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Anomalies of the Systemic and Pulmonary Arteries

  • Arno A. W. Roest
  • Lucia J. M. Kroft
  • Lars Grosse-Wortmann
Chapter
Part of the Medical Radiology book series

Abstract

Congenital abnormalities of the thoracic systemic and pulmonary arteries can occur in isolation or in the setting of a complex cardiovascular malformation. Severe or complex vascular defects are usually diagnosed and corrected in childhood. Infrequently, anomalies of the systemic and pulmonary arteries present during adulthood. After correction of a cardiovascular malformation most children survive into adulthood. In adults, the most common indication for imaging is to evaluate post-treatment sequelae. Understanding the underlying pathology and possible complications that may occur during follow-up is essential for planning imaging studies. Furthermore, knowledge on the different imaging modalities, including their strengths and limitations for assessing anomalies of the systemic and pulmonary arteries, is important.

Supplementary material

174_2017_165_MOESM1_ESM.avi (120.8 mb)
Movie 1 14-Year old male patient with recurrent pulmonary infection. CT angiography showing complete vascular ring: double aortic arch with large right arch and small left arch with an atretic segment (fibrous strand) in connection to the descending aorta. The movie highlights the symmetrical branching of the arch vessels and the small left arch with an atretic segment. See Fig. 11 for the vessel anatomy (AVI 123654 kb)

