Hostname: page-component-78c5997874-m6dg7 Total loading time: 0 Render date: 2024-11-10T08:33:47.143Z Has data issue: false hasContentIssue false

Management of adults with Tetralogy of Fallot

Published online by Cambridge University Press:  09 January 2014

Richard M. Martinez*
Affiliation:
Johns Hopkins All Children's Heart Institute, Department of Pediatrics, University of South Florida, Pediatric Cardiology Associates/Pediatrix Medical Group, All Children's Hospital, United States of America
Jeremy M. Ringewald
Affiliation:
Johns Hopkins All Children's Heart Institute, The Congenital Heart Institute of Florida, All Children' Hospital, St. Josephs Children's Hospital of Tampa, Pediatric Cardiology Associates/Pediatrix Medical Group, United States of America
Hector L. Fontanet
Affiliation:
The Congenital Heart Institute of Florida, All Children's Hospital, Children's Hospital of Tampa, Florida Medical Clinic, University of South Florida, Saint Petersburg and Tampa, Florida, United States of America
James A. Quintessenza
Affiliation:
Johns Hopkins All Children's Heart Institute, Johns Hopkins Children's Heart Surgery, All Children's Hospital and Florida Hospital for Children, United States of America
Jeffrey P. Jacobs
Affiliation:
Johns Hopkins All Children's Heart Institute, Johns Hopkins University, Saint Petersburg, Tampa, and Orlando, Florida, United States of America
*
Correspondence to: Richard M. Martinez, MD, Johns Hopkins All Children's Heart Institute, Saint Petersburg, Florida, United States of America. Tel: (727) 434-4486; E-mail: rmarti9209@aol.com

Abstract

Tetralogy of Fallot is the most common form of cyanotic congenital cardiac disease. Patients with previously repaired tetralogy of Fallot are the most common patients seen in the Program for Adults with Congenital Heart Disease at The Johns Hopkins All Children's Heart Institute. Guidelines for the management of these patients are available from multiple sources including The American College of Cardiology (ACC) and The American Heart Association (AHA), The Canadian Cardiovascular Society, and The European Society of Cardiology (ESC). These guidelines describe multiple components related to the care for these patients including strategies for medical follow-up, the management of arrhythmias and electrophysiological diseases, and the treatment of chronic pulmonary insufficiency and stenosis. Several new strategies are available for replacement of the pulmonary valve including transcatheter replacement of the pulmonary valve and replacement of the pulmonary valve with a self-manufactured bicuspid polytetrafluoroethylene pulmonary valve.

