Evidence-Based Approach to Imaging of Congenital Heart Disease

  • Rajesh Krishnamurthy
  • Pranav Chitkara

Key Points

■ Chest radiographs do not function as a screening test for suspected CHD in neonates. If CHD cannot be excluded by clinical examination, echo is the preferred modality (moderate evidence).

■ In patients with CHD, chest radiography has low accuracy for diagnosis of specific cardiac lesions (moderate evidence) but provides ancillary information regarding pulmonary vascularity which is helpful in initial management.

■ Routine daily chest radiographs are useful in the setting of pediatric and neonatal intensive care units and in the immediate post-operative period after repair of CHD (moderate evidence).

■ MRI should be considered the gold standard for evaluation of RV size and function in the setting of CHD (strong evidence). Two-dimensional echo measurements of RV size and mass correlate poorly with MRI parameters (moderate evidence). Three- and four-dimensional echo hold promise for measurement of RV volume and mass in the setting of CHD, but further study is required (limited evidence).

■ MRI parameters (RV end-diastolic volume, RV end-systolic volume, and biventricular ejection fraction) and EKG parameters (QRS duration on the resting EKG of >180 msec) are the best predictors of adverse clinical outcome in patients with treated tetralogy of Fallot (moderate evidence). The optimal timing of pulmonary valve replacement for patients with corrected TOF is undetermined but is influenced by MRI parameters of RV size and function (indexed RV volume and RV ejection fraction).

■ MRI can replace cardiac catheterization for routine evaluation of cardiovascular morphology and function prior to superior cavopulmonary connection in the majority of patients undergoing single-ventricle repair (moderate evidence).

■ The role of multidetector CT is mainly as a problem-solving tool when MRI is contraindicated, limited, or not available (limited evidence). MDCT is accurate for diagnosing coronary artery anomalies, coarctation, and post-operative complications, but concerns regarding high radiation exposure has limited its use.


