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Congenital Thoracic Vascular Anomalies: Evaluation with State-Of-The-Art MR Imaging and MDCT

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Congenital Thoracic Vascular Anomalies: Evaluation with State-Of-The-Art MR Imaging and MDCT Congenital Thoracic Vascular Anomalies: Evaluation with State-of-the-Art MR Imaging and MDCT Jeffrey C. Hellinger, MDa,*, Melissa Daubert, MDb, Edward Y. Lee, MD, MPHc,d, Monica Epelman, MDe KEYWORDS Aortic arch anomalies Pulmonary artery anomalies Thoracic systemic venous anomalies Pulmonary venous anomalies CT angiography MR imaging MR angiography Congenital thoracic vascular anomalies occur congenital thoracic vascular anomalies in pediatric in the thoracic aorta and branch arteries, pulmo- patients require fundamental understandings of nary arteries, thoracic systemic veins, and the imaging techniques, anatomic embryology and pulmonary veins (Table 1). Technological innova- characteristics, and underlying clinical pathophys- tions in magnetic resonance (MR) imaging and iology. Imagers should have knowledge of strate- multidetector-row computed tomography (MDCT) gies to optimize protocols to deliver accurate have greatly advanced the noninvasive diagnosis and safe cardiovascular imaging based on the of these anomalies in pediatric patients in recent suspected lesion(s) and the clinical stability of years. From the neonate to the adolescent, high- the patient. Equally important is the ability to resolution two-dimensional (2D) and three-dimen- adeptly use advanced postprocessing visualiza- sional (3D) MR imaging (MRI), noncontrast MR tion techniques for image display, interpretation, angiography (MRA), 3D contrast-enhanced MRA, and clinical management. This article assists the and 3D MDCT angiography (CTA) datasets provide reader in these objectives. Imaging strategies comprehensive multiprojectional, anatomic dis- and MR imaging-MR angiography/CTA tech- plays for interactive interpretation, treatment plan- niques are reviewed, followed by a discussion on ning, and postoperative and postendovascular the commonly encountered thoracic congenital evaluation. vascular anomalies, with emphasis on embry- Effective use and interpretation of MRI-MRA ology, clinical manifestations, and characteristic (Fig. 1) and CTA (Fig. 2) for the evaluation of imaging findings. a Advanced Cardiovascular Imaging, Advanced Imaging and Informatics Laboratory, Stony Brook Long Island Children’s Hospital, Stony Brook University School of Medicine, Stony Brook, NY, USA b Stony Brook University School of Medicine, Stony Brook, NY, USA c Division of Thoracic Imaging, Department of Radiology, Children’s Hospital Boston and Harvard Medical School, Boston, MA, USA d Pulmonary Division, Department of Medicine, Children’s Hospital Boston and Harvard Medical School, Boston, MA, USA e Neonatal Imaging, The Children’s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA * Corresponding author. E-mail address: [email protected] Radiol Clin N Am 49 (2011) 969–996 doi:10.1016/j.rcl.2011.06.013 0033-8389/11/$ – see front matter Ó 2011 Elsevier Inc. All rights reserved. radiologic.theclinics.com 970 Hellinger et al Table 1 Congenital thoracic vascular anomaly Arterial Venous Thoracic Aorta Pulmonary Artery Systemic Veins Pulmonary Veins Obstructive aortic arch lesions Pulmonary sling Persistent LSVC PAPVR Tubular hypoplasia Hypoplasia With an RSVC TAPVR Interruption of the Agenesis Mirror image drainage Type I aortic arch Aortic coarctation Stenosis Retroaortic left BCV Type II Aortic arch anomalies Type III Left aortic arch Mixed Aberrant RSCA Circumflex right DsAo Double aortic arch Right aortic arch Aberrant LSCA/BCA Mirror image branching Circumflex left DsAo Cervical aortic arch Innominate artery compression Abbreviations: BCA, brachiocephalic artery; BCV, brachiocephalic vein; DsAo, descending aorta; LSVC, left superior vena cava; PAPVR, partial anomalous pulmonary venous return; RSCA, right subclavian artery; RSVC, right superior vena cava; TAPVR, total anomalous pulmonary venous return. IMAGING STRATEGIES cyanosis) transthoracic echocardiography (TTE) is usually obtained next. TTE, which requires no Chest radiography, echocardiography, vascular radiation, is readily performed and can assess ultrasound, esophagography, MRI-MRA, CTA, morphology and function (eg, flow dynamics, catheter angiography, or a combination thereof pressure gradients). However, TTE is limited by may be performed for diagnostic evaluation of the acoustic window (inversely related to patient congenital thoracic vascular anomalies. In the age and size), acoustic impedance (air), operator past, catheter angiography was regarded as the skill, and the ability to visualize peripheral vascular standard for angiographic evaluation of these segments (eg, pulmonary arteries, pulmonary disorders but, in the past decade, MR angiography