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Congenital Heart Disease

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Essential Cardiology

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Abstract

Congenital heart disease (CHD) is defined as a cardiovascular defect that is present since birth. Due to substantial medical and surgical advances, the majority of patients with CHD now survive into adulthood and form an exponentially growing population of more than 1,000,000 in the USA. Despite improved survival, it has become apparent that timely interventions are reparative and not curative with significant sequelae and residual hemodynamic lesions in most cases. The complexity of the anatomy and physiology of patients with CHD presents physicians with challenges that are unique to this population. Thus, familiarization with the principles of management of patients with CHD is essential. This book chapter provides an introduction to the most prevalent CHD lesions and summarizes specific issues which are topical to adult patients with CHD.

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References

  1. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12):1890–900.

    Article  PubMed  Google Scholar 

  2. Pierpont ME, Basson CT, Benson Jr DW, et al. Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation. 2007;115(23):3015–38.

    Article  PubMed  Google Scholar 

  3. Gatzoulis MA, Hechter S, Siu SC, Webb GD. Outpatient clinics for adults with congenital heart disease: increasing workload and evolving patterns of referral. Heart. 1999;81(1):57–61.

    PubMed  CAS  Google Scholar 

  4. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2008;52(23):e143–263.

    Article  PubMed  Google Scholar 

  5. Ho SY. Cardiac morphology and nomenclature. In: Gatzoulis MA, Webb GD, Daubeney PEF, editors. Diagnosis and management of adult congenital heart disease. 2nd ed. Philadelphia: WB Saunders; 2011. p. 5–13.

    Google Scholar 

  6. Gatzoulis MA, Freeman MA, Siu SC, Webb GD, Harris L. Atrial arrhythmia after surgical closure of atrial septal defects in adults. N Engl J Med. 1999;340(11):839–46.

    Article  PubMed  CAS  Google Scholar 

  7. Neumayer U, Stone S, Somerville J. Small ventricular septal defects in adults. Eur Heart J. 1998;19(10):1573–82.

    Article  PubMed  CAS  Google Scholar 

  8. Suzuki K, Ho SY, Anderson RH, et al. Morphometric analysis of atrioventricular septal defect with common valve orifice. J Am Coll Cardiol. 1998;31(1):217–23.

    Article  PubMed  CAS  Google Scholar 

  9. Feldt RH, DuShane JW, Titus JL. The atrioventricular conduction system in persistent common atrioventricular canal defect: correlations with electrocardiogram. Circulation. 1970;42(3):437–44.

    Article  PubMed  CAS  Google Scholar 

  10. Berger TJ, Blackstone EH, Kirklin JW, Bargeron Jr LM, Hazelrig JB, Turner Jr ME. Survival and probability of cure without and with operation in complete atrioventricular canal. Ann Thorac Surg. 1979;27(2):104–11.

    Article  PubMed  CAS  Google Scholar 

  11. Roberts WC, Ko JM. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Circulation. 2005;111(7):920–5.

    Article  PubMed  Google Scholar 

  12. Prapa M, Ho SY. Risk stratification in bicuspid aortic valve disease: still more work to do. Eur J Cardiothorac Surg. 2012;41(2):327–8.

    Article  PubMed  Google Scholar 

  13. Lewin MB, Otto CM. The bicuspid aortic valve: adverse outcomes from infancy to old age. Circulation. 2005;111(7):832–4.

    Article  PubMed  Google Scholar 

  14. Oliver JM, Gallego P, Gonzalez A, Aroca A, Bret M, Mesa JM. Risk factors for aortic complications in adults with coarctation of the aorta. J Am Coll Cardiol. 2004;44(8):1641–7.

    Article  PubMed  Google Scholar 

  15. Gatzoulis MA, Till JA, Somerville J, Redington AN. Mechano­electrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation. 1995;92(2):231–7.

    Article  PubMed  CAS  Google Scholar 

  16. Babu-Narayan SV, Kilner PJ, Li W, et al. Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of fallot and its relationship to adverse markers of clinical outcome. Circulation. 2006;113(3):405–13.

    Article  PubMed  CAS  Google Scholar 

  17. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein’s anomaly. Circulation. 2007;115(2):277–85.