Right Ventricular Congenital Heart Disease Right Ventricular Outflow Tract Right Ventricular Ejection Fraction Pulmonary Valve Replacement 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Circulation 2007;115(2):163–172.CrossRefPubMedGoogle Scholar
  2. 2.
    Ungerleider RM, Bengur AR, Kessenich AL, Liekweg RJ, Hart EM et al. Ann Thorac Surg 1997; 64(1):44–48; discussion 49.CrossRefPubMedGoogle Scholar
  3. 3.
    Connor JA, Gauvreau K, Jenkins KJ. Pediatrics 2005; 116(3):689–695.CrossRefPubMedGoogle Scholar
  4. 4.
    Birkebaek NH, Hansen LK, Elle B, Andersen PE, Friis M et al. Pediatrics 1999; 103(2):E15.CrossRefPubMedGoogle Scholar
  5. 5.
    Fonseca B, Chang RK, Senac M, Knight G, Sklansky MS. Pediatr Cardiol 2005; 26:367–372, .CrossRefPubMedGoogle Scholar
  6. 6.
    Laya BF, Goske MJ, Morrison S, Reid JR, Swischuk L et al. Pediatr Radiol 2006; 36: 677–681.CrossRefPubMedGoogle Scholar
  7. 7.
    Oeppen RS, Fairhurst JJ, Argent JD. Clinical Radiol 2002; 57:736–740.CrossRefGoogle Scholar
  8. 8.
    Pedrosa de Azevedo AC, Osibote AO, Bastos Boechat MC. Appl Radiat Isot 2006; 64(12):1637.CrossRefPubMedGoogle Scholar
  9. 9.
    Graat ME, Stoker J, Vroom MB, Schultz MJ. J Intensive Care Med 2005; 20(4):238–246.CrossRefPubMedGoogle Scholar
  10. 10.
    Graat ME, Hendrikse KA, Spronk PE, Korevaar JC, Stoker J et al. BMC Med Imaging 2006; 18:Hornick PI, Harris P, Cousins C, Taylor KM, Keogh BE. 6–8.Google Scholar
  11. 11.
    Hornick PI, Harris P, Cousins C, Taylor KM, Keogh BE. Ann Thorac Surg 1995; 59:1150–1154.Google Scholar
  12. 12.
    Mets O, Spronk PE, Binnekade J, Stoker J, de Mol BA et al. J Thorac Cardiovasc Surg 2007; 134(1):139–144.CrossRefPubMedGoogle Scholar
  13. 13.
    Krivopal M, Shlobin OA, Schwartzstein RM. Chest 2003; 123(5):1607–1614.CrossRefPubMedGoogle Scholar
  14. 14.
    Hall JB, White SR, Karrison T. Crit Care Med 1991; 19(5):689–693.CrossRefPubMedGoogle Scholar
  15. 15.
    Marik PE, Janower ML. Am J Crit Care 1997; 6(2):95–98.PubMedGoogle Scholar
  16. 16.
    Hauser GJ, Pollack MM, Sivit CJ, Taylor GA, Bulas DI et al. Pediatrics 1989 Apr; 83(4):465–470. Erratum in: Pediatrics 1989; 84(1):17.PubMedGoogle Scholar
  17. 17.
    Plötz FB, Valk JW. Pediatrics 2002; 110(2 Pt 1):421; author reply 421.CrossRefPubMedGoogle Scholar
  18. 18.
    Valk JW, Plötz FB, Schuerman FA, van Vught H, Kramer PP et al. Pediatr Radiol 2001; 31(5):343–347.CrossRefPubMedGoogle Scholar
  19. 19.
    Grothues F, Moon JC, Bellenger NG, Smith GS, Klein HU et al. Am Heart J 2004; 147:218–223.CrossRefPubMedGoogle Scholar
  20. 20.
    Mooij CF, de Wit CJ, Graham DA, Powell AJ, Geva T. J Magn Reson Imaging 2008; 28(1):67–73.CrossRefPubMedGoogle Scholar
  21. 21.
    Helbing WA, Rebergen SA, Maliepaard C et al. Am Heart J 1995; 130:828–837.CrossRefPubMedGoogle Scholar
  22. 22.
    Beygui F, Furber A, Delépine S, Helft G, Metzger JP et al. Int J Cardiovasc Imaging 2004; 20(6):509–516.CrossRefPubMedGoogle Scholar
  23. 23.
    Schwerzmann M, Samman AM, Salehian O, Holm J, Provost Y et al. Am J Cardiol 2007; 99(11):1593–1597.CrossRefPubMedGoogle Scholar
  24. 24.
    Lai WW, Gauvreau K, Rivera ES, Saleeb S, Powell AJ et al. Int J Cardiovasc Imaging 2008; 24(7):691–698.CrossRefPubMedGoogle Scholar
  25. 25.
    Grison A, Maschietto N, Reffo E, Stellin G, Padalino M et al. J Am Soc Echocardiogr 2007; 20(8):921–929.CrossRefPubMedGoogle Scholar
  26. 26.
    Sheehan FH, Bolson EL. Catheter Cardiovasc Interv 2004; 62(1):46–51.CrossRefPubMedGoogle Scholar
  27. 27.
    Heusch A, Lawrenz W, Olivier M, Schmidt KG. Cardiol Young 2006; 16(2):135–140.CrossRefPubMedGoogle Scholar
  28. 28.
    Hubka M, Mantei K, Bolson E, Coady K, Sheehan F. Comput Cardiol 2000; 27:703–706.PubMedGoogle Scholar
  29. 29.
    Niemann PS, Pinho L, Balbach T, Galuschky C, Blankenhagen M et al. J Am Coll Cardiol 2007; 50(17):1668–1676.CrossRefPubMedGoogle Scholar
  30. 30.
    Gatzoulis MA, Balaji S, Webber SA, Siu SC, Poile C et al. Lancet 2000; 356:975–981.CrossRefPubMedGoogle Scholar
  31. 31.
    Geva T. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2006:9; 11–22.CrossRefGoogle Scholar
  32. 32.
    Therrien J, Webb GD, Siu S. J Am Coll Cardiol 2000; 36:1670–1675.CrossRefPubMedGoogle Scholar
  33. 33.
