Pediatric Cardiology

, Volume 33, Issue 3, pp 439–451

Noninvasive Imaging Modalities and Sudden Cardiac Arrest in the Young: Can They Help Distinguish Subjects With a Potentially Life-Threatening Abnormality From Normals?

Review Article


Sudden cardiac arrest (SCA) in the young is always tragic, but fortunately it is an unusual event. When it does occur, it usually happens in active individuals, often while they are participating in physical activity. Depending on the population’s characteristics, the most common causes of sudden cardiac arrest in these subjects are hypertrophic cardiomyopathy, congenital coronary abnormalities, arrhythmia in the presence of a structurally normal heart (ion channelopathies or abnormal conduction pathways), aortic rupture, and arrhythmogenic right-ventricular cardiomyopathy. Two-dimensional echocardiography (2-DE) has been proposed as a screening tool that can potentially detect four of these five causes of SCA, and many groups now sponsor community-based 2-DE SCA-screening programs. “Basic” 2-DE screening may include assessment of ventricular volumes, mass, and function; left atrial size; and cardiac and thoracic vascular (including coronary) anatomy. “Advanced” echocardiographic techniques, such as tissue Doppler and strain imaging, can help in diagnosis when the history, electrocardiogram (ECG), and/or standard 2-DE screening suggest there may be an abnormality, e.g., to help differentiate those with “athlete’s heart” from hypertrophic or dilated cardiomyopathy. Cardiac magnetic resonance imaging or cardiac computed tomography can be added to increase diagnostic sensitivity and specificity in select cases when an abnormality is suggested during SCA screening. Test availability, cost, and ethical issues related to who to screen, as well as the detection of those with potential disease but low risk, must be balanced when deciding what tests to perform to assess for increased SCA risk.


