Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease.
Adolescent
Cardiac Catheterization
Case-Control Studies
Child
Child, Preschool
Computed Tomography Angiography
Early Diagnosis
Familial Primary Pulmonary Hypertension
/ diagnostic imaging
Female
Humans
Hypertension, Pulmonary
/ diagnostic imaging
Infant
Male
Predictive Value of Tests
Pulmonary Veno-Occlusive Disease
/ diagnostic imaging
Radiographic Image Interpretation, Computer-Assisted
Retrospective Studies
Sensitivity and Specificity
Adolescents
Children
Computed tomography angiography
Familial primary pulmonary hypertension
Pulmonary veno-occlusive disease
Telangiectasia
Journal
Pediatric radiology
ISSN: 1432-1998
Titre abrégé: Pediatr Radiol
Pays: Germany
ID NLM: 0365332
Informations de publication
Date de publication:
05 2019
05 2019
Historique:
received:
21
03
2018
accepted:
12
12
2018
revised:
22
10
2018
pubmed:
18
1
2019
medline:
18
5
2019
entrez:
18
1
2019
Statut:
ppublish
Résumé
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. 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.
Sections du résumé
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.
Identifiants
pubmed: 30652195
doi: 10.1007/s00247-018-04331-y
pii: 10.1007/s00247-018-04331-y
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
575-585Références
J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D34-41
pubmed: 24355639
J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D117-26
pubmed: 24355636
Pediatr Radiol. 2007 Apr;37(4):345-50
pubmed: 17279402
Lancet. 2012 Feb 11;379(9815):537-46
pubmed: 22240409
J Med Genet. 2013 Aug;50(8):500-6
pubmed: 23592887
Clin Radiol. 2009 Aug;64(8):751-60
pubmed: 19589413
Eur Respir J. 2015 Oct;46(4):903-75
pubmed: 26318161
Radiology. 2005 Apr;235(1):274-81
pubmed: 15703314
Semin Respir Crit Care Med. 2009 Aug;30(4):411-20
pubmed: 19634080
Circulation. 2005 Nov 1;112(18):2778-85
pubmed: 16267251
J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D13-21
pubmed: 24355637
Hum Genet. 2014 May;133(5):471-9
pubmed: 24442418
Clin Radiol. 2007 Jun;62(6):587-95
pubmed: 17467397
Circulation. 2015 Nov 24;132(21):2037-99
pubmed: 26534956
Chest. 2014 Jul;146(1):167-174
pubmed: 24503954
AJR Am J Roentgenol. 2004 Jan;182(1):67-72
pubmed: 14684514
Cancer. 1950 Jan;3(1):32-5
pubmed: 15405679
Pediatr Radiol. 2016 Sep;46(10):1378-83
pubmed: 27194438
Radiol Med. 2014 Sep;119(9):667-73
pubmed: 24297597
Chest. 2013 Feb 1;143(2):344-348
pubmed: 22922444
Nat Genet. 2014 Jan;46(1):65-9
pubmed: 24292273
Radiology. 2008 Mar;246(3):697-722
pubmed: 18195376
Radiographics. 2008 Jan-Feb;28(1):171-84
pubmed: 18203937
Anatol J Cardiol. 2015 Oct;15(10):843-7
pubmed: 26477721
Heart. 2016 May;102 Suppl 2:ii30-5
pubmed: 27053695
Medicine (Baltimore). 2014 Dec;93(27):e256
pubmed: 25501096