Pediatric Cardiology

, Volume 35, Issue 2, pp 323–331 | Cite as

Electrocardiographic Screening for Hypertrophic Cardiomyopathy and Long QT Syndrome: The Drivers of Cost-Effectiveness for the Prevention of Sudden Cardiac Death

  • Brett R. Anderson
  • Sean McElligott
  • Daniel Polsky
  • Victoria L. Vetter
Original Article

Abstract

It is universally recognized that the prevention of sudden cardiac death (SCD) in youth is an important public health initiative. The best approach remains uncertain. Many European and Asian countries support the use of electrocardiograms (ECGs). In the United States, this is highly controversial. Many debate its cost-effectiveness. We designed a comprehensive economic model of two of the most prevalent causes of SCD identifiable by ECG, hypertrophic cardiomyopathy (HCM) and long QT syndrome (LQTS), to determine the drivers of uncertainty in the estimate of cost-effectiveness. We compared the cost-effectiveness of screening with history and physical examination (H&P) plus ECG to the current United States standard, H&P alone, for the detection and treatment of HCM and LQTS. We used a Markov model on a theoretical cohort of healthy 12-year-olds over a 70-year time horizon from a societal perspective, employing extensive univariable and probabilistic sensitivity analyses, to determine drivers of costs and effectiveness. The incremental cost-effectiveness of adding ECGs to H&Ps was $41,400/life-year saved. The model was highly sensitive to the effect of identification and treatment of previously undiagnosed individuals with HCM; however, it was insensitive to many variables commonly assumed to be significant, including the costs of ECGs, echocardiograms, and genetic testing, as well as the sensitivity and specificity of ECGs. No LQTS-related parameters were significant. This study suggests that the key to determining the cost-effectiveness of ECG screening in the United States lies in developing a better understanding of disease progression in the previously undiagnosed HCM population.

Keywords

Cost-effectiveness ECG screening Sudden death Hypertrophic cardiomyopathy Long QT syndrome 

Notes

Conflict of interest

None.

Supplementary material

246_2013_779_MOESM1_ESM.docx (148 kb)
Supplementary material 1 (DOCX 147 kb)

