File:Alagille syndrome with pulmonary hypertension (Radiopaedia 49384-54502 Sagittal MRA 1).jpg

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Description	Radiopaedia case ID: 49384 Image ID: 26450502 Plane projection: Sagittal Aux modality: MRA Modality: MRI System: Cardiac Findings: Pulmonary MRA and perfusion study clearly demonstrate left upper lobe pulmonary artery occlusion and right lower lobe segmental stenoses. Multiple segmental and subsegmental stenoses are also discernible within the right upper and left lower lobes. Systemic vessel from the left chest wall and bronchial artery branches supply the underperfused pulmonary lobes. These features are not demonstrated as well on CT pulmonary angiography (CTPA). Corresponding large left upper lobe pulmonary perfusion defect. Corresponding large right lower lobe pulmonary perfusion defect. Sagittal MRA showing multiple left lower lobe subsegmental stenoses, particularly affecting the anterobasal and basolateral segmental arteries. Cardiac 4 chamber steady state free precession (SSFP) demonstrates right ventricular hypertrophy with septal bowing to the left, which indicates development of associated pulmonary hypertension.
Date	Published: 22nd Nov 2016
Source	https://radiopaedia.org/cases/alagille-syndrome-with-pulmonary-hypertension
Author	Rachael O'Rourke
Permission (Permission-reusing-text)	http://creativecommons.org/licenses/by-nc-sa/3.0/

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