Skip to main content
SpringerLink
Log in

CT Spectrum of Congenital Heart Disease

  • Chapter
  • First Online:
CT of the Heart

Part of the book series: Contemporary Medical Imaging ((CMI))

  • 2767 Accesses

Abstract

The following chapter is a brief overview of congenital heart disease, with a specific focus on the aspects that are relevant to cardiac computed tomography (CCT). A brief rationale for the use of CCT in congenital heart disease is given, followed by a review of sequential segmental analysis, the basis of the morphologic assessment of the heart. The anatomical findings for the most common forms of congenital heart disease are described, as well as the expected physiology and clinical course. The relevant CT findings for each heart defect are illustrated and correlated to the anatomy to aid the imaging specialist in comprehension.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Gatzoulis MA, Webb GD. Adults with congenital heart diseases: a growing population. Edinburgh: Churchill Livingstone; 2003.

    Google Scholar 

  2. Kaemmerer H, Stern H, Fratz S, Prokop M, Schwaiger M, Hess J. Imaging in adults with congenital cardiac disease (ACCD). Thorac Cardiovasc Surg. 2000;48(6):328–35. https://doi.org/10.1055/s-2000-8344.

    Article  CAS  PubMed  Google Scholar 

  3. Boxt LM. Magnetic resonance and computed tomographic evaluation of congenital heart disease. J Magn Reson Imaging. 2004;19(6):827–47. https://doi.org/10.1002/jmri.20077.

    Article  PubMed  Google Scholar 

  4. de Roos A, Roest AA. Evaluation of congenital heart disease by magnetic resonance imaging. Eur Radiol. 2000;10(1):2–6. https://doi.org/10.1007/s003300050002.

    Article  PubMed  Google Scholar 

  5. Gutierrez FR, Siegel MJ, Fallah JH, Poustchi-Amin M. Magnetic resonance imaging of cyanotic and noncyanotic congenital heart disease. Magn Reson Imaging Clin N Am. 2002;10(2):209–35.

    Article  PubMed  Google Scholar 

  6. Haramati LB, Glickstein JS, Issenberg HJ, Haramati N, Crooke GA. MR imaging and CT of vascular anomalies and connections in patients with congenital heart disease: significance in surgical planning. Radiographics. 2002;22(2):337–47; discussion 348–339. https://doi.org/10.1148/radiographics.22.2.g02mr09337.

    Article  PubMed  Google Scholar 

  7. Kulkarni A, Hsu HH, Ou P, Kutty S. Computed tomography in congenital heart disease: clinical applications and technical considerations. Echocardiography. 2016;33(4):629–40. https://doi.org/10.1111/echo.13147.

    Article  PubMed  Google Scholar 

  8. Han BK, Rigsby CK, Hlavacek A, Leipsic J, Nicol ED, Siegel MJ, Bardo D, Abbara S, Ghoshhajra B, Lesser JR, Raman S, Crean AM. Computed tomography imaging in patients with congenital heart disease part I: rationale and utility. An expert consensus document of the Society of Cardiovascular Computed Tomography (SCCT): endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr. 2015;9(6):475–92. https://doi.org/10.1016/j.jcct.2015.07.004.

    Article  PubMed  Google Scholar 

  9. Han BK, Lesser AM, Vezmar M, Rosenthal K, Rutten-Ramos S, Lindberg J, Caye D, Lesser JR. Cardiovascular imaging trends in congenital heart disease: a single center experience. J Cardiovasc Comput Tomogr. 2013;7(6):361–6. https://doi.org/10.1016/j.jcct.2013.11.002.

    Article  PubMed  Google Scholar 

  10. Goo HW, Park IS, Ko JK, Kim YH, Seo DM, Park JJ. Computed tomography for the diagnosis of congenital heart disease in pediatric and adult patients. Int J Cardiovasc Imaging. 2005;21(2–3):347–65; discussion 367. https://doi.org/10.1007/s10554-004-4015-0.