    Article  PubMed  Google Scholar 

  18. Hornung TS, Derrick GP, Deanfield JE, Redington AN. Transposition complexes in the adult: a changing perspective. Cardiol Clin. 2002;20(3):405–20.

    Article  PubMed  Google Scholar 

  19. Gelatt M, Hamilton RM, McCrindle BW, et al. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol. 1997;29(1):194–201.

    Article  PubMed  CAS  Google Scholar 

  20. Dos L, Teruel L, Ferreira IJ, et al. Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries. Heart. 2005;91(5):652–6.

    Article  PubMed  CAS  Google Scholar 

  21. Warnes CA. Transposition of the great arteries. Circulation. 2006;114(24):2699–709.

    Article  PubMed  Google Scholar 

  22. Ammash NM, Warnes CA. Survival into adulthood of patients with unoperated single ventricle. Am J Cardiol. 1996;77(7):542–4.

    Article  PubMed  CAS  Google Scholar 

  23. d’Udekem Y, Iyengar AJ, Cochrane AD, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation. 2007;116(11 Suppl):I157–64.

    PubMed  Google Scholar 

  24. Gatzoulis MA, Munk MD, Williams WG, Webb GD. Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology. Heart. 2000;83(1):51–7.

    Article  PubMed  CAS  Google Scholar 

  25. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573–619.

    Article  PubMed  Google Scholar 

  26. Cantor WJ, Harrison DA, Moussadji JS, et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol. 1999;84(6):677–81.

    Article  PubMed  CAS  Google Scholar 

  27. Dimopoulos K, Diller GP, Piepoli MF, Gatzoulis MA. Exercise intolerance in adults with congenital heart disease. Cardiol Clin. 2006;24(4):641–60, vii.

    Article  PubMed  Google Scholar 

  28. Horton RP, Canby RC, Kessler DJ, et al. Ablation of ventricular tachycardia associated with tetralogy of Fallot: demonstration of bidirectional block. J Cardiovasc Electrophysiol. 1997;8(4):432–5.

    Article  PubMed  CAS  Google Scholar 

  29. Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). J Am Coll Cardiol. 2006;48(5):e247–346.

    Article  PubMed  Google Scholar 

  30. Khairy P, Ouyang DW, Fernandes SM, Lee-Parritz A, Economy KE, Landzberg MJ. Pregnancy outcomes in women with congenital heart disease. Circulation. 2006;113(4):517–24.

    Article  PubMed  Google Scholar 

  31. Joglar JA, Page RL. Antiarrhythmic drugs in pregnancy. Curr Opin Cardiol. 2001;16(1):40–5.

    Article  PubMed  CAS  Google Scholar 

Recommended Reading

  • Gatzoulis MA, Webb GD, Broberg C, Uemura H, editors. Cases in adult congenital heart disease. Philadelphia: Churchill Livingstone; 2010.

    Google Scholar 

  • Gatzoulis MA, Webb GD, Daubeney PEF, editors. Diagnosis and management of adult congenital heart disease. 2nd ed. Philadelphia: WB Saunders; 2011.

    Google Scholar 

  • Steer PJ, Gatzoulis MA, Baker P, editors. Heart disease and pregnancy. London: The Royal College of Obstetricians and Gynaecologists; 2006.

    Google Scholar 

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Authors and Affiliations

  1. Adult Congenital Heart Disease, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK

    Matina Prapa MD, Dimitra Krexi MD, Anselm Uebing MD, PhD & Michael A. Gatzoulis MD, PhD

Authors
  1. Matina Prapa MD
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  2. Dimitra Krexi MD
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  3. Anselm Uebing MD, PhD
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  4. Michael A. Gatzoulis MD, PhD
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Corresponding author

Correspondence to Matina Prapa MD .

Editor information

Editors and Affiliations

  1. The Mount Sinai School of Medicine, The James J. Peters VA Medical Center, Department of Medicine, West Kingsbridge Rd 130, Bronx, 10468, New York, USA

    Clive Rosendorff

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Prapa, M., Krexi, D., Uebing, A., Gatzoulis, M.A. (2013). Congenital Heart Disease. In: Rosendorff, C. (eds) Essential Cardiology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6705-2_20

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  • DOI: https://doi.org/10.1007/978-1-4614-6705-2_20

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-6704-5

  • Online ISBN: 978-1-4614-6705-2

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