veins, and supra-aortic branch arteries). and CTA have gradually replaced catheter angiog- MR imaging-MR angiography or CTA are indi- raphy for diagnostic purposes. Currently, catheter- cated based on the TTE findings and performance. based angiography is reserved for endovascular State-of-the-art MRI-MRA with parallel imaging interventions and obtaining direct hemodynamic should be considered before CTA in pediatric measurements. patients, because it does not require radiation or In most pediatric patients presenting with a sus- iodinated contrast medium and can evaluate pected congenital thoracic vascular anomaly, vascular hemodynamics as well as vascular a frontal (and preferably also a lateral) chest radio- morphology with high-resolution anatomic detail. graph is the initial imaging modality. The chest MDCT angiography is indicated when MR radiograph is a fast and inexpensive means to ob- imaging-MR angiography is not available, is con- tain initial direct or indirect evidence for a congenital traindicated, is nondiagnostic, or has a high vascular lesion. Although the chest radiograph may pretest probability for being nondiagnostic. CTA not always yield the specific diagnosis, it is useful should be considered in the patient at high risk to exclude other potential causes, guide initial with sedation or anesthesia and when airway, management, and direct selection of subsequent lung parenchyma, and other noncardiovascular imaging for confirmation and characterization. structures require more detailed imaging. If cardiovascular symptoms predominate (eg, If respiratory symptoms predominate (eg, stridor, congestive heart failure, systemic hypoperfusion, exercise intolerance, apnea, cyanosis, recurrent Congenital Thoracic Vascular Anomalies 971 Fig. 1. Innominate artery compression on the trachea. A young child with a history of stridor underwent MR imaging-MR angiography for assessment of a possible vascular ring. (A) Sagittal 3D volume rendered image of the MR angiography shows a left aortic arch with normal 3 vessel branches and slightly horizontal course (arrow) of the innominate artery (IA). (B) Oblique MPR projection from a single-shot fast spin echo dark blood acquisition shows the relationship of the IA (long arrow) to the trachea (short arrows). (C–F) Transverse dark blood images show slightly more than 50% extrinsic compression on the trachea (arrow) by the IA (arrowhead), as the IA courses from left to right. LSCA, left subclavian artery; MPR, multiplanar reconstruction; RSCA, right subclavian artery. upper and lower respiratory infections), MR function, and exclude other congenital cardio- imaging-MR angiography or CTA is performed vascular lesions. In this clinical setting, CTA is ad- following chest radiography per the guidelines dis- vantageous to assess for tracheobronchomalacia, cussed earlier. Echocardiography is indicated after tracheal rings, and extrinsic tracheal compression. a positive MR imaging-MR angiography or CTA Paired inspiration-expiration MDCT angiographic to further evaluate cardiac morphology, assess techniques with controlled ventilation have proved 972 Hellinger et al Fig. 2. Tubular hypoplasia of the aortic arch. A neonate with congenital aortic stenosis underwent MDCT angi- ography to further define aortic arch anatomy. (A–C) 3D volume rendered images show moderate to severe tubular hypoplasia of the aortic arch (A, arrow) with a small patent ductus arteriosus (B, C; arrow). to be a reliable means to diagnose clinically signif- for an intravenous catheter (eg, CEMRA, CTA) icant tracheomalacia and innominate artery and whether sedation or anesthesia is required. compression on the trachea in pediatric patients.1 If gastrointestinal symptoms predominate (eg, MR imaging-MR angiography feeding intolerance, failure to thrive, dysphagia, A standard MRI-MRA protocol for evaluation of aspiration), an esophagram may be obtained pediatric thoracic vascular anomalies includes following chest radiography. MRI-MRA or CTA is electrocardiogram-gated black-blood and bright- performed if the esophagram is positive, based blood sequences in conjunction with an angio- on the guidelines discussed earlier. If symptoms graphic sequence. Black-blood and bright-blood persist following a negative esophagram and there MR imaging sequences provide comprehensive remains a high index of suspicion for a thoracic anatomic detail, in particular vessel course, vascular anomaly, consideration should be given caliber, and arterial branching or venous drainage to either MR imaging-MR angiography or CTA. pattern. Black-blood imaging is also applied to Following a positive MRI-MRA or CTA, if not previ- evaluate the central airway. Techniques for ously obtained, TTE may be indicated to assess for black-blood MR imaging typically include either congenital heart structural abnormalities. single-shot fast
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