Dual-Energy CT of Unilateral Pulmonary Artery Agenesis

HomeRadiologyVol. 310, No. 3 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyDual-Energy CT of Unilateral Pulmonary Artery AgenesisFei Zheng, Xian-Zheng Tan Fei Zheng, Xian-Zheng Tan Author AffiliationsFrom the Department of Radiology, Hunan Provincial People's Hospital (First Affiliated Hospital of Hunan Normal University), No. 61 Jiefang W Rd, Changsha 410005, China.Address correspondence to X.Z.T. (email: [email protected]).Fei ZhengXian-Zheng Tan Published Online:Mar 12 2024https://doi.org/10.1148/radiol.232487MoreSectionsPDF ToolsAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookXLinked In Supplemental material is available for this article.A 58-year-old woman presented with 5 years of exertional dyspnea and bilateral leg edema, with progressive worsening over the past 2 months. Dual-energy CT pulmonary angiography helped confirm the diagnosis of congenital right pulmonary artery agenesis, with contralateral pulmonary arterial hypertension and ipsilateral aortopulmonary collateral vessels (Figure, A and B; Movie). Lung iodine mapping showed right lung hypoperfusion (Figure, C and D). The patient received medical therapy with bosentan and diuretics. Her condition improved, and she was discharged.Figure 1: Images in a 58-year-old woman with congenital right pulmonary artery agenesis. (A) Three-dimensional CT pulmonary angiogram in anteroposterior view reveals isolated absence of the right pulmonary artery. Tortuous, dilated central pulmonary arteries of the left lung indicate pulmonary arterial hypertension. (B) Volume-rendered three-dimensional reconstruction image in posteroanterior view demonstrates multiple dilated bronchial arteries (arrows) supplying the right lung, suggesting that a systemic collateral supply had developed. (C, D) Dual-energy lung perfused blood volume images (ie, iodine maps) in anteroposterior view show a shrunken right lung with substantially decreased perfusion compared with the left lungFigure 1:Download as PowerPoint Movie: Coronal contrast-enhanced CT images in a 58-year-old woman with congenital right pulmonary artery agenesis. Consecutive images show right pulmonary artery agenesis, with contralateral pulmonary arterial hypertension and ipsilateral aortopulmonary collateral vessels.Download Original Video (18.1 MB)Unilateral pulmonary artery agenesis is a rare developmental anomaly caused by failure in the connection of the sixth aortic arch with the pulmonary trunk (1,2). Herein, we present a case of unilateral pulmonary artery agenesis evaluated using dual-energy CT. This technique not only clearly shows the morphologic characteristics of the pulmonary vessels, but also demonstrates the perfusion abnormalities of this rare congenital anomaly (3).Disclosures of conflicts of interest: F.Z. No relevant relationships. X.Z.T. No relevant relationships.Supported by the Youth Medical Key Talents Foundation of Hunan Province (grant 20230508-1034) and Huxiang Youth Talent Support Program (grant 2020RC3067).References1. Cucci CE, Doyle EF, Lewis EW Jr. Absence of a primary division of the pulmonary trunk. an ontogenetic theory. Circulation 1964;29(1):124–131. Crossref, Medline, Google Scholar2. Taniguchi H, Saito J, Abo H, et al. Isolated unilateral absence of the pulmonary artery. Am J Respir Crit Care Med 2015;192(4):518–519. Crossref, Medline, Google Scholar3. Johnson TR, Thieme SF, Deutsch MA, et al. Images in cardiovascular medicine: unilateral pulmonary artery agenesis: noninvasive diagnosis with dual-source computed tomography. Circulation 2009;119(8):1158–1160. Crossref, Medline, Google ScholarArticle HistoryReceived: Sept 19 2023Revision requested: Nov 9 2023Revision received: Nov 22 2023Accepted: Dec 8 2023Published online: Mar 12 2024 FiguresReferencesRelatedDetailsRecommended Articles Major Aortopulmonary Collateral ArteriesRadiology: Cardiothoracic Imaging2022Volume: 4Issue: 1Approach to Pulmonary Hypertension: From CT to Clinical DiagnosisRadioGraphics2018Volume: 38Issue: 2pp. 357-373Perfusion Scintigraphy in Diagnosis and Management of Thromboembolic Pulmonary HypertensionRadioGraphics2019Volume: 39Issue: 1pp. 169-185Multidisciplinary Approach to Chronic Thromboembolic Pulmonary Hypertension: Role of RadiologistsRadioGraphics2022Volume: 43Issue: 2Imaging of Pulmonary Hypertension in Adults: A Position Paper from the Fleischner SocietyRadiology2021Volume: 298Issue: 3pp. 531-549See More RSNA Education Exhibits Pearls and Pitfalls in Pulmonary Vascular DiseaseDigital Posters2022Chronic Pulmonary Embolism: What Radiologists Should KnowDigital Posters2022Chronic Thromboembolic Pulmonary Hypertension (CTEPH): A Multimodality Imaging ReviewDigital Posters2022 RSNA Case Collection Interrupted proximal pulmonary ArteryRSNA Case Collection2021Pulmonary artery intimal sarcomaRSNA Case Collection2021Pulmonary embolism with right heart strain RSNA Case Collection2020 Vol. 310, No. 3 Metrics Altmetric Score PDF download