Noninvasive imaging Sudden cardiac arrest Athletes 


  1. 1.
    Abhayaratna WP, Seward JB, Appleton CP, Douglas PS, Oh JK, Tajik AJ et al (2006) Left atrial size: physiologic determinants and clinical applications. J Am Coll Cardiol 47:2357–2363PubMedCrossRefGoogle Scholar
  2. 2.
    Afonso LC, Bernal J, Bax JJ, Abraham TP (2008) Echocardiography in hypertrophic cardiomyopathy: the role of conventional and emerging technologies. JACC Cardiovasc Imaging 1:787–800PubMedCrossRefGoogle Scholar
  3. 3.
    Afonso L, Hari P, Pidlaoan V, Kondur A, Jacob S, Khetarpal V (2010) Acute myocarditis: can novel echocardiographic techniques assist with diagnosis? Eur J Echocardiogr 11:E5PubMedCrossRefGoogle Scholar
  4. 4.
    American College of Cardiology Foundation Task Force on Expert Consensus Documents, Hundley WG, Bluemke DA, Finn JP, Flamm SD, Fogel MA, et al (2010) ACCF/ACR/AHA/NASCI/SCMR 2010 expert consensus document on cardiovascular magnetic resonance: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents. J Am Coll Cardiol 5523:2614–2662Google Scholar
  5. 5.
    Arrigan MT, Killeen RP, Dodd JD, Torreggiani WC (2011) Imaging spectrum of sudden athlete cardiac death. Clin Radiol 663:203–223CrossRefGoogle Scholar
  6. 6.
    Atchley AE Jr, Douglas PS (2007) Left ventricular hypertrophy. Cardiol Clin 25:371–382PubMedCrossRefGoogle Scholar
  7. 7.
    Baggish AL, Thompson PD (2007) The Athlete’s heart 2007: diseases of the coronary circulation. Cardiol Clin 25:431–440PubMedCrossRefGoogle Scholar
  8. 8.
    Baggish AL, Hutter AM Jr, Wang F, Yared K, Weiner RB, Kupperman E et al (2010) Cardiovascular screening in college athletes with and without electrocardiography: a cross-sectional study. Ann Intern Med 152:269–275PubMedGoogle Scholar
  9. 9.
    Baggish AL, Wood MJ (2011) Athlete’s heart and cardiovascular care of the athlete: scientific and clinical update. Circulation 123:2723–2735PubMedCrossRefGoogle Scholar
  10. 10.
    Basavarajaiah S, Wilson M, Junagde S, Jackson G, Whyte G, Sharma S et al (2006) Physiological left ventricular hypertrophy or hypertrophic cardiomyopathy in an elite adolescent athlete: role of detraining in resolving the clinical dilemma. Br J Sports Med 40:727–729PubMedCrossRefGoogle Scholar
  11. 11.
    Basavarajaiah S, Boraita A, Whyte G, Wilson M, Carby L, Shah A et al (2008) Ethnic differences in left ventricular remodelling in highly trained athletes: relevance to differentiating physiologic left ventricular hypertrophy from hypertrophic cardiomyopathy. J Am Coll Cardiol 51:2256–2262PubMedCrossRefGoogle Scholar
  12. 12.
    Basso C, Maron BJ, Corrado D et al (2000) Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol 35:1493–1501PubMedCrossRefGoogle Scholar
  13. 13.
    Basso C, Carturan E, Corrado D, Thiene G (2007) Myocarditis and dilated cardiomyopathy in athletes: diagnosis, management, and recommendations for sport activity. Cardiol Clin 25:423–429PubMedCrossRefGoogle Scholar
  14. 14.
    Benjamin EJ, D’Agostino RB, Belanger AJ, Wolf PA, Levy D (1995) Left atrial size and the risk of stroke and death: the Framingham heart study. Circulation 92:835–841PubMedGoogle Scholar
  15. 15.
    Black A, Black MM, Gensini G (1975) Exertion and acute coronary artery injury. Angiology 26:759–783PubMedCrossRefGoogle Scholar
  16. 16.
    Bonow RO (2008) Bicuspid aortic valves and dilated aortas: a critical review of the ACC/AHA practice guidelines recommendations. Am J Cardiol 102:111–114PubMedCrossRefGoogle Scholar
  17. 17.
    Butz T, van Buuren F, Mellwig KP, Langer C, Plehn G, Meissner A, Trappe HJ et al (2010) Two-dimensional strain analysis of the global and regional myocardial function for the differentiation of pathologic and physiologic left ventricular hypertrophy: a study in athletes and in patients with hypertrophic cardiomyopathy. Int J Cardiovasc Imaging 27(1):91–100PubMedCrossRefGoogle Scholar
  18. 18.
    Butz T, van Buuren F, Mellwig KP, Langer C, Oldenburg O, Treusch KA et al (2010) Systolic and early diastolic left ventricular velocities assessed by tissue Doppler imaging in 100 top-level handball players. Eur J Cardiovasc Prev Rehabil 17:342–348PubMedGoogle Scholar
  19. 19.
    Calderón FJ, Díaz V, Peinado AB, Benito PJ, Maffulli N (2010) Cardiac dimensions over 5 years in highly trained long-distance runners and sprinters. Phys Sportsmed 38:112–118PubMedCrossRefGoogle Scholar
  20. 20.
    Corrado D, Basso C, Schiavon M, Thiene G (1998) Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med 339:364–369PubMedCrossRefGoogle Scholar
  21. 21.
    Corrado D, Pelliccia A, Heidbuchel H, Sharma S, Link M, Basso C et al (2010) Recommendations for interpretation of 12-lead electrocardiogram in the athlete. Eur Heart J 31:243–259PubMedCrossRefGoogle Scholar
  22. 22.