References

  1. Backer CL, Mavroudis C (2000a) Congenital heart surgery nomenclature and database project: patent ductus arteriosus, coarctation of the aorta, interrupted aortic arch. Ann Thorac Surg 69.4(Suppl):S298–S307Google Scholar
  2. Backer CL, Mavroudis C (2000b) Congenital heart surgery nomenclature and database project: vascular rings, tracheal stenosis, pectus excavatum. Ann Thorac Surg 69.4(Suppl):S308–S318Google Scholar
  3. Backer CL et al (2005) Trends in vascular ring surgery. J Thorac Cardiovasc Surg 129(6):1339–1347Google Scholar
  4. Barnes ME, Mitchell ME, Tweddell JS (2011) Aortopulmonary window. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 14(1):67–74Google Scholar
  5. Bartelings MM, Gittenberger-de Groot AC (1989) The outflow tract of the heart—embryologic and morphologic correlations. Int J Cardiol 22(3):289–300Google Scholar
  6. Baumgartner H et al (2010) ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 31(23):2915–2957Google Scholar
  7. Berdon WE (2000) Rings, slings, and other things: vascular compression of the infant trachea updated from the midcentury to the millennium—the legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD. Radiology 216(3):624–632Google Scholar
  8. Bjornard K et al (2013) Patterns in the prevalence of congenital heart defects, metropolitan Atlanta, 1978 to 2005. Birth Defects Res A Clin Mol Teratol 97(2):87–94Google Scholar
  9. Bobylev D et al (2014) Aortopulmonary window: a rare untreated adult case. Heart Lung Circ 23(10):e235–e236Google Scholar
  10. Brown ML et al (2010) Late outcomes of reintervention on the descending aorta after repair of aortic coarctation. Circulation 122(11 Suppl):S81–S84Google Scholar
  11. Browne LP (2009) What is the optimal imaging for vascular rings and slings? Pediatr Radiol 39(Suppl 2):S191–S195Google Scholar
  12. Cartin-Ceba R, Swanson KL, Krowka MJ (2013) Pulmonary arteriovenous malformations. Chest 144(3):1033–1044Google Scholar
  13. Castaner E et al (2006) Congenital and acquired pulmonary artery anomalies in the adult: radiologic overview. Radiographics 26(2):349–371Google Scholar
  14. Chattranukulchai P et al (2013) Undetected large aortopulmonary window in an adult: a confluence of great vessels. J Am Coll Cardiol 62(19):e439Google Scholar
  15. Chen PC et al (2013) Predictors of reintervention after repair of interrupted aortic arch with ventricular septal defect. Ann Thorac Surg 96(2):621–628Google Scholar
  16. Cramer JW et al (2013) Aortic aneurysms remain a significant source of morbidity and mortality after use of Dacron((R)) patch aortoplasty to repair coarctation of the aorta: results from a single center. Pediatr Cardiol 34(2):296–301Google Scholar
  17. Dillman JR et al (2008) Interrupted aortic arch: spectrum of MRI findings. Am J Roentgenol 190(6):1467–1474Google Scholar
  18. Dillman JR et al (2011) Common and uncommon vascular rings and slings: a multi-modality review. Pediatr Radiol 41(11):1440–1454Google Scholar
  19. Edwards JE (1948) Anomalies of the derivatives of the aortic arch system. Med Clin North Am 32:925–949Google Scholar
  20. Etesami M et al (2014) Computed tomography in the evaluation of vascular rings and slings. Insights Imaging 5(4):507–521Google Scholar
  21. Ferguson EC, Krishnamurthy R, Oldham SA (2007) Classic imaging signs of congenital cardiovascular abnormalities. Radiographics 27(5):1323–1334Google Scholar
  22. Forbes TJ et al (2011) Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (congenital cardiovascular interventional study consortium). J Am Coll Cardiol 58(25):2664–2674Google Scholar
  23. Fratz S et al (2013) Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease. J Cardiovasc Magn Reson 15:51Google Scholar
  24. Gill SS et al (2015) Pulmonary arteriovenous malformations and their mimics. Clin Radiol 70(1):96–110Google Scholar
  25. Gittenberger-de Groot AC, Azhar M, Molin DG (2006) Transforming growth factor beta-SMAD2 signaling and aortic arch development. Trends Cardiovasc Med 16(1):1–6Google Scholar
  26. Gould SW et al (2015) Useful signs for the assessment of vascular rings on cross-sectional imaging. Pediatr Radiol 45(13):2004–2016Google Scholar
  27. Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39(12):1890–1900Google Scholar
  28. Hoffman JL et al (2014) Screening for aortic aneurysm after treatment of coarctation. Pediatr Cardiol 35(1):47–52Google Scholar
  29. Hope MD et al (2010a) Bicuspid aortic valve: four-dimensional MR evaluation of ascending aortic systolic flow patterns. Radiology 255(1):53–61Google Scholar
