ArticlePediatric radiology2019
Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease.
Article in Pediatric radiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers, 1 of them a synthesis that pooled it.
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Who cites it
6 citing papers in PubMed, 1 synthesis or guideline pooled it, 11 citations in OpenAlex.
- Interventional Strategies for Children with Progressive Pulmonary Hypertension Despite Optimal Therapy: An Official American Thoracic Society Clinical Practice Guideline.American journal of respiratory and critical care medicine · 2025Guideline
- CT Features of Sickle Cell Disease-Associated Pulmonary Hypertension.Journal of clinical medicine · 2026Article
- Diagnostic performance of the main pulmonary artery-to-ascending aorta diameter ratio for pulmonary hypertension defined by mean pulmonary arterial pressure in children with left-to-right shunt congenital heart disease.Quantitative imaging in medicine and surgery · 2026Article
- Growth-adjusted reference values for the main pulmonary artery-to-ascending aorta ratio derived from clinically indicated chest CT in children.European radiology · 2026Article
- Pulmonary veno-occlusive disease in childhood-a rare disease not to be missed.Cardiovascular diagnosis and therapy · 2021Review
- Cross-sectional imaging of congenital pulmonary artery anomalies.The international journal of cardiovascular imaging · 2019Review
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIn children, idiopathic and heritable pulmonary arterial hypertension present echocardiographic and heart catheterization findings similar to findings in pulmonary veno-occlusive disease.
objectiveTo 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
methodsWe 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.
resultsThe 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).
conclusionCT angiography could contribute to early diagnosis and prediction of severity in children with pulmonary arterial hypertension.
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