References

  1. 1.
    Ackerman MJ, Priori SG, Willems S, Berul C, Brugada R, Calkins H et al (2011) HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies. This document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm 8:1308–1339CrossRefPubMedGoogle Scholar
  2. 2.
    Arias E (2011) United States life tables, 2007. Natl Vital Stat Rep 59:1–60PubMedGoogle Scholar
  3. 3.
    Centers for Medicare and Medicaid Services (2010) Available at: http://www.cms.gov/Medicare/Medicare.html. Accessed 15 June 2010
  4. 4.
    Chaitman BR (2007) An electrocardiogram should not be included in routine preparticipation screening of young athletes. Circulation 116:2610–2615CrossRefPubMedGoogle Scholar
  5. 5.
    Corrado D, Pelliccia A, Bjornstad HH, Vanhees L, Biffi A, Borjesson M et al (2005) Cardiovascular pre-participation screening of young competitive athletes for prevention of sudden death: proposal for a common European protocol. Consensus statement of the Study Group of Sport Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology. Eur Heart J 26:516–524CrossRefPubMedGoogle Scholar
  6. 6.
    Corrado D, Basso C, Pavei A, Michieli P, Schiavon M, Thiene G (2006) Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program. JAMA 296:1593–1601CrossRefPubMedGoogle Scholar
  7. 7.
    Costco (2010) Pharmacy drug information. Available at: http://www.costco.com/Pharmacy/DrugInformationaspx?p=1. Accessed 15 June 2010
  8. 8.
    Denchev P, Kaltman JR, Schoenbaum M, Vitiello B (2010) Modeled economic evaluation of alternative strategies to reduce sudden cardiac death among children treated for attention deficit/hyperactivity disorder. Circulation 121:1329–1337CrossRefPubMedGoogle Scholar
  9. 9.
    Elliott P, Poloniecki J, Dickie S, Sharma S, Monserrat L, Varnava AM et al (2000) Sudden death in hypertrophic cardiomyopathy identification of high risk patients. J Am Coll Cardiol 36:2212–2218CrossRefPubMedGoogle Scholar
  10. 10.
    Fuller C (2000) Cost effectiveness analysis of screening of high school athletes for risk of sudden cardiac death. Med Sci Sports Exerc 32:887–890CrossRefPubMedGoogle Scholar
  11. 11.
    Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS et al (2011) 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy. A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 58:e212–e260CrossRefPubMedGoogle Scholar
  12. 12.
    Goldenberg I, Moss AJ, Peterson DR, McNitt S, Zareba W, Andrews ML et al (2008) Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome. Circulation 117:2184–2191PubMedCentralCrossRefPubMedGoogle Scholar
  13. 13.
    Goldenberg I, Zareba W, Moss AJ (2008) Long QT syndrome. Curr Probl Cardiol 33:629–694CrossRefPubMedGoogle Scholar
  14. 14.
    Griebsch I (2005) Quality-adjusted life-years lack quality in pediatric care: a critical review of published cost-utility studies in child health. Pediatrics 115:e600–e614CrossRefPubMedGoogle Scholar
  15. 15.
    Halkin A, Steinvil A, Rosso R, Adler A, Rozovski U, Viskin S (2012) Preventing sudden death of athletes with electrocardiographic screening: what is the absolute benefit and how much will it cost? J Am Coll Cardiol 60:2271–2276CrossRefPubMedGoogle Scholar
  16. 16.
    Harris KM (2006) Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation 114:216–225CrossRefPubMedGoogle Scholar
  17. 17.
    Hobbs JB, Peterson DR, Moss AJ, McNitt S, Zareba W, Goldenberg I et al (2006) Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome. JAMA 296:1249–1254CrossRefPubMedGoogle Scholar
  18. 18.
    International Olympic Committee Medical Commission (2004) Sudden cardiovascular death in sports. Lousanne recommendations. Available at: http://www.olympic.org/Documents/Reports/EN/en_report_886.pdf.
  19. 19.
    Kaltman JR, Thompson PD, Lantos J, Berul CI, Botkin J, Cohen JT et al (2011) Screening for sudden cardiac death in the young: report from a National Heart, Lung, and Blood Institute Working Group. Circulation 123:1911–1918CrossRefPubMedGoogle Scholar
  20. 20.
    Leslie LK, Cohen JT, Newburger JW, Alexander ME, Wong JB, Sherwin ED et al (2012) The costs and benefits of targeted screening for causes of sudden cardiac death in children and adolescents. Circulation 125:2621–2629PubMedCentralCrossRefPubMedGoogle Scholar
  21. 21.
    Liberthson R (1996) Sudden death from cardiac causes in children and young adults. N Engl J Med 334:1039–1044CrossRefPubMedGoogle Scholar
  22. 22.
    Maron BJ (1999) Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA 281:650–655CrossRefPubMedGoogle Scholar
  23. 23.
    Maron B, Roberts W, Epstein S (1982) Sudden death in hypertrophic cardiomyopathy a profile of 78 patients. Circulation 65:1388–1394CrossRefPubMedGoogle Scholar
