Skip to main content
SpringerLink
Log in

Controversies in Our Current Decade Surrounding the Management of the Adult with Congenital Heart Disease

  • Reference work entry
  • First Online:
Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care

  • 384 Accesses

Abstract

Heart defects occur in 75 of 1,000 live births, 25 % of which are at least moderate in severity. Less than 33 % of infants born with congenital heart disease 50 years ago survived to adulthood. With current advances in pediatric cardiology and surgery, it is now estimated in developed countries that up to 95 % are expected to reach adulthood. As life expectancy improves, the population of adults with congenital heart disease continues to grow. For the first time, it is now estimated that the number of adults with congenital heart disease has surpassed the number of children with congenital heart disease. While this is a remarkable achievement in the field of medicine, it is now apparent that early surgical interventions were reparative and not curative. Adults with congenital heart disease are increasingly requiring medical services and late complications are becoming increasingly apparent. As result, healthcare systems are now challenged to meet the demands of this complex and largely underserved population. This chapter discusses and highlights some of the important advances and controversies in the modern era in the management of the adult with congenital heart disease.

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 1,299.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 1,799.99
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

References

  1. Shimazaki Y, Blackstone EH, Kirklin JW (1984) The natural history of isolated congenital pulmonary valve incompetence: surgical implications. Thorac Cardiovasc Surg 32(4):257–259

    Article  CAS  PubMed  Google Scholar 

  2. Bouzas B, Kilner PJ, Gatzoulis MA (2005) Pulmonary regurgitation: not a benign lesion. Eur Heart J 26(5):433–439

    Article  PubMed  Google Scholar 

  3. Geva T (2006) Indications and timing of pulmonary valve replacement after tetralogy of Fallot repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu: 11–22

    Google Scholar 

  4. Gatzoulis MA et al (2000) Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 356(9234):975–981

    Article  CAS  PubMed  Google Scholar 

  5. Frigiola A et al (2004) Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot. Circulation 110(11 Suppl 1):II153–II157

    CAS  PubMed  Google Scholar 

  6. Carvalho JS et al (1992) Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of residual pulmonary regurgitation. Br Heart J 67(6):470–473

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Gatzoulis MA et al (1995) Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation 92(2):231–237

    Article  CAS  PubMed  Google Scholar 

  8. Oechslin EN et al (1999) Reoperation in adults with repair of tetralogy of fallot: indications and outcomes. J Thorac Cardiovasc Surg 118(2):245–251

    Article  CAS  PubMed  Google Scholar 

  9. Gengsakul A et al (2007) The impact of pulmonary valve replacement after tetralogy of Fallot repair: a matched comparison. Eur J Cardiothorac Surg 32(3):462–468

    Article  PubMed  Google Scholar 

  10. Frigiola A et al (2008) Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome? Circulation 118(14 Suppl):S182–S190

    Article  PubMed  Google Scholar 

  11. Therrien J et al (2001) Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot. Circulation 103(20):2489–2494

    Article  CAS  PubMed  Google Scholar 

  12. van der Wall EE, Mulder BJ (2005) Pulmonary valve replacement in patients with tetralogy of Fallot and pulmonary regurgitation: early surgery similar to optimal timing of surgery? Eur Heart J 26(24):2614–2615

    Article  PubMed  Google Scholar 

  13. Walsh R et al (2011) Repeatability of cardiac-MRI-measured right ventricular size and function in congenital heart disease. Pediatr Radiol 41(8):1000–1007

    Article  PubMed  Google Scholar 

  14. Therrien J et al (2005) Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair. Am J Cardiol 95(6):779–782

    Article  PubMed  Google Scholar 

  15. Dave HH et al (2005) Early insertion of a pulmonary valve for chronic regurgitation helps restoration of ventricular dimensions. Ann Thorac Surg 80(5):1615–1620, discussion 1620–1621

    Article  PubMed  Google Scholar 

  16. Buechel ER et al (2005) Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: assessment by cardiovascular magnetic resonance. Eur Heart J 26(24):2721–2727

    Article  PubMed  Google Scholar 

  17. van Straten A et al (2005) Time course of diastolic and systolic function improvement after pulmonary valve replacement in adult patients with tetralogy of Fallot. J Am Coll Cardiol 46(8):1559–1564

