儿童特发性、遗传性及静脉闭塞性肺动脉高压：疾病初始评估中的计算机断层扫描血管造影特征
Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease.
作者信息
Berteloot Laureline, Proisy Maïa, Jais Jean-Philippe, Lévy Marilyne, Boddaert Nathalie, Bonnet Damien, Raimondi Francesca
机构信息
Department of Pediatric Radiology, Hôpital Universitaire Necker-Enfants malades, Assistance Publique des Hôpitaux de Paris, Paris, France.
UMR 1163, Institut Imagine, Paris, France.
出版信息
Pediatr Radiol. 2019 May;49(5):575-585. doi: 10.1007/s00247-018-04331-y. Epub 2019 Jan 16.
BACKGROUND
In children, idiopathic and heritable pulmonary arterial hypertension present echocardiographic and heart catheterization findings similar to findings in pulmonary veno-occlusive disease.
OBJECTIVE
To provide a systematic analysis of CT angiography anomalies in children with idiopathic or heritable pulmonary arterial hypertension, or pulmonary veno-occlusive disease. We also sought to identify correlations between CT findings and patients' baseline characteristics.
MATERIALS AND METHODS
We retrospectively analyzed CT features of children with idiopathic and heritable pulmonary arterial hypertension or pulmonary veno-occlusive disease and 30 age-matched controls between 2008 and 2014. We compared CT findings and patient characteristics, including gene mutation type, and disease outcome until 2017.
RESULTS
The pulmonary arterial hypertension group included idiopathic (n=15) and heritable pulmonary arterial hypertension (n=11) and pulmonary veno-occlusive disease (n=4). Median age was 6.5 years. Children with pulmonary arterial hypertension showed enlargement of pulmonary artery and right cardiac chambers. A threshold for the ratio between the pulmonary artery and the ascending aorta of ≥1.2 had a sensitivity of 90% and a specificity of 100% for pulmonary arterial hypertension. All children with pulmonary veno-occlusive disease had thickened interlobular septa, centrilobular ground-glass opacities, and lymphadenopathy. In children with idiopathic and heritable pulmonary arterial hypertension, presence of intrapulmonary neovessels and enlargement of the right atrium were correlated with higher mean pulmonary artery pressure (P=0.011) and pulmonary vascular resistance (P=0.038), respectively. Mediastinal lymphadenopathy was associated with disease worsening within the first 2 years of follow-up (P=0.024).
CONCLUSION
CT angiography could contribute to early diagnosis and prediction of severity in children with pulmonary arterial hypertension.
背景
在儿童中，特发性和遗传性肺动脉高压的超声心动图及心导管检查结果与肺静脉闭塞病的检查结果相似。
目的
对特发性或遗传性肺动脉高压或肺静脉闭塞病患儿的CT血管造影异常进行系统分析。我们还试图确定CT表现与患者基线特征之间的相关性。
材料与方法
我们回顾性分析了2008年至2014年间特发性和遗传性肺动脉高压或肺静脉闭塞病患儿以及30名年龄匹配的对照儿童的CT特征。我们比较了CT表现和患者特征，包括基因突变类型，以及至2017年的疾病转归。
结果
肺动脉高压组包括特发性肺动脉高压（n = 15）、遗传性肺动脉高压（n = 11）和肺静脉闭塞病（n = 4）。中位年龄为6.5岁。肺动脉高压患儿表现为肺动脉和右心腔扩大。肺动脉与升主动脉比值≥1.2对肺动脉高压的敏感性为90%，特异性为100%。所有肺静脉闭塞病患儿均有小叶间隔增厚、小叶中心磨玻璃样影和淋巴结肿大。在特发性和遗传性肺动脉高压患儿中，肺内新生血管的存在和右心房扩大分别与较高的平均肺动脉压（P = 0.011）和肺血管阻力（P = 0.038）相关。纵隔淋巴结肿大与随访前2年内疾病恶化相关（P = 0.024）。
结论
CT血管造影有助于肺动脉高压患儿的早期诊断和严重程度预测。
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