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.

Keywords::

• hemolysis
• nitric oxide
• pediatrics
• pulmonary hypertension
• sickle cell disease
• tricuspid regurgitant jet velocity

Financial & competing interests disclosure

Erika B Rosenzweig has received clinical research support from Actelion, Eli Lilly, Gilead Science, Pfizer, United Therapeutics and Novartis. She has also received honoraria for attendance at Actelion, Gilead Science and United Therapeutics scientific advisory board meetings. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.