Division 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.
Department of Pediatrics, McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth Houston) and Children's Memorial Hermann Hospital, Houston, Texas, USA.
Pediatr Blood Cancer. 2023 Jan;70(1):e29980. doi: 10.1002/pbc.29980. Epub 2022 Sep 7.
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.
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.
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.
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.
推荐对镰状细胞病（SCD）患儿进行肺动脉高压（PHT）筛查。然而，最佳方法描述不足。我们研究了 PHT 症状、超声心动图（ECHO）、N 末端前脑利钠肽（NT-proBNP）和 BNP 在 SCD 儿科人群中筛查 PHT 的作用。
前瞻性纳入 SCD-HbSS 和 HbSthal°的儿童（8-18 岁）并进行 PHT 筛查。筛查包括全面的 PHT 症状评估、ECHO 测量以及 NT-proBNP 和 BNP 水平。
共纳入 73 例患者（平均年龄 12±5.7 岁；80%以上接受羟基脲治疗），其中 37%存在符合 PHT 的症状，包括劳力性呼吸困难（26.5%）、疲劳（17.6%）、心悸（14.7%）和胸痛（10.3%）。53 例患者（72.6%）获得了 ECHO，仅有 48 例患者的 ECHO 结果被纳入最终分析。仅在 48 例患者中发现 2 例（4.2%）ECHO 峰三尖瓣反流速度（TRV）>2.5m/s 或存在间接提示 PHT 的发现。在比较有和无症状 PHT 的患者时，NT-proBNP、BNP、血红蛋白、肺功能检查、呼气一氧化氮分数、哮喘、氧饱和度和睡眠呼吸暂停无显著差异。
在 SCD 患儿中，PHT 症状与 ECHO、NT-proBNP 或 BNP 结果不一致。羟基脲治疗的儿童中基于 TRV 的 PHT 患病率较低，因此可能不需要对该人群进行筛查。