  30. Hope MD et al (2010b) Clinical evaluation of aortic coarctation with 4D flow MR imaging. J Magn Reson Imaging 31(3):711–718Google Scholar
  31. Hope MD et al (2011) 4D flow CMR in assessment of valve-related ascending aortic disease. JACC Cardiovasc Imaging 4(7):781–787Google Scholar
  32. Jacobs JP et al (2000) Congenital heart surgery nomenclature and database project: aortopulmonary window. Ann Thorac Surg 69.4(Suppl):S44–S49Google Scholar
  33. Jimenez-Juan L et al (2014) Cardiovascular magnetic resonance imaging predictors of pregnancy outcomes in women with coarctation of the aorta. Eur Heart J Cardiovasc Imaging 15(3):299–306Google Scholar
  34. Kamphuis VP et al (2017) Unravelling cardiovascular disease using four dimensional flow cardiovascular magnetic resonance. Int J Cardiovasc Imaging (7):1069–81. doi: 10.1007/s10554-016-1031-9. Epub 2016 Nov 25CrossRefGoogle Scholar
  35. Karaosmanoglu AD et al (2015) CT and MRI of aortic coarctation: pre- and postsurgical findings. Am J Roentgenol 204(3):W224–W233Google Scholar
  36. Kimura-Hayama ET et al (2010) Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics 30(1):79–98Google Scholar
  37. Kir M et al (2012) Vascular rings: presentation, imaging strategies, treatment, and outcome. Pediatr Cardiol 33(4):607–617Google Scholar
  38. Kiran VS et al (2008) Lessons learned from a series of patients with missed aortopulmonary windows. Cardiol Young 18(5):480–484Google Scholar
  39. Knapper JT et al (2014) Interrupted aortic arch in an active, asymptomatic adult. Eur Heart J Cardiovasc Imaging 15(10):1185Google Scholar
  40. Kroft LJ, Roelofs JJ, Geleijns J (2010) Scan time and patient dose for thoracic imaging in neonates and small children using axial volumetric 320-detector row CT compared to helical 64-, 32-, and 16- detector row CT acquisitions. PediatrRadiol 40(3):294–300Google Scholar
  41. Leonardi B et al (2015) Imaging modalities in children with vascular ring and pulmonary artery sling. Pediatr Pulmonol 50(8):781–788Google Scholar
  42. Levitt B, Richter JE (2007) Dysphagia lusoria: a comprehensive review. Dis Esophagus 20(6):455–460Google Scholar
  43. Mahle WT, Kreeger J, Silverman NH (2010) Echocardiography of the aortopulmonary window, aorto-ventricular tunnels, and aneurysm of the sinuses of Valsalva. Cardiol Young 20(Suppl 3):100–106Google Scholar
  44. Maki DD et al (2001) Pulmonary arteriovenous malformations: three-dimensional gadolinium-enhanced MR angiography-initial experience. Radiology 219(1):243–246Google Scholar
  45. Marianeschi SM, McElhinney DB, Reddy VM (1998) Pulmonary arteriovenous malformations in and out of the setting of congenital heart disease. Ann Thorac Surg 66(2):688–691Google Scholar
  46. McElhinney DB et al (1998) Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age. Am J Cardiol 81(2):195–201Google Scholar
  47. Meadows J et al (2015) Intermediate outcomes in the prospective, multicenter coarctation of the aorta stent trial (COAST). Circulation 131(19):1656–1664Google Scholar
  48. Monge MC et al (2013) Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes. J Thorac Cardiovasc Surg 145(2):476–481Google Scholar
  49. Morgan CT et al (2017) Understanding the mechanism for branch pulmonary artery stenosis after the arterial switch operation for transposition of the great arteries. Eur Heart J Cardiovasc Imaging 18(2):180–185Google Scholar
  50. Muzzarelli S et al (2011) Prediction of hemodynamic severity of coarctation by magnetic resonance imaging. Am J Cardiol 108(9):1335–1340Google Scholar
  51. Naimo PS et al (2014) Outcomes of aortopulmonary window repair in children: 33 years of experience. Ann Thorac Surg 98(5):1674–1679Google Scholar
  52. Nakayama M et al (2012) Prevalence of pulmonary arteriovenous malformations as estimated by low-dose thoracic CT screening. Intern Med 51(13):1677–1681Google Scholar
  53. Nance JW, Ringel RE, Fishman EK (2016) Coarctation of the aorta in adolescents and adults: a review of clinical features and CT imaging. J Cardiovasc Comput Tomogr 10(1):1–12Google Scholar
  54. Nihoyannopoulos P et al (1987) Accuracy of two-dimensional echocardiography in the diagnosis of aortic arch obstruction. J Am Coll Cardiol 10(5):1072–1077Google Scholar
  55. Ohno Y et al (2002) Contrast-enhanced MR perfusion imaging and MR angiography: utility for management of pulmonary arteriovenous malformations for embolotherapy. Eur J Radiol 41(2):136–146Google Scholar
  56. Pierpont ME et al (2007) Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation 115(23):3015–3038Google Scholar
