Skip to main content
Log in

Clinical applications of radionuclide imaging in the evaluation and management of patients with congenital heart disease

  • CME ARTICLE
  • Review Article
  • Published:
Journal of Nuclear Cardiology Aims and scope

Abstract

Non-invasive testing of children with congenital heart disease (CHD) began in the 1950s with the introduction of radionuclide studies to assess shunt fractions, pulmonary blood flow, and ventricular contractile function. Echocardiography and cardiac magnetic resonance imaging have since replaced radionuclide imaging in many of these roles. Concurrently, percutaneous and surgical repairs of complex CHD evolved, creating new roles for radionuclide imaging. In this paper on applications of radionuclide imaging in CHD, we review the multiple mechanisms for myocardial ischemia in CHD. We critically compare optimal radionuclide imaging techniques to other imaging modalities for assessing ischemia in CHD. We present the current role of nuclear imaging for assessing viability and pulmonary blood flow. We highlight the value added by advances in dedicated cardiac SPECT scanners, novel reconstruction software, and cardiac PET in performing low-dose radionuclide imaging in CHD. Finally, we discuss the emerging clinical indications for radionuclide imaging in CHD including coronary flow reserve assessment and evaluation of cardiovascular prosthesis and device infections.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
Figure 7
Figure 8
Figure 9
Figure 10

Similar content being viewed by others

Abbreviations

CHD:

Congenital heart disease

CMR:

Cardiac magnetic resonance

CAD:

Coronary artery disease

TGA:

Transposition of the great arteries

ALCAPA:

Anomalous left coronary artery from the pulmonary artery

RCA:

Right coronary artery

MPI:

Myocardial perfusion imaging

VSD:

Ventricular septal defect

CFR:

Coronary flow reserve

CIED:

Cardiovascular implantable electronic device

References

  1. Sanders RJ, Morrow AG. The identification and quantification of left-to-right circulatory shunts; a new diagnostic method utilizing the inhalation of a radioactive gas, Kr85. Am J Med 1959;26:508-16.

    Article  CAS  PubMed  Google Scholar 

  2. Dollery CT, West JB, Wilcken DE, Hugh-Jones P. A comparison of the pulmonary blood flow between left and right lungs in normal subjects and patients with congenital heart disease. Circulation 1961;24:617-25.

    Article  CAS  PubMed  Google Scholar 

  3. Jones RH, Austin EH, Peter CA, Sabiston DC Jr. Radionuclide angiocardiography in the diagnosis of congenital heart disorders. Ann Surg 1981;193:710-8.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  4. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: Changing prevalence and age distribution. Circulation 2007;115:163-72.

    Article  PubMed  Google Scholar 

  5. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JI, et al. Task force 1: The changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001;37:1170-5.

    Article  CAS  PubMed  Google Scholar 

  6. Moons P, Van Deyk K, Dedroog D, Troost E, Budts W. Prevalence of cardiovascular risk factors in adults with congenital heart disease. Eur J Cardiovasc Prev Rehabil 2006;13:612-6.

    Article  PubMed  Google Scholar 

  7. Giannakoulas G, Dimopoulos K, Engel R, Goktekin O, Kucukdurmaz Z, Vatankulu MA, et al. Burden of coronary artery disease in adults with congenital heart disease and its relation to congenital and traditional heart risk factors. Am J Cardiol 2009;103:1445-50.

    Article  PubMed  Google Scholar 

  8. Stulak JM, Dearani JA, Burkhart HM, Ammash NM, Phillips SD, Schaff HV. Coronary artery disease in adult congenital heart disease: Outcome after coronary artery bypass grafting. Ann Thorac Surg 2012;93:116-22; discussion 22-3.

    Article  PubMed  Google Scholar 

  9. Yalonetsky S, Horlick EM, Osten MD, Benson LN, Oechslin EN, Silversides CK. Clinical characteristics of coronary artery disease in adults with congenital heart defects. Int J Cardiol 2013;164:217-20.