    Article  PubMed  Google Scholar 

  11. Leschka S, Oechslin E, Husmann L, Desbiolles L, Marincek B, Genoni M, Pretre R, Jenni R, Wildermuth S, Alkadhi H. Pre- and postoperative evaluation of congenital heart disease in children and adults with 64-section CT. Radiographics. 2007;27(3):829–46. https://doi.org/10.1148/rg.273065713.

    Article  PubMed  Google Scholar 

  12. Girshin M, Shapiro V, Rhee A, Ginsberg S, Inchiosa MA Jr. Increased risk of general anesthesia for high-risk patients undergoing magnetic resonance imaging. J Comput Assist Tomogr. 2009;33(2):312–5. https://doi.org/10.1097/RCT.0b013e31818474b8.

    Article  PubMed  Google Scholar 

  13. Wolla CD, Hlavacek AM, Schoepf UJ, Bucher AM, Chowdhury S. Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr. 2013;7(6):408–16. https://doi.org/10.1016/j.jcct.2013.11.008.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Loomba RS, Hlavacek AM, Spicer DE, Anderson RH. Isomerism or heterotaxy: which term leads to better understanding? Cardiol Young. 2015;25(6):1037–43. https://doi.org/10.1017/s1047951115001122.

    Article  PubMed  Google Scholar 

  15. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12):1890–900.

    Article  PubMed  Google Scholar 

  16. Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult, vol. 1. 8th ed. Philadelphia: Lippincott Williams & Wilkins; 2013.

    Google Scholar 

  17. Anderson RH, Spicer DE, Hlavacek AM, Cook AC, Backer CL. Wilcox’s surgical anatomy of the heart. 4th ed. Cambridge, UK: Cambridge University Press; 2013.

    Book  Google Scholar 

  18. Osawa K, Miyoshi T, Morimitsu Y, Akagi T, Oe H, Nakagawa K, Takaya Y, Kijima Y, Sato S, Kanazawa S, Ito H. Comprehensive assessment of morphology and severity of atrial septal defects in adults by CT. J Cardiovasc Comput Tomogr. 2015;9(4):354–61. https://doi.org/10.1016/j.jcct.2015.04.007.

    Article  PubMed  Google Scholar 

  19. Ko SF, Liang CD, Yip HK, Huang CC, Ng SH, Huang CF, Chen MC. Amplatzer septal occluder closure of atrial septal defect: evaluation of transthoracic echocardiography, cardiac CT, and transesophageal echocardiography. AJR Am J Roentgenol. 2009;193(6):1522–9. https://doi.org/10.2214/AJR.09.2854.

    Article  PubMed  Google Scholar 

  20. Lee T, Tsai IC, Fu YC, Jan SL, Wang CC, Chang Y, Chen MC. MDCT evaluation after closure of atrial septal defect with an Amplatzer septal occluder. AJR Am J Roentgenol. 2007;188(5):W431–9. https://doi.org/10.2214/ajr.06.0709.

    Article  PubMed  Google Scholar 

  21. Marini D, Ou P, Boudjemline Y, Kenny D, Bonnet D, Agnoletti G. Midterm results of percutaneous closure of very large atrial septal defects in children: role of multislice computed tomography. EuroIntervention. 2012;7(12):1428–34. https://doi.org/10.4244/eijv7i12a223.

    Article  PubMed  Google Scholar 

  22. Amat F, Le Bret E, Sigal-Cinqualbre A, Coblence M, Lambert V, Rohnean A, Paul JF. Diagnostic accuracy of multidetector spiral computed tomography for preoperative assessment of sinus venosus atrial septal defects in children. Interact Cardiovasc Thorac Surg. 2011;12(2):179–82. https://doi.org/10.1510/icvts.2010.251298.