    Article  PubMed  Google Scholar 

  18. Vliegen HW et al (2002) Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of fallot. Circulation 106(13):1703–1707

    Article  PubMed  Google Scholar 

  19. Khambadkone S et al (2005) Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. Circulation 112(8):1189–1197

    Article  PubMed  Google Scholar 

  20. Lurz P et al (2008) Percutaneous pulmonary valve implantation: impact of evolving technology and learning curve on clinical outcome. Circulation 117(15):1964–1972

    Article  PubMed  Google Scholar 

  21. McElhinney DB et al (2010) Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. Circulation 122(5):507–516

    Article  PubMed  PubMed Central  Google Scholar 

  22. Eicken A et al (2011) Percutaneous pulmonary valve implantation: two-centre experience with more than 100 patients. Eur Heart J 32(10):1260–1265

    Article  PubMed  Google Scholar 

  23. Kenny D et al (2011) Percutaneous implantation of the Edwards SAPIEN transcatheter heart valve for conduit failure in the pulmonary position: early phase 1 results from an international multicenter clinical trial. J Am Coll Cardiol 58(21):2248–2256

    Article  PubMed  Google Scholar 

  24. Spence M (2011) Valvular aortic stenosis. In: Gatzoulis WG, Daubeney PEF (eds) Diagnosis and management of adult congenital heart disease. Elsevier Saunders, Philadelphia

    Google Scholar 

  25. David TE (2009) Ross procedure at the crossroads. Circulation 119(2):207–209

    Article  PubMed  Google Scholar 

  26. Sievers H et al (2003) Midterm results of the Ross procedure preserving the patient's aortic root. Circulation 108(Suppl 1):II55–II60

    PubMed  Google Scholar 

  27. Luciani GB et al (2005) Ross operation in the young: a ten-year experience. Ann Thorac Surg 80(6):2271–2277

    Article  PubMed  Google Scholar 

  28. Sievers HH et al (2006) A critical reappraisal of the Ross operation: renaissance of the subcoronary implantation technique? Circulation 114(1 Suppl):I504–I511

    PubMed  Google Scholar 

  29. Elkins RC (1999) The Ross operation: a 12-year experience. Ann Thorac Surg 68(3 Suppl):S14–S18

    Article  CAS  PubMed  Google Scholar 

  30. Mokhles MM et al (2011) Survival comparison of the Ross procedure and mechanical valve replacement with optimal self-management anticoagulation therapy: propensity-matched cohort study. Circulation 123(1):31–38

    Article  PubMed  Google Scholar 

  31. Venkataraman R et al (2009) Late dissection of pulmonary autograft treated by valve-sparing aortic root replacement. J Card Surg 24(4):443–445

    Article  PubMed  Google Scholar 

  32. Luciani GB, Mazzucco A (2006) Aortic root disease after the Ross procedure. Curr Opin Cardiol 21(6):555–560

    Article  PubMed  Google Scholar 

  33. Luciani GB et al (2003) Fate of the aortic root late after Ross operation. Circulation 108(Suppl 1):II61–II67

    PubMed  Google Scholar 

  34. Kincaid EH et al (2004) Dissection in a pulmonary autograft. Ann Thorac Surg 77(2):707–708

    Article  PubMed  Google Scholar 

  35. Elkins RC et al (2008) Ross operation: 16-year experience. J Thorac Cardiovasc Surg 136(3):623–630, 630 e1-5

    Article  PubMed  Google Scholar 

  36. Kouchoukos NT et al (2004) The Ross procedure: long-term clinical and echocardiographic follow-up. Ann Thorac Surg 78(3):773–781, discussion 773–781

    Article  PubMed  Google Scholar 

  37. Chan V et al (2011) Reoperation of left heart valve bioprostheses according to age at implantation. Circulation 124(11 Suppl):S75–S80

    Article  PubMed  Google Scholar 

  38. Klieverik LM et al (2007) Dissection of a dilated autograft root. J Thorac Cardiovasc Surg 133(3):817–818

    Article  PubMed  Google Scholar 

  39. Pasquali SK et al (2007) Midterm outcomes and predictors of reintervention after the Ross procedure in infants, children, and young adults. J Thorac Cardiovasc Surg 133(4):893–899

    Article  PubMed  Google Scholar 

  40. Webb G (2005) Treatment of coarctation and late complications in the adult. Semin Thorac Cardiovasc Surg 17:139–142

    Article  PubMed  Google Scholar 

  41. Crafoord C, Nylin G (1945) Congenital coarctation of the aorta and its surgical treatment. J Thorac Surg 14:347–361

    Google Scholar 

  42. Rothman A (1998) Coarctation of the aorta: an update. Curr Probl Pediatr 28:33–60

    CAS  PubMed  Google Scholar 

  43. Toro-Salazar OH, Steinberger J, Thomas W et al (2002) Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol 89:541–547