    Mulder BJM, de Winter RJ, Wilde AAM. Neth Heart J 2007; 15(1):3–4.PubMedGoogle Scholar
  34. 34.
    Samyn MM, Powell AJ, Garg R, Sena L, Geva T. J Magn Reson Imaging 2007; 26(4):934–940.CrossRefPubMedGoogle Scholar
  35. 35.
    van den Berg J, Wielopolski PA, Meijboom FJ, Witsenburg M, Bogers AJ et al. Radiology 2007; 243(1):212–219.CrossRefPubMedGoogle Scholar
  36. 36.
    Ghez O, Tsang VT, Frigiola A, Coats L, Taylor A et al. Eur J Cardiothorac Surg 2007; 31(4):654–658.CrossRefPubMedGoogle Scholar
  37. 37.
    Knauth AL, Gauvreau K, Powell AJ, Landzberg MJ, Walsh EP et al. Heart 2008; 94:211–216.CrossRefPubMedGoogle Scholar
  38. 38.
    Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ. J Am Coll Cardiol 2004; 43:1068–1074.CrossRefPubMedGoogle Scholar
  39. 39.
    Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM et al. J Am Coll Cardiol 2002; 40:2044–2052.CrossRefPubMedGoogle Scholar
  40. 40.
    Therrien J, Provost Y, Merchant N, Williams W, Colman J et al. Am J Cardiol 2005; 95(6):779–782.CrossRefPubMedGoogle Scholar
  41. 41.
    Henkens IR, van Straten A, Schalij MJ. Ann Thorac Surg 2007; 83:907–911.CrossRefPubMedGoogle Scholar
  42. 42.
    Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk AP et al. Circulation 2007; 116:545–551.CrossRefPubMedGoogle Scholar
  43. 43.
    Nakanishi T. Pediatr Cardiol 2005; 26(2):159–161.CrossRefPubMedGoogle Scholar
  44. 44.
    Fogel MA. Pediatr Cardiol 2005; 26(2):154–158.CrossRefPubMedGoogle Scholar
  45. 45.
    Ro PS, Rychik J, Cohen MS, Mahle WT, Rome JJ. J Am Coll Cardiol 2004; 44(1):184–187.CrossRefPubMedGoogle Scholar
  46. 46.
    Brown DW, Gauvreau K, Powell AJ, Lang P, Colan SD et al. Circulation 2007; 116(23):2718–2725.CrossRefPubMedGoogle Scholar
  47. 47.
    Muthurangu V, Taylor AM, Hegde SR, Johnson R, Tulloh R et al. Circulation 2005; 112:3256–3263.CrossRefPubMedGoogle Scholar
  48. 48.
    Festa P, Ait Ali L, Bernabei M, De Marchi D. Cardiol Young 2005; 15(Suppl 3):51–56.CrossRefPubMedGoogle Scholar
  49. 49.
    Freed MD, Nadas AS, Norwood WI, Castaneda AR. J Am Coll Cardiol 1984; 4:333–336.CrossRefPubMedGoogle Scholar
  50. 50.
    Lipshultz SE, Sanders SP, Mayer JE, Colan SD, Lock JE. J Am Coll Cardiol 1988; 11:373–378.CrossRefPubMedGoogle Scholar
  51. 51.
    Pfammatter JP, Berdat PA, Carrel TP, Stocker FP. Ann Thorac Surg 1999; 68:532–536.CrossRefPubMedGoogle Scholar
  52. 52.
    Prakash A, Powell AJ, Krishnamurthy R, Geva T. Am J Cardiol 2004; 93:657–661.CrossRefPubMedGoogle Scholar
  53. 53.
    Achenbach S, Daniel WG. Herz 2007 Mar; 32(2):97–107.CrossRefPubMedGoogle Scholar
  54. 54.
    Gilkeson RC, Ciancibello L, Zahka K. AJR2003; 180(4):973–980.PubMedGoogle Scholar
  55. 55.
    Sena L, Krishnamurthy R, Chung T. In Lucaya J, Strife JL (eds.). Pediatric Chest Imaging, 2nd ed. Berlin: Springer Publishers, 2007.Google Scholar
  56. 56.
    Beier UH, Jelnin V, Jain S, Ruiz CE. Catheter Cardiovasc Interv 2006; 68(3):441–449.CrossRefPubMedGoogle Scholar
  57. 57.
    Hu XH, Huang GY, Pa M, Li X, Wu L et al. Pediatr Cardiol 2008; 29(4):726–731.CrossRefPubMedGoogle Scholar
  58. 58.
    Ropers D, Moshage W, Daniel WG, Jessl J, Gottwik M et al. Am J Cardiol 2001; 87:193–197.CrossRefPubMedGoogle Scholar
  59. 59.
    Deibler AR, Kuzo RS, Vohringer M, Page EE, Safford RE et al. Mayo Clin Proc 2004; 79(8):1017–1023.CrossRefPubMedGoogle Scholar
  60. 60.
    Kacmaz F, Ozbulbul NI, Alyan O, Maden O, Demir AD et al. Coron Artery Dis 2008; 19(3):203–209.CrossRefPubMedGoogle Scholar
  61. 61.
    Srinivasan KG, Gaikwad A, Kannan BR, Ritesh K, Ushanandini KP. J Med Imaging Radiat Oncol 2008; 52(2):148–154.CrossRefPubMedGoogle Scholar
  62. 62.
    Dogan OF, Karcaaltincaba M, Yorgancioglu C, Demircin M, Dogan R et al. Heart Surg Forum 2007; 10(1):E90–E94.CrossRefPubMedGoogle Scholar
  63. 63.
    Waite S, Ng T, Afari A, Gohari A, Lowery R. J Thorac Imaging 2008; 23(2):145–147.CrossRefPubMedGoogle Scholar
  64. 64.
    Saremi F, Channual S, Abolhoda A, Gurudevan SV, Narula J et al. Am J Roentgenol 2008; 190(6):1569–1575.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.EB Singleton Department of Diagnostic ImagingTexas Children’s Hospital, Baylor College of MedicineHoustonUSA
  2. 2.Department of Internal MedicineBaylor College of MedicineHoustonUSA

Personalised recommendations