    Article  PubMed  Google Scholar 

  23. Lembcke A, Razek V, Kivelitz D, Rogalla N, Rogalla P. Sinus venosus atrial septal defect with partial anomalous pulmonary venous return: diagnosis with 64-slice spiral computed tomography at low radiation dose. J Pediatr Surg. 2008;43(2):410–1.

    Article  PubMed  Google Scholar 

  24. Yamasaki Y, Nagao M, Kawanami S, Kamitani T, Sagiyama K, Yamanouchi T, Sakamoto I, Yamamura K, Yabuuchi H, Honda H. One-stop shop assessment for atrial septal defect closure using 256-slice coronary CT angiography. Eur Radiol. 2017;27:697. https://doi.org/10.1007/s00330-016-4407-2.

    Article  PubMed  Google Scholar 

  25. Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998–2005. J Pediatr. 2008;153(6):807–13. https://doi.org/10.1016/j.jpeds.2008.05.059.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect. Ann Thorac Surg. 2000;69(4 Suppl):S25–35.

    Article  CAS  PubMed  Google Scholar 

  27. Rudolph AM, Drorbaugh JE, Auld PA, Rudolph AJ, Nadas AS, Smith CA, Hubbell JP. Studies on the circulation in the neonatal period. The circulation in the respiratory distress syndrome. Pediatrics. 1961;27:551–66.

    CAS  PubMed  Google Scholar 

  28. Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births. Incidence and natural history. Circulation. 1971;43(3):323–32.

    Article  CAS  PubMed  Google Scholar 

  29. Kitterman JA, Edmunds LH Jr, Gregory GA, Heymann MA, Tooley WH, Rudolph AM. Patent ducts arteriosus in premature infants. Incidence, relation to pulmonary disease and management. N Engl J Med. 1972;287(10):473–7. https://doi.org/10.1056/nejm197209072871001.

    Article  CAS  PubMed  Google Scholar 

  30. Schneider DJ, Moore JW. Patent ductus arteriosus. Circulation. 2006;114(17):1873–82. https://doi.org/10.1161/circulationaha.105.592063.

    Article  PubMed  Google Scholar 

  31. Sardesai SH, Marshall RJ, Farrow R, Mourant AJ. Dissecting aneurysm of the pulmonary artery in a case of unoperated patent ductus arteriosus. Eur Heart J. 1990;11(7):670–3.

    Article  CAS  PubMed  Google Scholar 

  32. Lin MT, Wang JK, Chen YS, Lee WJ, Chiu HH, Chen CA, Chiu SN, Wu ET, Lu CW, Huang SC, Chen SJ, Chiu IS, Chang CI, Wu MH. Detection of pulmonary arterial morphology in tetralogy of Fallot with pulmonary atresia by computed tomography: 12 years of experience. Eur J Pediatr. 2012;171(3):579–86. https://doi.org/10.1007/s00431-011-1621-4.

    Article  PubMed  Google Scholar 

  33. Meinel FG, Huda W, Schoepf UJ, Rao AG, Cho YJ, Baker GH, Hlavacek AM. Diagnostic accuracy of CT angiography in infants with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Cardiovasc Comput Tomogr. 2013;7(6):367–75. https://doi.org/10.1016/j.jcct.2013.11.001.

    Article  PubMed  Google Scholar 

  34. Braunwald E, Goldblatt A, Aygen MM, Rockoff SD, Morrow AG. Congenital aortic stenosis. I. Clinical and hemodynamic findings in 100 patients. II. Surgical treatment and the results of operation. Circulation. 1963;27:426–62.

    Article  CAS  PubMed  Google Scholar 

  35. Xu J, Zhao H, Wang X, Bai Y, Liu L, Liu Y, Wei M, Li J, Zheng M. 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. 2014;21(10):1248–54. https://doi.org/10.1016/j.acra.2014.04.019.

    Article  PubMed  Google Scholar 

  36. Budoff MJ, Shittu A, Roy S. Use of cardiovascular computed tomography in the diagnosis and management of coarctation of the aorta. J Thorac Cardiovasc Surg. 2013;146(1):229–32. https://doi.org/10.1016/j.jtcvs.2013.01.024.

