Pediatric Radiology

, Volume 49, Issue 5, pp 575–585 | Cite as

Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease

  • Laureline BertelootEmail author
  • Maïa Proisy
  • Jean-Philippe Jais
  • Marilyne Lévy
  • Nathalie Boddaert
  • Damien Bonnet
  • Francesca Raimondi
Original Article



In children, idiopathic and heritable pulmonary arterial hypertension present echocardiographic and heart catheterization findings similar to findings in pulmonary veno-occlusive disease.


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.


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).


CT angiography could contribute to early diagnosis and prediction of severity in children with pulmonary arterial hypertension.


Adolescents Children Computed tomography angiography Familial primary pulmonary hypertension Pulmonary veno-occlusive disease Telangiectasia 


Compliance with ethical standards

Conflicts of interest


Supplementary material

247_2018_4331_MOESM1_ESM.docx (19 kb)
ESM 1 (DOCX 18 kb)
247_2018_4331_MOESM2_ESM.png (13 kb)
Supplementary Figure 1 Receiver operating characteristic curve for the assessment of the best threshold value for the ratio between the diameter of the main pulmonary artery and the diameter of ascending aorta (95% confidence interval [CI]: 0.92–1.00; 2,000 stratified bootstrap replicates) (PNG 12 kb)
247_2018_4331_MOESM3_ESM.png (13 kb)
Supplementary Figure 2 Receiver operating characteristic curve for the assessment of the best threshold value for the ratio between the transverse diameter of right atrium and the transverse diameter of the left atrium (95% CI: 0.81–0.96; 2,000 stratified bootstrap replicates) (PNG 13 kb)
247_2018_4331_MOESM4_ESM.png (13 kb)
Supplementary Figure 3 Receiver operating characteristic curve for the assessment of the best threshold value for the ratio between the transverse diameter of the right ventricle and the transverse diameter of the left ventricle (95% CI: 0.91–0.99; 2,000 stratified bootstrap replicates) (PNG 12 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Pediatric RadiologyHôpital Universitaire Necker-Enfants malades, Assistance Publique des Hôpitaux de ParisParisFrance
  2. 2.UMR 1163, Institut ImagineParisFrance
  3. 3.Department of Pediatric RadiologyCentre Hospitalier UniversitaireRennesFrance
  4. 4.Department of BiostatisticsHôpital Necker-Enfants maladesParisFrance
  5. 5.PRES Sorbonne Paris CitéUniversity René DescartesParisFrance
  6. 6.M3C-Necker, Congenital and Pediatric CardiologyHôpital Universitaire Necker-Enfants maladesParisFrance

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