    Article  PubMed  Google Scholar 

  44. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC (1989) Coarctation of the aorta. Long-term follow-up and prediction of outcome after surgical correction. Circulation 80:840–845

    Article  CAS  PubMed  Google Scholar 

  45. Forbes TJ, Kim DW, Du W, Turner DR, Holzer R, Amin Z, Hijazi Z, Ghasemi A, Rome JJ, Nykanen D, Zahn E, Cowley C, Hoyer M, Waight D, Gruenstein D, Javois A, Foerster S, Kreutzer J, Sullivan N, Khan A, Owada C, Hagler D, Lim S, Canter J, Zellers T (2011) CCISC I. Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium). J Am Coll Cardiol 58:2664–2674

    Article  PubMed  Google Scholar 

  46. Singer MI, Rowen M, Dorsey TJ (1982) Transluminal aortic balloon angioplasty for coarctation of the aorta in the newborn. Am Heart J 103:131–132

    Article  CAS  PubMed  Google Scholar 

  47. Shaddy RE, Boucek MM, Sturtevant JE, Ruttenberg HD, Jaffe RB, Tani LY, Judd VE, Veasy LG, McGough EC, Orsmond GS (1993) Comparison of angioplasty and surgery for unoperated coarctation of the aorta. Circulation 87:793–799

    Article  CAS  PubMed  Google Scholar 

  48. Holzer R, Qureshi S, Ghasemi A, Vincent J, Sievert H, Gruenstein D, Weber H, Alday L, Peirone A, Zellers T, Cheatham J, Slack M, Rome J (2010) Stenting of aortic coarctation: acute, intermediate, and long-term results of a prospective multi-institutional registry–Congenital Cardiovascular Interventional Study Consortium (CCISC). Catheter Cardiovasc Interv 76:553–563

    Article  PubMed  Google Scholar 

  49. Cribbs MG, Daniels CJ, Cheatham JP, Zaidi AN (2012) From Balloon Angioplasty to Covered Stents in the Management of Coarctation of the Aorta in Adults with Congenital Heart Disease. Progr Pediatr Cardiol 34(2):97–103

    Article  Google Scholar 

  50. Tzifa A, Ewert P, Brzezinska-Rajszys G, Peters B, Zubrzycka M, Rosenthal E, Berger F, Qureshi SA (2006) Covered Cheatham-platinum stents for aortic coarctation: early and intermediate-term results. J Am Coll Cardiol 47:1457–1463

    Article  CAS  PubMed  Google Scholar 

  51. Chessa M, Carrozza M, Butera G, Piazza L, Negura DG, Bussadori C, Bossone E, Giamberti A, Carminati M (2005) Results and mid-long-term follow-up of stent implantation for native and recurrent coarctation of the aorta. Eur Heart J 26:2728–2732

    Article  PubMed  Google Scholar 

  52. Krasemann T, Bano M, Rosenthal E, Qureshi SA (2011) Results of stent implantation for native and recurrent coarctation of the aorta-follow-up of up to 13 years. Catheter Cardiovasc Interv 78(3):405–412

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Wisconsin Adult Congenital Heart Disease Program (WAtCH), Medical College of Wisconsin, Milwaukee, WI, USA

    Scott Cohen, Peter Bartz, Tejas Shah & Michael G. Earing

Authors
  1. Scott Cohen
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Peter Bartz
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Tejas Shah
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Michael G. Earing
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Scott Cohen .

Editor information

Editors and Affiliations

  1. Dept. of Pediatric Cardiology, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    Eduardo M. Da Cruz

  2. Dept. Pediatric Cardiology, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    Dunbar Ivy

  3. Pediatric Cardiac Surgery, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    James Jaggers

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag London

About this entry

Cite this entry

Cohen, S., Bartz, P., Shah, T., Earing, M.G. (2014). Controversies in Our Current Decade Surrounding the Management of the Adult with Congenital Heart Disease. In: Da Cruz, E., Ivy, D., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4619-3_180

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-4619-3_180

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-4618-6

  • Online ISBN: 978-1-4471-4619-3

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation