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Title of Journal: Pediatr Radiol

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Abbravation: Pediatric Radiology

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Springer Berlin Heidelberg

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1432-1998

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CTdefined phenotype of pulmonary artery stenoses

Authors: Renee M Rodriguez Jeffrey A Feinstein Frandics P Chan
Publish Date: 2016/04/04
Volume: 46, Issue: 8, Pages: 1120-1127
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Abstract

A retrospective chart review identified patients with Alagille syndrome who had undergone CT angiography Pulmonary trunk MPA left main pulmonary artery LPA and right main pulmonary artery RPA diameters in Alagille patients were compared with those from matched control subjects Stenoses at lobar and segmental pulmonary arteries were categorized as Grade 1 33 stenosis Grade 2 3366 stenosis or Grade 3 66 stenosis Involvement among the different lung regions was then comparedFifteen patients ages 6 months to 17 years were identified one had surgical augmentation of the central pulmonary arteries and was excluded from the central main right and left pulmonary artery analysis The proximal LPA and RPA but not the MPA were significantly smaller than those of the control subjects P001 The proximal LPA was significantly smaller than the proximal RPA P001 in the Alagille group 055 LPARPA ratio Within the Alagille group 75 of the lobar and segmental branches showed mild or no stenoses Grade 1 17 showed moderate stenosis Grade 2 and 8 showed severe stenosis Grade 3 While not statistically significant the right lung demonstrated a greater percentage of Grades 2 and 3 stenoses 28 right vs 20 left P=01 The right middle and lingula lobes of both lungs showed more Grade 2 and 3 stenoses 33 upper/middle vs 18 lower P001We describe a common pattern pulmonary artery stenosis in Alagille patients consisting of severe proximal LPA stenosis heavy involvement of the lobar and segmental branches more often right than left and a greater involvement of the upper lobes Knowledge of this phenotypic pattern can help in the diagnosis of Alagille syndrome in patients presenting with pulmonary artery stenosis

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