Joye Raphael, Wacker Julie, Nguyen Duy-Anh, Hachulla Anne-Lise, Maggio Albane B R, Cimasoni Laurent, Lador Frederic, Ansari Marc, Beghetti Maurice
Pediatric Cardiology Unit, Department of Woman, Child, and Adolescent Medicine, Geneva University Hospitals, Geneva, Switzerland.
Pulmonary Hypertension Program, Geneva University Hospital, Geneva, Switzerland.
Front Pediatr. 2023 Aug 30;11:1221977. doi: 10.3389/fped.2023.1221977. eCollection 2023.
Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD.
This prospective observational pilot study was conducted at the Geneva University Hospitals and was approved by the local human ethics committee (CCER 2019-01975). A written informed consent was obtained from the patients and/or their legal guardian. Eight children (consisting of five girls and three boys) with homozygous SCD were included in the study. They underwent full cardiological workup using transthoracic echocardiography (TTE) and cardiopulmonary exercise test (CPET), as well as DECT.
The median age of the children was 11 years old (range 8-12). All patients exhibited a normal biventricular systo-diastolic function using the TTE. The median tricuspid regurgitant jet velocity value was 2.24 m/s (range 1.96-2.98). Four children were found to have signs of vasculopathy detected on DECT. Of them, two had abnormal screening test results. They both had an increased VE/VCO slope during CPET and an increased TVR of >2.5 m/s on TTE.
DECT is capable of identifying early signs of pulmonary vascular disease in children with SCD. Further studies are needed to understand the correlation between DECT abnormalities and hemodynamic pulmonary circulation better.
肺动脉高压（PH）是镰状细胞病（SCD）一种罕见但致命的并发症，如果早期治疗可能可逆。双能计算机断层扫描（DECT）是诊断PH的一种有价值的工具。我们试图确定DECT能否检测出SCD患儿PH的早期迹象。
这项前瞻性观察性试点研究在日内瓦大学医院进行，并获得当地人类伦理委员会（CCER 2019-01975）的批准。从患者和/或其法定监护人处获得书面知情同意书。八名纯合子SCD患儿（五名女孩和三名男孩）纳入研究。他们接受了经胸超声心动图（TTE）、心肺运动试验（CPET）以及DECT的全面心脏检查。
患儿的中位年龄为11岁（范围8-12岁）。所有患者经TTE检查显示双心室收缩-舒张功能正常。三尖瓣反流喷射速度的中位值为2.24 m/s（范围1.96-2.98）。四名患儿经DECT检查发现有血管病变迹象。其中两名筛查试验结果异常。他们在CPET期间VE/VCO斜率均增加，且经TTE检查三尖瓣反流速度均>2.5 m/s。
DECT能够识别SCD患儿肺血管疾病的早期迹象。需要进一步研究以更好地了解DECT异常与肺循环血流动力学之间的相关性。
