title: Prospective echocardiography assessment of pulmonary hypertension and its potential etiologies in children with sickle cell disease.
abstract: 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.
authors.affiliation: Department of Cardiology, Children's National Medical Center, Washington, District of Columbia, USA.
authors.forename: Niti
authors.initials: N
authors.lastname: Dham
authors.forename: Gregory
authors.initials: G
authors.lastname: Ensing
authors.forename: Caterina
authors.initials: C
authors.lastname: Minniti
authors.forename: Andrew
authors.initials: A
authors.lastname: Campbell
authors.forename: Manuel
authors.initials: M
authors.lastname: Arteta
authors.forename: Sohail
authors.initials: S
authors.lastname: Rana
authors.forename: Deepika
authors.initials: D
authors.lastname: Darbari
authors.forename: Mehdi
authors.initials: M
authors.lastname: Nouraie
authors.forename: Onyinye
authors.initials: O
authors.lastname: Onyekwere
authors.forename: Malgorzata
authors.initials: M
authors.lastname: Lasota
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Kato
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
authors.forename: Oswaldo
authors.initials: O
authors.lastname: Castro
authors.forename: Victor
authors.initials: V
authors.lastname: Gordeuk
authors.forename: Craig
authors.initials: C
authors.lastname: Sable
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D002302:Cardiac Output; D002648:Child; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D008297:Male; D011446:Prospective Studies; D014261:Tricuspid Valve / Q000503:physiopathology; D014655:Vascular Resistance; D016277:Ventricular Function, Left
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