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Critical Care Management of the Adult with Tetralogy of Fallot

  • Giuseppe Isgro
  • Marco Ranucci
  • Massimo Chessa
Chapter
Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)

Abstract

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease and usually is corrected in infancy with low mortality rate; after repair, pulmonary regurgitation is common, but in general surgery is not required until adulthood. Pulmonary valve replacement (PVR) is the most frequent operation in adult TOF patients, while complete correction in natural history is less frequent in developed countries but can take place in those patients coming from developing countries where a cardiac surgery program is not available. Operated TOF patients can suffer also from residual defects which derive from previous surgeries. Morbidity and mortality rates after reoperation are usually low, but different conditions can increase the postoperative risk. Adult TOF patients requiring any surgery are always a challenge for anesthesiologists and intensivists, and a very good knowledge of the pathophysiology and associated treatments is important to reduce the incidence of adverse events correlated mainly to right ventricle (RV) failure.

Keywords

Tetralogy of Fallot Right ventricle Pulmonary regurgitation Pulmonary valve replacement Residual ventricular septal defect Acute heart failure Acute kidney injury Arrhythmias Right ventricular failure Postoperative myocardial infarction 

References

  1. 1.
    Sadiq A, Shyamkrishnan KG, Theodore S, Gopalakrishnan S, Tarakan JM, Karunakan J. Long-term functional assessment after correction of tetralogy of Fallot in adulthood. Ann Thorac Surg. 2007;83(5):1790–5.CrossRefGoogle Scholar
  2. 2.
    Attenhofer Jost CH, Connolly HM, Burkhart HM, et al. Tetralogy of Fallot repair in patients 40 years or older. Mayo Clin Proc. 2010;85:1090–4.CrossRefGoogle Scholar
  3. 3.
    Spencer KT, Weinert L, Lang RM. Effect of age, heart rate and tricuspid regurgitation on the Doppler echocardiography evaluation of right ventricular diastolic function. Cardiology. 1999;92:59–64. PubMed CrossRef.CrossRefGoogle Scholar
  4. 4.
    Cicala S, Galderisi M, Caso P, et al. Right ventricular diastolic dysfunction in arterial systemic hypertension: analysis by pulsed tissue Doppler. Eur J Echocardiogr. 2002;3:135–42. PubMed CrossRef.CrossRefGoogle Scholar
  5. 5.
    Nomura T, Lebowitz L, Koide Y, et al. Evaluation of hepatic venous flow using transesophageal echocardiography in coronary artery bypass surgery: an index of right ventricular function. J Cardiothorac Vasc Anesth. 1995;9:9–17. PubMed CrossRef.CrossRefGoogle Scholar
  6. 6.
    Gatzoulis MA, Clark AL, Cullen S, et al. Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot: restrictive physiology predicts superior exercise performance. Circulation. 1995;91:1775–81. PubMed CrossRef.CrossRefGoogle Scholar
  7. 7.
    Bacha EA, Scheule AM, Zurakowski D, et al. Long-term results after early primary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 2001;122:154–61.CrossRefGoogle Scholar
  8. 8.
    Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet. 2009;374:1462–71.CrossRefGoogle Scholar
  9. 9.
    Friedberg MK, Redington AN. Right versus left ventricular failure differences, similarities, and interactions. Circulation. 2014;129:1033–44.CrossRefGoogle Scholar
  10. 10.
    Alonso-González R, Dimopoulos K, Ho SY, Oliver JM, Gatzoulis MA. The right heart in adults with congenital heart disease. Rev Esp Cardiol. 2010;63(9):1070–86.CrossRefGoogle Scholar
  11. 11.
    Lucy Roche S, Redington AN. The failing right ventricle in congenital heart disease. Can J Cardiol. 2013;29:768–78.CrossRefGoogle Scholar
  12. 12.
    Villafañe J, et al. Hot topics in Tetralogy of Fallot. J Am Coll Cardiol. 2013;62(23):2155–66.CrossRefGoogle Scholar
  13. 13.
    Mehta RH, et al. Levosimendan in patients with left ventricular dysfunction undergoing cardiac surgery. New Engl J Med. 2017;376:2032–42.  https://doi.org/10.1056/NEJMoa1616218.CrossRefPubMedGoogle Scholar
  14. 14.
    Landoni G, et al. Levosimendan for hemodynamic support after cardiac surgery. New Engl J Med. 2017;376:2021–31.  https://doi.org/10.1056/NEJMoa1616325.CrossRefPubMedGoogle Scholar
  15. 15.
    Dos L, et al. Pulmonary valve replacement in repaired Tetralogy of Fallot: determinants of early postoperative adverse outcomes. J Thorac Cardiovasc Surg. 2009;138:553–9.CrossRefGoogle Scholar
  16. 16.
    Acheampong B, et al. Postcardiotomy ECMO support after high-risk operations in adult congenital heart disease. Congenit Heart Dis. 2016;11:751–5.CrossRefGoogle Scholar
  17. 17.
    Morgan C, et al. Chronic kidney disease in congenital heart disease patients: a narrative review of evidence. Can J Kidney Health Dis. 2015;2:27.CrossRefGoogle Scholar
  18. 18.
    Allan CK. Intensive care of the adult patient with congenital heart disease. Prog Cardiovasc Dis. 2011;53:274–80.CrossRefGoogle Scholar
  19. 19.
    Ranucci M. Outcome measures and quality markers for perioperative blood loss and transfusion in cardiac surgery. Can J Anaesth. 2016;63(2):169–75.CrossRefGoogle Scholar
  20. 20.
    Page RL, et al. 2015 ACC/AHA/HRS guideline for the management of adult patients with supraventricular tachycardia. A report of the American College Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2016;133:e506–74.PubMedGoogle Scholar
  21. 21.
    Giamberti A, et al. Surgery for supraventricular tachycardia and congenital heart defects: long-term efficacy of the combined approach in adult patients. Europace. 2016;19:1542–8.Google Scholar
  22. 22.
    Warnes CA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. J Am Coll Cardiol. 2008;52(23):e143–263.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Giuseppe Isgro
    • 1
  • Marco Ranucci
    • 2
  • Massimo Chessa
    • 3
  1. 1.Congenital Cardiac Intensive Care UnitIRCCS Policlinico San DonatoMilanItaly
  2. 2.Department of Cardiothoracic and Vascular Anesthesia and Intensive CareIRCCS Policlinico San DonatoMilanItaly
  3. 3.Pediatric and Adult Congenital Heart CenterIRCCS Policlinico San DonatoMilanItaly

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