    Article  PubMed  Google Scholar 

  37. Backer CL, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: patent ductus arteriosus, coarctation of the aorta, interrupted aortic arch. Ann Thorac Surg. 2000;69(4 Suppl):S298–307.

    Article  CAS  PubMed  Google Scholar 

  38. Braunlin E, Peoples WM, Freedom RM, Fyler DC, Goldblatt A, Edwards JE. Interruption of the aortic arch with aorticopulmonary septal defect. An anatomic review. Pediatr Cardiol. 1982;3(4):329–35. https://doi.org/10.1007/bf02427036.

    Article  CAS  PubMed  Google Scholar 

  39. Yang DH, Goo HW, Seo DM, Yun TJ, Park JJ, Park IS, Ko JK, Kim YH. Multislice CT angiography of interrupted aortic arch. Pediatr Radiol. 2008;38(1):89–100. https://doi.org/10.1007/s00247-007-0662-3.

    Article  PubMed  Google Scholar 

  40. Schreiber C, Cook A, Ho SY, Augustin N, Anderson RH. Morphologic spectrum of Ebstein’s malformation: revisitation relative to surgical repair. J Thorac Cardiovasc Surg. 1999;117(1):148–55.

    Article  CAS  PubMed  Google Scholar 

  41. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein’s anomaly. Circulation. 2007;115(2):277–85. https://doi.org/10.1161/circulationaha.106.619338.

    Article  PubMed  Google Scholar 

  42. Allwork SP, Bentall HH, Becker AE, Cameron H, Gerlis LM, Wilkinson JL, Anderson RH. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol. 1976;38(7):910–23.

    Article  CAS  PubMed  Google Scholar 

  43. Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol. 1965;16(3):406–25.

    Article  PubMed  Google Scholar 

  44. Collett RW, Edwards JE. Persistent truncus arteriosus; a classification according to anatomic types. Surg Clin North Am. 1949;29(4):1245–70.

    Article  CAS  PubMed  Google Scholar 

  45. Konstantinov IE, Karamlou T, Blackstone EH, Mosca RS, Lofland GK, Caldarone CA, Williams WG, Mackie AS, McCrindle BW. Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study. Ann Thorac Surg. 2006;81(1):214–22. https://doi.org/10.1016/j.athoracsur.2005.06.072.

    Article  PubMed  Google Scholar 

  46. Keane JF, Lock JE, Fyler DC. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders; 2006.

    Google Scholar 

  47. Ramos-Duran L, Nance JW Jr, Schoepf UJ, Henzler T, Apfaltrer P, Hlavacek AM. Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol. 2012;198(5):W466–74. https://doi.org/10.2214/AJR.11.6982.

    Article  PubMed  Google Scholar 

  48. Leonardi B, Secinaro A, Cutrera R, Albanese S, Trozzi M, Franceschini A, Silvestri V, Toma P, Carotti A, Pongiglione G. Imaging modalities in children with vascular ring and pulmonary artery sling. Pediatr Pulmonol. 2015;50(8):781–8. https://doi.org/10.1002/ppul.23075.

    Article  PubMed  Google Scholar 

  49. Lee EY, Zurakowski D, Waltz DA, Mason KP, Riaz F, Ralph A, Boiselle PM. MDCT evaluation of the prevalence of tracheomalacia in children with mediastinal aortic vascular anomalies. J Thorac Imaging. 2008;23(4):258–65. https://doi.org/10.1097/RTI.0b013e31817fbdf7.

    Article  PubMed  Google Scholar 

  50. Dyer KT, Hlavacek AM, Meinel FG, De Cecco CN, McQuiston AD, Schoepf UJ, Pietris NP. Imaging in congenital pulmonary vein anomalies: the role of computed tomography. Pediatr Radiol. 2014;44(9):1158–68; quiz 1155–7. https://doi.org/10.1007/s00247-014-3044-7.