    Article  PubMed  Google Scholar 

  10. Roifman I, Therrien J, Ionescu-Ittu R, Pilote L, Guo L, Kotowycz MA, et al. Coarctation of the aorta and coronary artery disease: Fact or fiction? Circulation 2012;126:16-21.

    Article  PubMed  Google Scholar 

  11. Bonnet D, Bonhoeffer P, Piechaud JF, Aggoun Y, Sidi D, Planche C, et al. Long-term fate of the coronary arteries after the arterial switch operation in newborns with transposition of the great arteries. Heart 1996;76:274-9.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  12. Tanel RE, Wernovsky G, Landzberg MJ, Perry SB, Burke RP. Coronary artery abnormalities detected at cardiac catheterization following the arterial switch operation for transposition of the great arteries. Am J Cardiol 1995;76:153-7.

    Article  CAS  PubMed  Google Scholar 

  13. Hauser M, Bengel FM, Kuhn A, Sauer U, Zylla S, Braun SL, et al. Myocardial blood flow and flow reserve after coronary reimplantation in patients after arterial switch and ross operation. Circulation 2001;103:1875-80.

    Article  CAS  PubMed  Google Scholar 

  14. Legendre A, Losay J, Touchot-Kone A, Serraf A, Belli E, Piot JD, et al. Coronary events after arterial switch operation for transposition of the great arteries. Circulation 2003;108:II186-90.

    Article  PubMed  Google Scholar 

  15. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation 2008;118:2395-451.

    Article  PubMed  Google Scholar 

  16. Eckart RE, Scoville SL, Campbell CL, Shry EA, Stajduhar KC, Potter RN, et al. Sudden death in young adults: A 25-year review of autopsies in military recruits. Ann Intern Med 2004;141:829-34.

    Article  PubMed  Google Scholar 

  17. Perret X, Bonvini RF, Aggoun Y, Verin V. Aberrant right coronary artery occlusion during the percutaneous pulmonary trunk stenting in a patient with tetralogy of Fallot. Heart Vessels 2008;23:140-3.

    Article  PubMed  Google Scholar 

  18. Eicken A, Ewert P, Hager A, Peters B, Fratz S, Kuehne T, et al. Percutaneous pulmonary valve implantation: Two-centre experience with more than 100 patients. Eur Heart J 2011;32:1260-5.

    Article  PubMed  Google Scholar 

  19. McElhinney DB, Hellenbrand WE, Zahn EM, Jones TK, Cheatham JP, Lock JE, et al. Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. Circulation 2010;122:507-16.

    Article  PubMed Central  PubMed  Google Scholar 

  20. Morray BH, McElhinney DB, Cheatham JP, Zahn EM, Berman DP, Sullivan PM, et al. Risk of coronary artery compression among patients referred for transcatheter pulmonary valve implantation: A multicenter experience. Circ Cardiovasc Interv 2013;6:535-42.

    Article  PubMed  Google Scholar 

  21. Hasan BS, McElhinney DB, Brown DW, Cheatham JP, Vincent JA, Hellenbrand WE, et al. Short-term performance of the transcatheter Melody valve in high-pressure hemodynamic environments in the pulmonary and systemic circulations. Circ Cardiovasc Interv 2011;4:615-20.

    Article  PubMed  Google Scholar 

  22. Millane T, Bernard EJ, Jaeggi E, Howman-Giles RB, Uren RF, Cartmill TB, et al. Role of ischemia and infarction in late right ventricular dysfunction after atrial repair of transposition of the great arteries. J Am Coll Cardiol 2000;35:1661-8.

    Article  CAS  PubMed  Google Scholar 

  23. Valente AM, Lock JE, Gauvreau K, Rodriguez-Huertas E, Joyce C, Armsby L, et al. Predictors of long-term adverse outcomes in patients with congenital coronary artery fistulae. Circ Cardiovasc Interv 2010;3:134-9.

