CT of the chest in the evaluation of idiopathic pulmonary arterial hypertension in children.
BACKGROUND
Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease in children. By definition it is a diagnosis of exclusion, and CT of the chest is primarily performed to exclude other causes. Previous studies have defined CT features suggestive of the diagnosis of IPAH, but these have all been limited to the adult population.


OBJECTIVE
Contrast-enhanced chest CT and high-resolution CT findings in IPAH were evaluated in an attempt to define features consistently seen in children with this condition.


METHODS
The chest CT scans performed at initial presentation were reviewed in 17 children with echocardiographic or angiographic evidence of IPAH.


RESULTS
There were nine boys and eight girls, ranging in age from 1 month to 17 years. The extrapulmonary findings included cardiomegaly with right-sided cardiac enlargement, which was seen in 13 children. The central pulmonary arteries were enlarged in 15 children, with peripheral enlargement in two. In six children this resulted in bronchial compression. In addition, mediastinal and hilar lymphadenopathy was noted in three children. Prominent intrapulmonary features included a peripheral vasculopathy, with enlarged tortuous vessels, seen in eight children. Ill-defined ground-glass centrilobular opacities were also noted in eight children, representing the most common parenchymal abnormality. Other findings included septal lines in five, diffuse ground-glass opacification in four and focal hyperlucent zones in three. Mosaic attenuation was seen in one child.


CONCLUSIONS
A variety of imaging findings are identified in IPAH. Features particularly consistent with the diagnosis include peripheral vasculopathy and centrilobular opacities in the setting of cardiomegaly and central pulmonary arterial enlargement.
Chaudry
Gulraiz
G
Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Canada. gulraiz.chaudry@childrens.harvard.edu
MacDonald
Cathy
C
Adatia
Ian
I
Gundogan
Munire
M
Manson
David
D
D000293:Adolescent; D000792:Angiography; D001982:Bronchial Diseases / Q000000981:diagnostic imaging; D006332:Cardiomegaly / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D004452:Echocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging*; D017380:Hypertrophy, Right Ventricular / Q000000981:diagnostic imaging; D007091:Image Processing, Computer-Assisted / Q000379:methods; D007223:Infant; D019568:Lung, Hyperlucent / Q000000981:diagnostic imaging; D008297:Male; D016491:Peripheral Vascular Diseases / Q000000981:diagnostic imaging; D011651:Pulmonary Artery / Q000000981:diagnostic imaging; D013902:Radiography, Thoracic / Q000379:methods; D014057:Tomography, X-Ray Computed / Q000379:methods
D003287:Contrast Media