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Updated: Aug 29, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Pulmonary hypertension screening in children with sickle cell disease
Kok Hoe Chan1, Syeda Hiba Rizvi2, Wilfredo De Jesus-Rojas3
1Division of Hematology/Oncology, Department of Internal Medicine, McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Insights
Pulmonary hypertension (PHT) screening in children with sickle cell disease (SCD) using symptoms, echocardiograms, or biomarkers is unreliable. PHT prevalence was low in children on hydroxyurea, suggesting screening may not be necessary for this group.
Area of Science:
- Pediatric Hematology
- Cardiology
- Pulmonology
Background:
- Pulmonary hypertension (PHT) screening is recommended for children with sickle cell disease (SCD).
- Current screening methods for PHT in pediatric SCD are not well-defined.
- This study evaluated PHT screening tools in children with SCD.
Purpose of the Study:
- To assess the utility of PHT symptoms, echocardiography (ECHO), NT-proBNP, and BNP in screening for PHT in pediatric SCD patients.
- To determine the correlation between PHT symptoms and objective PHT findings in this population.
Main Methods:
- Prospective study of children (8-18 years) with SCD-HbSS and HbSthal° undergoing PHT screening.
- Screening included symptom evaluation, ECHO, NT-proBNP, and BNP levels.
- Analysis compared symptomatic and asymptomatic children regarding biomarkers and other clinical factors.
Main Results:
- Of 73 children, 37% reported PHT symptoms (e.g., exertional dyspnea, fatigue).
- Only 4.2% of analyzed ECHO results (n=48) indicated PHT.
- No significant differences in NT-proBNP, BNP, or other tested parameters were found between symptomatic and asymptomatic children.
Conclusions:
- PHT symptoms do not correlate with ECHO, NT-proBNP, or BNP findings in pediatric SCD.
- PHT prevalence was low in children on hydroxyurea, questioning the need for routine screening in this subgroup.
- Current screening approaches for PHT in pediatric SCD require re-evaluation.
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.
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