    Article  PubMed  Google Scholar 

  24. Giglia TM, Mandell VS, Connor AR, Mayer JE Jr, Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992;86:1516-28.

    Article  CAS  PubMed  Google Scholar 

  25. Partington SL, Valente AM, Bruyere J, Landzberg MJ, Di Carli MF, Grant F, et al. Diagnostic value of Tc99 m SPECT myocardial perfusion imaging in complex congenital heart disease. J Am Coll Cardiol 2014;63:1097.

    Article  Google Scholar 

  26. Partington SL, Lanka V, Hainer J, Blankstein R, Skali H, Forman DE, et al. Safety and feasibility of regadenoson use for suboptimal heart rate response during symptom-limited standard Bruce exercise stress test. J Nucl Cardiol 2012;19:970-8.

    Article  PubMed Central  PubMed  Google Scholar 

  27. Strigl S, Beroukhim R, Valente AM, Annese D, Harrington JS, Geva T, et al. Feasibility of dobutamine stress cardiovascular magnetic resonance imaging in children. J Magn Reson Imaging 2009;29:313-9.

    Article  PubMed  Google Scholar 

  28. Shaw LJ, Hage FG, Berman DS, Hachamovitch R, Iskandrian A. Prognosis in the era of comparative effectiveness research: Where is nuclear cardiology now and where should it be? J Nucl Cardiol 2012;19:1026-43.

    Article  PubMed  Google Scholar 

  29. Depuey EG, Mahmarian JJ, Miller TD, Einstein AJ, Hansen CL, Holly TA, et al. Patient-centered imaging. J Nucl Cardiol 2012;19:185-215.

    Article  PubMed  Google Scholar 

  30. Einstein AJ, Blankstein R, Andrews H, Fish M, Padgett R, Hayes SW, et al. Comparison of image quality, myocardial perfusion, and left ventricular function between standard imaging and single-injection ultra-low-dose imaging using a high-efficiency SPECT camera: The MILLISIEVERT study. J Nucl Med 2014;55:1430-7.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  31. Dorbala S, Di Carli MF, Delbeke D, Abbara S, DePuey EG, Dilsizian V, et al. SNMMI/ASNC/SCCT guideline for cardiac SPECT/CT and PET/CT 1.0. J Nucl Med 2013;54:1485-507.

    Article  PubMed  Google Scholar 

  32. Kondo C. Myocardial perfusion imaging in pediatric cardiology. Ann Nucl Med 2004;18:551-61.

    Article  PubMed  Google Scholar 

  33. Hayes AM, Baker EJ, Kakadeker A, Parsons JM, Martin RP, Radley-Smith R, et al. Influence of anatomic correction for transposition of the great arteries on myocardial perfusion: Radionuclide imaging with technetium-99 m 2-methoxy isobutyl isonitrile. J Am Coll Cardiol 1994;24:769-77.

    Article  CAS  PubMed  Google Scholar 

  34. Weindling SN, Wernovsky G, Colan SD, Parker JA, Boutin C, Mone SM, et al. Myocardial perfusion, function and exercise tolerance after the arterial switch operation. J Am Coll Cardiol 1994;23:424-33.

    Article  CAS  PubMed  Google Scholar 

  35. Hornung TS, Bernard EJ, Jaeggi ET, Howman-Giles RB, Celermajer DS, Hawker RE. Myocardial perfusion defects and associated systemic ventricular dysfunction in congenitally corrected transposition of the great arteries. Heart 1998;80:322-6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  36. Lubiszewska B, Gosiewska E, Hoffman P, Teresinska A, Rozanski J, Piotrowski W, et al. Myocardial perfusion and function of the systemic right ventricle in patients after atrial switch procedure for complete transposition: Long-term follow-up. J Am Coll Cardiol 2000;36:1365-70.