    Cox MG, van der Zwaag PA, van der Werf C, van der Smagt JJ, Noorman M, Bhuiyan ZA et al (2011) Arrhythmogenic right ventricular dysplasia/cardiomyopathy: pathogenic desmosome mutations in index-patients predict outcome of family screening: Dutch arrhythmogenic right ventricular dysplasia/cardiomyopathy genotype-phenotype follow-up study. Circulation 123:2690–2700PubMedCrossRefGoogle Scholar
  23. 23.
    Elliot P (2000) Diagnosis and management of dilated cardiomyopathy. Heart 84:106–112CrossRefGoogle Scholar
  24. 24.
    Friedrich MG, Sechtem U, Schulz-Menger J, Holmvang G, Alakija P, Cooper LT et al (2009) Cardiovascular magnetic resonance in myocarditis: a JACC white paper. J Am Coll Cardiol 53:1475–1487PubMedCrossRefGoogle Scholar
  25. 25.
    Fukuda S, Shimada K, Kawasaki T, Fujimoto H, Maeda K, Inanami H (2009) Pocket-sized transthoracic echocardiography device for the measurement of cardiac chamber size and function. Circ J 73(6):1092–1096PubMedCrossRefGoogle Scholar
  26. 26.
    Ganame J, Mertens L, Eidem BW, Claus P, D’hooge J, Havemann LM et al (2007) Regional myocardial deformation in children with hypertrophic cardiomyopathy: morphological and clinical correlations. Eur Heart J 28:2886–2894PubMedCrossRefGoogle Scholar
  27. 27.
    Germans T, Rüssel IK, Götte MJ, Spreeuwenberg MD, Doevendans PA, Pinto YM et al (2010) How do hypertrophic cardiomyopathy mutations affect myocardial function in carriers with normal wall thickness? Assessment with cardiovascular magnetic resonance. J Cardiovasc Magn Reson 12:13PubMedCrossRefGoogle Scholar
  28. 28.
    Gordon JB, Kahn AM, Burns JC (2009) When children with Kawasaki disease grow up: myocardial and vascular complications in adulthood. J Am Coll Cardiol 54:1911–1920PubMedCrossRefGoogle Scholar
  29. 29.
    Harris KM, Sponsel A, Hutter AM Jr, Maron BJ (2006) Brief communication: Cardiovascular screening practices of major North American professional sports teams. Ann Intern Med 145:507–511PubMedGoogle Scholar
  30. 30.
    Hatzaras I, Tranquilli M, Coady M, Barrett PM, Bible J, Elefteriades JA (2007) Weight lifting and aortic dissection: more evidence for a connection. Cardiology 107:103–106PubMedCrossRefGoogle Scholar
  31. 31.
    Ho CY, Sweitzer NK, McDonough B, Maron BJ, Casey SA, Seidman JG et al (2002) Assessment of diastolic function with Doppler tissue imaging to predict genotype in preclinical hypertrophic cardiomyopathy. Circulation 105:2992–2997PubMedCrossRefGoogle Scholar
  32. 32.
    Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA et al (2005) Recommendations for chamber quantification: a report from the American Society of Echocardiography’s guidelines and standards committee and the chamber quantification writing group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 18:1440–1463PubMedCrossRefGoogle Scholar
  33. 33.
    Lopez L, Colan SD, Frommelt PC, Ensing GL, Kendall K, Younoszai AK et al (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 23:465–495PubMedCrossRefGoogle Scholar
  34. 34.
    Losi MA, Betocchi S, Barbati G, Parisi V, Tocchetti CG, Pastore F et al (2009) Prognostic significance of left atrial volume dilatation in patients with hypertrophic cardiomyopathy. J Am Soc Echocardiogr 22:76–81PubMedCrossRefGoogle Scholar
  35. 35.
    Losi MA, Nistri S, Galderisi M, Betocchi S, Cecchi F, Olivotto I et al (2010) Echocardiography in patients with hypertrophic cardiomyopathy: usefulness of old and new techniques in the diagnosis and pathophysiological assessment. Cardiovasc Ultrasound 8:7–26PubMedGoogle Scholar
  36. 36.
    Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA et al (2010) Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation 121:1533–1541PubMedCrossRefGoogle Scholar
  37. 37.
    Maron BJ (2002) Hypertrophic cardiomyopathy: a systematic review. JAMA 287:1308–1320PubMedCrossRefGoogle Scholar
  38. 38.
    Maron BJ, Pelliccia A (2006) The heart of trained athletes: cardiac remodeling and the risks of sports, including sudden death. Circulation 114:1633–1644PubMedCrossRefGoogle Scholar
  39. 39.
    Maron BJ, Thompson PD, Ackerman MJ, Balady G, Berger S, Cohen D et al (2007) Recommendations and considerations related to preparticipation screening for cardiovascular abnormalities in competitive athletes: 2007 update. Circulation 115:1643–1655PubMedCrossRefGoogle Scholar
  40. 40.
    Maron BJ (2009) Distinguishing hypertrophic cardiomyopathy from athlete’s heart physiological remodelling: clinical significance, diagnostic strategies and implications for preparticipation screening. Br J Sports Med 43:649–656PubMedCrossRefGoogle Scholar
  41. 41.
    Menon SC, Ackerman MJ, Cetta F, O’Leary PW, Eidem BW (2008) Significance of left atrial volume in patients <20 years of age with hypertrophic cardiomyopathy. Am J Cardiol 102:1390–1393PubMedCrossRefGoogle Scholar
  42. 42.
    Nagueh SF, McFalls J, Meyer D, Hill R, Zoghbi WA, Tam JW et al (2003) Tissue Doppler imaging predicts the development of hypertrophic cardiomyopathy in subjects with sub-clinical disease. Circulation 108:395–398PubMedCrossRefGoogle Scholar
  43. 43.
