Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Jan;70(1):e29980.
doi: 10.1002/pbc.29980. Epub 2022 Sep 7.

Pulmonary hypertension screening in children with sickle cell disease

Kok Hoe Chan  1 , Syeda Hiba Rizvi  2 , Wilfredo De Jesus-Rojas  3 , James M Stark  2 , Ricardo A Mosquera  2 , Adriana Carolina Prada-Ruiz  2 , Traci Gonzales  4 , Deborah L Brown  2 , Neethu M Menon  2 , Trinh T Nguyen  4 , Cindy K Jon  2 , Aravind Yadav  2
Affiliations

Pulmonary hypertension screening in children with sickle cell disease

Kok Hoe Chan et al. Pediatr Blood Cancer. 2023 Jan.

Abstract

Background: Screening for pulmonary hypertension (PHT) is recommended in children with sickle cell disease (SCD). However, best approaches are poorly described. We examined the utility of PHT symptoms, echocardiogram (ECHO), N-terminal-pro hormone brain natriuretic peptide (NT-proBNP), and BNP to screen for PHT in the SCD pediatric population.

Methods: Children (8-18 years old) with SCD-HbSS and HbSthal° were prospectively included and underwent PHT screening. The screening consisted of a comprehensive PHT symptoms evaluation, ECHO measurement, and NT-proBNP and BNP levels.

Results: A total of 73 patients were included (mean age 12 ± 5.7 years; >80% on hydroxyurea), of which 37% had a symptom consistent with PHT, including exertional dyspnea (26.5%), fatigue (17.6%), palpitation (14.7%), and chest pain (10.3%). ECHO was obtained in 53 (72.6%) patients, with only ECHO of 48 patients included in the final analysis. Elevated ECHO peak tricuspid regurgitant jet velocity (TRV) >2.5 m/s or indirect findings to suggest PHT were seen in only two of 48 (4.2%). No significant differences were seen between those with and without PHT symptoms when compared for NT-proBNP, BNP, hemoglobin, pulmonary function testing, fractional exhaled nitric oxide, asthma, oxygen saturation, and sleep apnea.

Conclusion: PHT symptoms are not consistent with ECHO, NT-proBNP nor BNP findings in children with SCD. PHT prevalence based on TRV was low in children on hydroxyurea, therefore screening may not be warranted for this group.

Keywords: BNP; ECHO; proBNP; pulmonary hypertension; screening; sickle cell disease.

PubMed Disclaimer

References

REFERENCES

    1. Gladwin MT, Sachdev V, Jison ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. 2004;350(9):886-895.
    1. Ataga KI, Moore CG, Jones S, et al. Pulmonary hypertension in patients with sickle cell disease: a longitudinal study. Br J Haematol. 2006;134(1):109-115.
    1. De Castro LM, Jonassaint JC, Graham FL, Ashley-Koch A, Telen MJ. Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes. Am J Hematol. 2008;83(1):19-25.
    1. Sachdev V, Machado RF, Shizukuda Y, et al. Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease. J Am Coll Cardiol. 2007;49(4):472-479.
    1. Allen KY, Jones S, Jackson T, et al. Echocardiographic screening of cardiovascular status in pediatric sickle cell disease. Pediatr Cardiol. 2019;40(8):1670-1678.