title: Pulmonary hypertension in children with sickle cell disease.
abstract: Pulmonary hypertension and cor pulmonale have long been known to be complications of sickle cell disease, thought mostly to affect the adult population. Recently, pediatric studies in sickle cell patients have uncovered a similar prevalence of elevated tricuspid regurgitation jet velocities, a finding consistent with increased right-sided pressures, when compared with adult counterparts. Implications on mortality to date, while significant in the adult sickle cell population, appear to be unsubstantiated in children and adolescents. We review the recent literature in an attempt to discuss the prevalence, etiology and implications of an elevated tricuspid regurgitation jet velocity in pediatric sickle cell patients. We also suggest screening, diagnostics, treatment and follow-up plans that may improve the disease burden of pulmonary hypertension in both pediatric and adult sickle cell populations.
authors.affiliation: Columbia University Medical Center, 3959 Broadway, New York, NY 10032, USA. wz2116@columbia.edu
authors.forename: Warren A
authors.initials: WA
authors.lastname: Zuckerman
authors.forename: Erika B
authors.initials: EB
authors.lastname: Rosenzweig
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D011237:Predictive Value of Tests; D012307:Risk Factors; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000503:physiopathology; D014262:Tricuspid Valve Insufficiency / Q000209:etiology / Q000503:physiopathology; D055815:Young Adult