    Nagueh SF, Mahmarian JJ (2006) Noninvasive cardiac imaging in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 48:2410–2422PubMedCrossRefGoogle Scholar
  44. 44.
    Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC et al (2004) Diagnosis, treatment, and long-term management of Kawasaki Disease. A statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Endorsed by the American Academy of Pediatrics. Circulation 110:2747–2771PubMedCrossRefGoogle Scholar
  45. 45.
    Nistri S, Olivotto I, Betocchi S, Losi MA, Valsecchi G, Pinamonti B et al (2006) Prognostic significance of left atrial size in patients with hypertrophic cardiomyopathy (from the Italian Registry for Hypertrophic Cardiomyopathy). Am J Cardiol 98:960–965PubMedCrossRefGoogle Scholar
  46. 46.
    Osborn RQ, Taylor WC, Oken K, Luzano M, Heckman M, Fletcher G (2007) Echocardiographic characterisation of left ventricular geometry of professional male tennis players. Br J Sports Med 41:789–792PubMedCrossRefGoogle Scholar
  47. 47.
    Pelliccia A, Maron BJ, Spataro A, Proschan MA, Spirito P (1991) The upper limit of physiologic cardiac hypertrophy in highly trained elite athletes. N Engl J Med 324:295–301PubMedCrossRefGoogle Scholar
  48. 48.
    Prakken NH, Velthuis BK, Cramer MJ, Mosterd A (2009) Advances in cardiac imaging: the role of magnetic resonance imaging and computed tomography in identifying athletes at risk. Br J Sports Med 43:677–684PubMedCrossRefGoogle Scholar
  49. 49.
    Prakken NH, Cramer MJ, Olimulder MA, Agostoni P, Mali WP, Velthuis BK (2010) Screening for proximal coronary artery anomalies with 3-dimensional MR coronary angiography. Int J Cardiovasc Imaging 266:701e–710eCrossRefGoogle Scholar
  50. 50.
    Quarta G, Muir A, Pantazis A, Syrris P, Gehmlich K, Garcia-Pavia P et al (2011) Familial evaluation in arrhythmogenic right ventricular cardiomyopathy. Impact of genetics and revised task force criteria. Circulation 123:2701–2709PubMedCrossRefGoogle Scholar
  51. 51.
    Rawlins J, Bhan A, Sharma S (2009) Left ventricular hypertrophy in athletes. Eur J Echocardiogr 10:350–356PubMedCrossRefGoogle Scholar
  52. 52.
    Rawlins J, Carre F, Kervio G, Papadakis M, Chandra N, Edwards C et al (2010) Ethnic differences in physiological cardiac adaptation to intense physical exercise in highly trained female athletes. Circulation 121:1078–1085PubMedCrossRefGoogle Scholar
  53. 53.
    Rickers C, Wilke NM, Jerosch-Herold M, Casey SA, Panse P, Panse N et al (2005) Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy. Circulation 112:855–861PubMedCrossRefGoogle Scholar
  54. 54.
    Rost R (1992) The athlete’s heart: historical perspective. In: Maron BJ (ed) Cardiology clinics, the athlete’s heart. Saunders, Philadelphia, pp 197–207Google Scholar
  55. 55.
    Rüssel IK, Brouwer WP, Germans T, Knaapen P, Marcus JT, van der Velden J et al (2010) Increased left ventricular torsion in hypertrophic cardiomyopathy mutation carriers with normal wall thickness. J Cardiovasc Magn Reson 10:3Google Scholar
  56. 56.
    Sparrow PJ, Merchant N, Provost YL, Doyle DJ, Nguyen ET, Paul NS (2009) CT and MR imaging findings in patients with acquired heart disease at risk for sudden cardiac death. Radiographics 29:805–823PubMedCrossRefGoogle Scholar
  57. 57.
    Spirito P, Bellone P, Harris KM, Bernabo P, Bruzzi P, Maron BJ (2000) Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N Engl J Med 342:1778–1785PubMedCrossRefGoogle Scholar
  58. 58.
    Stout M (2009) The Marfan syndrome: implications for athletes and their echocardiographic assessment. Echocardiography 269:1075–1081CrossRefGoogle Scholar
  59. 59.
    Taggart NW, Cetta F, O’Leary PW, Seward JB, Eidem BW (2010) Left atrial volume in children without heart disease and in those with ventricular septal defect or patent ductus arteriosus or hypertrophic cardiomyopathy. Am J Cardiol 106:1500–1504PubMedCrossRefGoogle Scholar
  60. 60.
    Tsuda E, Arakaki Y, Shimizu T et al (2005) Changes in causes of sudden deaths by decade in patients with coronary arterial lesions due to Kawasaki disease. Cardiol Young 15:481–488PubMedCrossRefGoogle Scholar
  61. 61.
    Tümüklü MM, Etikan I, Cinar CS (2008) Left ventricular function in professional football players evaluated by tissue Doppler imaging and strain imaging. Int J Cardiovasc Imaging 24:25–35PubMedCrossRefGoogle Scholar
  62. 62.
    Ujino K, Barnes ME, Cha SS, Langins AP, Bailey KR, Seward JB et al (2006) Two-dimensional echocardiographic methods for assessment of left atrial volume. Am J Cardiol 98:1185–1188PubMedCrossRefGoogle Scholar
  63. 63.
    Zeppilli P, Dello Russo A, Santini C, Palmieri V, Natale L, Giordano A et al (1998) In vivo detection of coronary artery anomalies in asymptomatic athletes by echocardiographic screening. Chest 114:89–93PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Division of Cardiology, Department of PediatricsRady Children’s Hospital, San Diego and University of California, San DiegoSan DiegoUSA

Personalised recommendations