Congenital central pulmonary artery anomalies: Part 1

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Congenital central pulmonary artery anomalies: Part 1 Beverley Newman 1

&

Noor Alkhori 2

Received: 27 December 2019 / Revised: 21 March 2020 / Accepted: 22 April 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. The authors summarize and illustrate well-recognized as well as more complex anomalies to provide a broad and comprehensive understanding of these lesions and their appearances on CT and MR imaging. Part 1 covers anomalous development or origin of the main pulmonary artery. Keywords Anomalies . Children . Computed tomography . Congenital . Magnetic resonance imaging . Pulmonary artery

Introduction A large number and variety of congenital abnormalities affect the central pulmonary arteries (PAs). These anomalies can be classified into several major categories, which is helpful for the purposes of recognition and organization, although there is considerable overlap among categories. Pulmonary artery abnormalities within the lung such as pulmonary arteriovenous malformation and pulmonary sequestration are not included in this discussion except when mentioned as associated anomalies. Other associated cardiothoracic abnormalities are also outlined but not discussed in detail. Abnormal vessel origin from a pulmonary artery, such as coronary or isolated subclavian artery, is also beyond the scope of this review. Computed tomography (CT) angiography and magnetic resonance (MR) angiography have become the primary imaging modalities for evaluating many cardiovascular anomalies CME activity This article has been selected as the CME activity for the current month. Please visit the SPR website at www.pedrad.org on the Education page and follow the instructions to complete this CME activity. * Beverley Newman [email protected] 1

Department of Radiology, Stanford Children’s Hospital, 725 Welch Road, Stanford, CA 94304, USA

2

Department of Clinical Radiology, Sidra Medicine, Weill Cornell Medicine, Doha, Qatar

including those affecting the PAs [1–3]. CT currently provides optimal detail of the airways, lung parenchyma and small vessels and has the advantages of speed and ease of access. MR can provide superior function and flow information and does not entail exposure to ionizing radiation. Catheter angiography might be required when detailed branch and collateral PA mapping is needed, pressure and oxygenation data are required, or for transcatheter interventions [4]. The bronchial circulation supplies nutrients to the lung and usually is not involved in gas exchange. However, when there is pulmonary artery obs