    Article  PubMed  Google Scholar 

  51. Bonelli-Sica JM, de la Mora-Cervantes R, Diaz-Zamudio M, Castillo-Castellon F, Ramirez-Carmona R, Velazquez-Moreno J, Kimura-Hayama E. Dual-source 256-MDCT for diagnosis of anomalous pulmonary venous drainage in pediatric population. AJR Am J Roentgenol. 2013;200(2):W163–9. https://doi.org/10.2214/ajr.11.8348.

    Article  PubMed  Google Scholar 

  52. Jonas RA, Smolinsky A, Mayer JE, Castaneda AR. Obstructed pulmonary venous drainage with total anomalous pulmonary venous connection to the coronary sinus. Am J Cardiol. 1987;59(5):431–5.

    Article  CAS  PubMed  Google Scholar 

  53. Krasemann Z, Scheld HH, Tjan TD, Krasemann T. Cor triatriatum: short review of the literature upon ten new cases. Herz. 2007;32(6):506–10. https://doi.org/10.1007/s00059-007-2882-6.

    Article  PubMed  Google Scholar 

  54. Tweddell JS, Hoffman GM, Mussatto KA, Fedderly RT, Berger S, Jaquiss RD, Ghanayem NS, Frisbee SJ, Litwin SB. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation. 2002;106(12 Suppl 1):I82–9.

    PubMed  Google Scholar 

  55. Hawkins JA, Doty DB. Aortic atresia: morphologic characteristics affecting survival and operative palliation. J Thorac Cardiovasc Surg. 1984;88(4):620–6.

    CAS  PubMed  Google Scholar 

  56. Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg. 1993;105(3):406–23, 424–407; discussion 423–404.

    CAS  PubMed  Google Scholar 

  57. Seguela PE, Houyel L, Loget P, Piot JD, Paul JF. Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum. Pediatr Radiol. 2011;41(8):1069–72. https://doi.org/10.1007/s00247-011-2068-5.

    Article  PubMed  Google Scholar 

  58. Tandon R, Edwards JE. Tricuspid atresia. A re-evaluation and classification. J Thorac Cardiovasc Surg. 1974;67(4):530–42.

    CAS  PubMed  Google Scholar 

  59. Sanders JM. Bilateral superior vena cavae. Anat Rec. 1946;94:657–62.

    Article  CAS  PubMed  Google Scholar 

  60. Mayo J, Gray R, St Louis E, Grosman H, McLoughlin M, Wise D. Anomalies of the inferior vena cava. AJR Am J Roentgenol. 1983;140(2):339–45. https://doi.org/10.2214/ajr.140.2.339.

    Article  CAS  PubMed  Google Scholar 

  61. Rubino M, Van Praagh S, Kadoba K, Pessotto R, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg. 1995;110(3):641–50. https://doi.org/10.1016/s0022-5223(95)70095-1.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, SC, USA

    David Steflik & Anthony M. Hlavacek

Authors
  1. David Steflik
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Anthony M. Hlavacek
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Anthony M. Hlavacek .

Editor information

Editors and Affiliations

  1. Department of Radiology, Center for Advanced Imaging Research (CAIR), Medical University of South Carolina, Charleston, SC, USA

    U. Joseph Schoepf

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Humana Press

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Steflik, D., Hlavacek, A.M. (2019). CT Spectrum of Congenital Heart Disease. In: Schoepf, U. (eds) CT of the Heart. Contemporary Medical Imaging. Humana, Totowa, NJ. https://doi.org/10.1007/978-1-60327-237-7_46

Download citation

  • DOI: https://doi.org/10.1007/978-1-60327-237-7_46

  • Published:

  • Publisher Name: Humana, Totowa, NJ

  • Print ISBN: 978-1-60327-236-0

  • Online ISBN: 978-1-60327-237-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation