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A2057 - Screening of Pulmonary Hypertension in a Cohort of Children with Sickle Cell Disease
Author Block: W. De Jesus-Rojas, R. A. Mosquera, C. DaCosta, J. M. Stark, C. K. Jon, K. McBeth, A. Yadav; Pediatric Pulmonary, McGovern Medical School, UTHealth, Houston, TX, United States.
Background: Pulmonary Hypertension (PHT) is a serious complication that affects individuals with Sickle Cell Disease (SCD). Approaches to early screening and detection of PHT in the pediatric population with SCD are poorly described. ProBNP and BNP levels may correlate with Echocardiogram (ECHO) as early markers to evaluate PHT in SCD children.
Objective: To determine the role of ProBNP and BNP levels compared to ECHO findings in the evaluation of PHT in Sickle cell anemia (HbSS subtype) children.
Methods: Forty-one (n=41) SCD-HgSS children (8-18 years-old) were prospectively enrolled from an outpatient comprehensive SCD Center [2016-2017]. PHT screening included: complete medical history for PHT symptoms, ProBNP, BNP levels and ECHO during steady state conditions. PHT was defined as a TRV > 2.5 m/s on ECHO.
Results: The median ProBNP and BNP were 64.5 (IQR: 41-146) pg/mL and 20.5 (IQR: 11-34) pg/mL, respectively. Thirteen percent (n=4/29) had PHT defined as an elevated TRV > 2.5 m/s on ECHO. Mean ProBNP and BNP levels in the TRV>2.5 m/s group were 238 (IQR: 104-341) pg/mL and 28.5 (IQR: 21-35) pg/mL, respectively as compared to non-TRV group [ProBNP: 86 (IQR: 42-142) pg/mL and BNP: 25 (IQR: 10-31)]. ProBNP levels were significantly elevated in children with a TRV>2.5 m/s as compare with normal TRV (p = 0.008). No significant difference in BNP levels was noted among groups (p=0.86).
Conclusion: ProBNP and/or ECHO maybe considered as part of a comprehensive evaluation for PHT in children with SCD-HbSS if clinically suspected.