    Article  CAS  PubMed  Google Scholar 

  37. Chhatriwalla AK, Prieto LR, Brunken RC, Cerqueira MD, Younoszai A, Jaber WA. Preliminary data on the diagnostic accuracy of rubidium-82 cardiac PET perfusion imaging for the evaluation of ischemia in a pediatric population. Pediatr Cardiol 2008;29:732-8.

    Article  PubMed  Google Scholar 

  38. Murthy VL, Naya M, Foster CR, Hainer J, Gaber M, Di Carli G, et al. Improved cardiac risk assessment with noninvasive measures of coronary flow reserve. Circulation 2011;124:2215-24.

    Article  PubMed Central  PubMed  Google Scholar 

  39. Oskarsson G. Coronary flow and flow reserve in children. Acta Paediatr 2004;93:20-5.

    Article  CAS  Google Scholar 

  40. Yasuoka K, Harada K, Tamura M, Takada G. Left anterior descending coronary artery flow and its relation to age in children. J Am Soc Echocardiogr 2002;15:69-75.

    Article  PubMed  Google Scholar 

  41. Hauser M, Bengel FM, Hager A, Kuehn A, Nekolla SG, Kaemmerer H, et al. Impaired myocardial blood flow and coronary flow reserve of the anatomical right systemic ventricle in patients with congenitally corrected transposition of the great arteries. Heart 2003;89:1231-5.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  42. Yates RW, Marsden PK, Badawi RD, Cronin BF, Anderson DR, Tynan MJ, et al. Evaluation of myocardial perfusion using positron emission tomography in infants following a neonatal arterial switch operation. Pediatr Cardiol 2000;21:111-8.

    Article  CAS  PubMed  Google Scholar 

  43. Singh TP, Di Carli MF, Sullivan NM, Leonen MF, Morrow WR. Myocardial flow reserve in long-term survivors of repair of anomalous left coronary artery from pulmonary artery. J Am Coll Cardiol 1998;31:437-43.

    Article  CAS  PubMed  Google Scholar 

  44. Hauser M, Bengel FM, Kuhn A, Sauer U, Nekolla SG, Eicken A, et al. Myocardial perfusion and coronary flow reserve assessed by positron emission tomography in patients after Fontan-like operations. Pediatr Cardiol 2003;24:386-92.

    Article  CAS  PubMed  Google Scholar 

  45. Brunken RC, Perloff JK, Czernin J, Campisi R, Purcell S, Miner PD, et al. Myocardial perfusion reserve in adults with cyanotic congenital heart disease. Am J Physiol Heart Circ Physiol 2005;289:H1798-806.

    Article  CAS  PubMed  Google Scholar 

  46. Bengel FM, Hauser M, Duvernoy CS, Kuehn A, Ziegler SI, Stollfuss JC, et al. Myocardial blood flow and coronary flow reserve late after anatomical correction of transposition of the great arteries. J Am Coll Cardiol 1998;32:1955-61.

    Article  CAS  PubMed  Google Scholar 

  47. Singh TP, Humes RA, Muzik O, Kottamasu S, Karpawich PP, Di Carli MF. Myocardial flow reserve in patients with a systemic right ventricle after atrial switch repair. J Am Coll Cardiol 2001;37:2120-5.

    Article  CAS  PubMed  Google Scholar 

  48. Rutz T, de Marchi SF, Schwerzmann M, Vogel R, Seiler C. Right ventricular absolute myocardial blood flow in complex congenital heart disease. Heart 2010;96:1056-62.

    Article  PubMed  Google Scholar 

  49. Donnelly JP, Raffel DM, Shulkin BL, Corbett JR, Bove EL, Mosca RS, et al. Resting coronary flow and coronary flow reserve in human infants after repair or palliation of congenital heart defects as measured by positron emission tomography. J Thorac Cardiovasc Surg 1998;115:103-10.

    Article  CAS  PubMed  Google Scholar 

  50. Perloff JK. Cyanotic congenital heart disease the coronary arterial circulation. Curr Cardiol Rev 2012;8:1-5.

    Article  PubMed Central  PubMed  Google Scholar 

  51. Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation 2007;115:534-45.