  57. Rengier F et al (2015) Noninvasive 4D pressure difference mapping derived from 4D flow MRI in patients with repaired aortic coarctation: comparison with young healthy volunteers. Int J Card Imaging 31(4):823–830Google Scholar
  58. Rider OJ, Bissell M, Myerson SG (2013) Congenital aortopulmonary window; an unusual cause of breathlessness. Heart 99(20):1546Google Scholar
  59. Roos-Hesselink JW et al (2003) Aortic valve and aortic arch pathology after coarctation repair. Heart 89(9):1074–1077Google Scholar
  60. Ruzmetov M et al (2009) Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience. J Pediatr Surg 44(7):1328–1332Google Scholar
  61. Schneider G et al (2008) MR angiography for detection of pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. AJR Am J Roentgenol 190(4):892–901Google Scholar
  62. Shepherd B et al (2015) MRI in adult patients with aortic coarctation: diagnosis and follow-up. Clin Radiol 70(4):433–445Google Scholar
  63. Shovlin CL (2014) Pulmonary arteriovenous malformations. Am J Respir Crit Care Med 190(11):1217–1228Google Scholar
  64. Slater BJ, Rothenberg SS (2016) Thoracoscopic management of patent ductus arteriosus and vascular rings in infants and children. J Laparoendosc Adv Surg Tech A 26(1):66–69Google Scholar
  65. Smith BM et al (2015) Rings and slings revisited. Magn Reson Imaging Clin N Am 23(1):127–135Google Scholar
  66. Sridharan S et al (2006) Assessment of differential branch pulmonary blood flow: a comparative study of phase contrast magnetic resonance imaging and radionuclide lung perfusion imaging. Heart 92(7):963–968Google Scholar
  67. Steffens JC et al (1994) Quantification of collateral blood flow in coarctation of the aorta by velocity encoded cine magnetic resonance imaging. Circulation 90(2):937–943Google Scholar
  68. Suh YJ et al (2012) Clinical course of vascular rings and risk factors associated with mortality. Korean Circ J 42(4):252–258Google Scholar
  69. Therrien J et al (2000) Repaired coarctation: a “cost-effective” approach to identify complications in adults. J Am Coll Cardiol 35(4):997–1002Google Scholar
  70. Tonelli AR et al (2015) Peripheral pulmonary artery stenosis as a cause of pulmonary hypertension in adults. Pulm Circ 5(1):204–210Google Scholar
  71. Torok RD et al (2015) Coarctation of the aorta: management from infancy to adulthood. World J Cardiol 7(11):765–775Google Scholar
  72. Tsai SF et al (2011) Usefulness of screening cardiovascular magnetic resonance imaging to detect aortic abnormalities after repair of coarctation of the aorta. Am J Cardiol 107(2):297–301Google Scholar
  73. Tworetzky W et al (1999) Echocardiographic diagnosis alone for the complete repair of major congenital heart defects. J Am Coll Cardiol 33(1):228–233Google Scholar
  74. Ungerleider RM et al (2013) Contemporary patterns of surgery and outcomes for aortic coarctation: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg 145(1):150–157Google Scholar
  75. Valsangiacomo Buechel ER et al (2015) Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI. Eur Heart J Cardiovasc Imaging 16(3):281–297Google Scholar
  76. van Gameren M et al (2006) Early complications of stenting in patients with congenital heart disease: a multicentre study. Eur Heart J 27(22):2709–2715Google Scholar
  77. Warnes CA et al (2008) ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association task force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 118(23):e714–e833Google Scholar
  78. Wong J et al (2012) Analysis of aortopulmonary window using cardiac magnetic resonance imaging. Circulation 126(15):e228–e229Google Scholar
  79. Xu J et al (2014) Accuracy, image quality, and radiation dose of prospectively ECG-triggered high-pitch dual-source CT angiography in infants and children with complex coarctation of the aorta. Acad Radiol 21(10):1248–1254Google Scholar
  80. Yang DH et al (2008) Multislice CT angiography of interrupted aortic arch. PediatrRadiol 38(1):89–100Google Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  • Arno A. W. Roest
    • 1
  • Lucia J. M. Kroft
    • 2
  • Lars Grosse-Wortmann
    • 3
  1. 1.Department of Paediatrics, Willem Alexander Children’s HospitalLeiden University Medical CenterLeidenThe Netherlands
  2. 2.Section Head of Cardiothoracic Radiology, Department of RadiologyLeiden University Medical CenterLeidenThe Netherlands
  3. 3.Section Head, Cardiovascular Magnetic Resonance, The Hospital for Sick ChildrenUniversity of TorontoTorontoCanada

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