  24. 24.
    Maron B, Shen W, Link M, Epstein A, Almquist A, Daubert J et al (2000) Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med 342:365–373CrossRefPubMedGoogle Scholar
  25. 25.
    Maron BJ, Olivotto I, Bellone P, Conte MR, Cecchi F, Flygenring BP et al (2002) Clinical profile of stroke in 900 patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 39:301–307CrossRefPubMedGoogle Scholar
  26. 26.
    Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE et al (2003) American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. J Am Coll Cardiol 42:1687–1713CrossRefPubMedGoogle Scholar
  27. 27.
    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: a scientific statement from the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: Endorsed by the American College of Cardiology Foundation. Circulation 115:1643–1655CrossRefPubMedGoogle Scholar
  28. 28.
    Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO (2009) Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980–2006. Circulation 119:1085–1092CrossRefPubMedGoogle Scholar
  29. 29.
    Myerburg RJ, Vetter VL (2007) Electrocardiograms should be included in preparticipation screening of athletes. Circulation 116:2616–2626CrossRefPubMedGoogle Scholar
  30. 30.
    O’Connor DP, Knoblauch MA (2010) Electrocardiogram testing during athletic preparticipation physical examinations. J Athl Train 45:265–272PubMedCentralCrossRefPubMedGoogle Scholar
  31. 31.
    Östman-Smith I, Wettrell G, Keeton B, Holmgren D, Ergander U, Gould S et al (2008) Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy. Eur Heart J 29:1160–1167CrossRefPubMedGoogle Scholar
  32. 32.
    Priori SG, Schwartz PJ, Napolitano C, Bloise R, Ronchetti E, Grillo M et al (2003) Risk stratification in the long-QT syndrome. N Engl J Med 348:1866–1874CrossRefPubMedGoogle Scholar
  33. 33.
    Quaglini S, Ragnoni C, Spazzolini C, Priori S, Mannarino S, Schwartz PJ (2006) Cost-effectiveness of neonatal ECG screening for the long QT syndrome. Eur Heart J 27:1824–1832CrossRefPubMedGoogle Scholar
  34. 34.
    Schoenbaum M, Denchev P, Vitiello B, Kaltman JR (2012) Economic evaluation of strategies to reduce sudden cardiac death in young athletes. Pediatrics 130:e380–e389PubMedCentralCrossRefPubMedGoogle Scholar
  35. 35.
    Spirito P, Rapezzi C, Autore C, Bruzzi P, Bellone P, Ortolani P et al (1994) Prognosis of asymptomatic patients with hypertrophic cardiomyopathy and nonsustained ventricular tachycardia. Circulation 90:2743–2747CrossRefPubMedGoogle Scholar
  36. 36.
    Spirito P, Seidman C, McKenna W, Maron B (1997) The management of hypertrophic cardiomyopathy. N Engl J Med 336:775–785CrossRefPubMedGoogle Scholar
  37. 37.
    Steinvil A, Chundadze T, Zeltser D, Rogowski O, Halkin A, Galily Y et al (2011) Mandatory electrocardiographic screening of athletes to reduce their risk for sudden death: proven fact or wishful thinking? J Am Coll Cardiol 57:1291–1296CrossRefPubMedGoogle Scholar
  38. 38.
    Tanaka Y, Yoshinaga M, Anan R, Tanaka Y, Nomura Y, Oku S et al (2006) Usefulness and cost effectiveness of cardiovascular screening of young adolescents. Med Sci Sports Exerc 38:2–6CrossRefPubMedGoogle Scholar
  39. 39.
    United States Census (2010) Available at: http://www.census.gov/population/age/. Accessed 2 Aug 2012
  40. 40.
    Wheeler M, Heidenreich P, Froelicher V, Hlatky M, Ashley E (2010) Cost-effectiveness of preparticipation screening for prevention of sudden cardiac death in young athletes. Ann Intern Med 152:276–286PubMedCentralCrossRefPubMedGoogle Scholar
  41. 41.
    Zupancic J, Triedman J, Alexander M, Walsh E, Richardson D, Berul C (2000) Cost-effectiveness and implications of newborn screening for prolongation of QT interval for the prevention of sudden infant death syndrome. J Pediatr 136:481–489CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Brett R. Anderson
    • 1
    • 2
    • 3
  • Sean McElligott
    • 4
  • Daniel Polsky
    • 5
  • Victoria L. Vetter
    • 6
  1. 1.The Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Division of Pediatric CardiologyNewYork-Presbyterian/Morgan Stanley Children’s HospitalNew YorkUSA
  3. 3.Columbia Presbyterian Medical CenterNew YorkUSA
  4. 4.Department of Healthcare Management and Economics, The Wharton SchoolUniversity of PennsylvaniaPhiladelphiaUSA
  5. 5.Division of General Internal Medicine, Perelman School of MedicineUniversity of PennsylvaniaPhiladelphiaUSA
  6. 6.Division of Pediatric Cardiology, The Children’s Hospital of Philadelphia, Perelman School of MedicineUniversity of PennsylvaniaPhiladelphiaUSA

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