estimated left atrial pressure. TRV remained elevated when controlling for left ventricular diameter and left atrial pressure. Echocardiographically derived pulmonary resistance was not significantly different between patients with SCD and controls, although it was elevated in the SCD subgroup with elevated TRV. When controlling for hemoglobin, TRV was no longer statistically different, but pulmonary insufficiency velocity, septal wall thickness, and estimated pulmonary resistance were statistically higher. TRV, pulmonary insufficiency end-diastolic velocity, and markers of increased cardiac output were correlated with indicators of adverse functional status, including history of acute chest syndrome, stroke, transfusions, and 6-minute walk distance. In conclusion, children with SCD had mildly increased TRV that was correlated with increased cardiac output and left ventricular filling pressures. Hemoglobin-adjusted analysis also suggested a contribution of primary vascular changes.
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U2 - 10.1016/j.amjcard.2009.04.034
DO - 10.1016/j.amjcard.2009.04.034
M3 - Article
C2 - 19699350
AN - SCOPUS:68749095213
SN - 0002-9149
VL - 104
SP - 713
EP - 720
JO - American Journal of Cardiology
JF - American Journal of Cardiology
IS - 5
ER -