    Article  PubMed  Google Scholar 

  52. Mulder BJ. Endocarditis in congenital heart disease: Who is at highest risk? Circulation 2013;128:1396-7.

    Article  PubMed  Google Scholar 

  53. Bensimhon L, Lavergne T, Hugonnet F, Mainardi JL, Latremouille C, Maunoury C, et al. Whole body [(18) F]fluorodeoxyglucose positron emission tomography imaging for the diagnosis of pacemaker or implantable cardioverter defibrillator infection: A preliminary prospective study. Clin Microbiol Infec 2011;17:836-44.

    Article  CAS  Google Scholar 

  54. Sarrazin JF, Philippon F, Tessier M, Guimond J, Molin F, Champagne J, et al. Usefulness of fluorine-18 positron emission tomography/computed tomography for identification of cardiovascular implantable electronic device infections. J Am Coll Cardiol 2012;59:1616-25.

    Article  PubMed  Google Scholar 

  55. Yedidya I, Stein GY, Vaturi M, Blieden L, Bernstine H, Pitlik SD, et al. Positron emission tomography/computed tomography for the diagnosis of endocarditis in patients with pulmonic stented valve/pulmonic stent. Ann Thorac Surg 2011;91:287-9.

    Article  PubMed  Google Scholar 

  56. Ruiz-Zafra J, Rodriguez-Fernandez A, Sanchez-Palencia A, Cueto A. Surgical adhesive may cause false positives in integrated positron emission tomography and computed tomography after lung cancer resection. Eur J Cardiothorac Surg 2013;43:1251-3.

    Article  PubMed  Google Scholar 

  57. Skali H, Schulman AR, Dorbala S. (18)F-FDG PET/CT for the assessment of myocardial sarcoidosis. Curr Cardiol Rep 2013;15:352.

    Article  PubMed Central  Google Scholar 

  58. Schwartz ML, Jonas RA, Colan SD. Anomalous origin of left coronary artery from pulmonary artery: Recovery of left ventricular function after dual coronary repair. J Am Coll Cardiol 1997;30:547-53.

    Article  CAS  PubMed  Google Scholar 

  59. Rickers C, Sasse K, Buchert R, Stern H, van den Hoff J, Lubeck M, et al. Myocardial viability assessed by positron emission tomography in infants and children after the arterial switch operation and suspected infarction. J Am Coll Cardiol 2000;36:1676-83.

    Article  CAS  PubMed  Google Scholar 

  60. Hernandez-Pampaloni M, Allada V, Fishbein MC, Schelbert HR. Myocardial perfusion and viability by positron emission tomography in infants and children with coronary abnormalities: Correlation with echocardiography, coronary angiography, and histopathology. J Am Coll Cardiol 2003;41:618-26.

    Article  PubMed  Google Scholar 

  61. Silverman JM, Julien PJ, Herfkens RJ, Pelc NJ. Quantitative differential pulmonary perfusion: MR imaging versus radionuclide lung scanning. Radiology 1993;189:699-701.

    Article  CAS  PubMed  Google Scholar 

  62. Sridharan S, Derrick G, Deanfield J, Taylor AM. Assessment of differential branch pulmonary blood flow: A comparative study of phase contrast magnetic resonance imaging and radionuclide lung perfusion imaging. Heart 2006;92:963-8.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

Download references

Disclosures

Dr. Dorbala was supported by National Heart Lung and Blood Institute grants K23HL092299.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Sharmila Dorbala MBBS, MPH, FACC.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Partington, S.L., Valente, A.M., Landzberg, M. et al. Clinical applications of radionuclide imaging in the evaluation and management of patients with congenital heart disease. J. Nucl. Cardiol. 23, 45–63 (2016). https://doi.org/10.1007/s12350-015-0185-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12350-015-0185-5

Keywords

Navigation