Type
Original Article
Copyright
Copyright © Cambridge University Press 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Huehnergarth, KV, Gurvitz, M, Stout, KK, et al. Repaired Tetralogy of Fallot in the adult: monitoring and management. Heart 2008; 94: 16631669.CrossRefGoogle ScholarPubMed
2.Apitz, C, Webb, GD, Redington, AN. Tetralogy of Fallot. Lancet 2009; 374: 14621471.CrossRefGoogle ScholarPubMed
3.Apitz, C, webb, GD, Reding, AN. Tetralogy of Fallot. Lancet 2009; 374: 14621471.CrossRefGoogle ScholarPubMed
4. The adult Tetralogy of Fallot patient. 30 April 2012. BCS Editorial. By Ee Ling Heng. London Deanery. Retrieved November 28, 2013, from http://www.bcs.com/pages/news_full.asp?NewsID=19792049Google Scholar
5.Dearani, Joseph A, Connolly, Heidi M, Martinez, Richard, Fontanet, Hector, Webb, Gary D. Carding for adults with congenital cardiac disease: successes and challenges for 2007 and beyond. Cardiol Young 2007; 17 (Suppl 2): 8796.Google Scholar
6. Thumbnail Guide to Congenital Heart Disease. This website was designed and written by Dr Richard Kirk MA MB BChir FRCP FRCPCH Consultant Paediatric Cardiologist at the Freeman Hospital, Newcastle upon Tyne, UK. Retrieved November 28, 2013, from http://www.crkirk.com/thumbnail/Google Scholar
7. The Society of Thoracic Surgeons: What is Pediatric Heart Disease? Written by Jeffrey P. Jacobs, MD and Heidi Hess, RN – Reviewed September 2012. Illustration by Steven P. Goldberg, MD. Retrieved November 28, 2013, from http://www.sts.org/patient-information/what-pediatric-heart-disease/tetralogy-fallot-tofGoogle Scholar
8.Baumgartner, H, Bonhoeffer, P, De Groot, NM, de Haan, F, Deanfield, JE, Galie, N, Gatzoulis, MA, Gohlke-Baerwolf, C, Kaemmerer, H, Kilner, P, Meijboom, F, Mulder, BJ, Oechslin, E, Oliver, JM, Serraf, A, Szatmari, A, Thaulow, E, Vouhe, PR, Walma, E; Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC); Association for European Paediatric Cardiology (AEPC); ESC Committee for Practice Guidelines (CPG). ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31: 29152957.Google Scholar
9.Gatzoulis, MA, Till, JA, Somerville, J, Redington, AN. Mechanoelectrical interaction in Tetralogy of Fallot. QRS prolongation relates to right ventricular size and predict malignant ventricular arrhythmias and sudden death. Circulation 1995; 92: 231237.CrossRefGoogle ScholarPubMed
10.Gatzoulis, MA, Balaji, S, Webber, SA, et al. Risk factors for arrhythmia and sudden death cardiac death later after repair of Tetralogy of Fallot: a multicentre study. Lancet 2000; 356: 975981.Google Scholar
11.Apits, C, Webb, GD, Redington, AN. Tetralogy of Fallot. Lancet 2009; 374: 14621471.CrossRefGoogle Scholar
12.Bonhoeffer, P, Boudjemline, Y, Qureshi, SA, et al. Percutaneous insertion of the pulmonary valve. J Am Coll Cardio 2002; 39: 16641669.Google Scholar
13.Silversides, CK, Marelli, A, Beauchesen, L, et al.Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: executive summary. Can J Cardiol 2010; 26: 143150.CrossRefGoogle Scholar
14.Naguib, MA, Dob, DP, Gatzoulis, MA. A functional understanding of moderate to complex congenital heart disease and the impact of pregnancy. Part II: Tetralogy of Fallot. Eisenmenger's syndrome and the Fontan operation. Int J Obstet Apesth 2010; 19: 306312.CrossRefGoogle ScholarPubMed
15. Ammash N. Aging with Tetralogy of Fallot, ACHA Webinsar, 2011.Google Scholar
16. Pub Med Central, Heart. 2006, 92: 1353–1359.Google Scholar
17. RadioGraphics. Retrieved November 28, 2013, from http://pubs.rsna.org/journal/radiographicsGoogle Scholar
18.Boxt, LM, Lipton, MJ, Kwong, RY, Rybicki, F, Clouse, ME. Computed tomography for assessment of cardiac chambers, valves, myocardium and pericardium. Cardiol Clin 2003; 21: 561585.CrossRefGoogle ScholarPubMed
19.Apitz, C, Webb, G, Reddington, A. Tetralogy of Fallot. Lancet 2009; 374: 14621471.Google Scholar
20.Therrien, J, Marx, GR, Gatzoulis, MA. Late problems in Tetralogy of Fallot – recognition, management, and prevention. Cardiol Clin 2002; 20: 395404.CrossRefGoogle ScholarPubMed
21.Geva, T, Sandweiss, BM, Gauvreau, K, Lock, JE, Powell, AJ. Factors associated with impaired clinical status in long-term survivors of Tetralogy of Fallot repair evaluated by magnetic resonance imaging. J Am Coll Cardiol 2004; 43: 10681074.Google Scholar
22.Broberg, CS, Aboulhosn, J, Mongeon, FP, et al. Prevalence of left ventricular systolic dysfunction in adults with repaired Tetralogy of Fallot. Am J Cardiol 2011; 107: 12151220.Google Scholar
23.Diller, GP, Kempny, A, Liodakis, E, et al. Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired Tetralogy of Fallot. Circulation 2012; 125: 24402446.Google Scholar
24.Niwa, K, Siu, SC, Webb, GD, Gatzoulis, MA. Progressive aortic root dilatation in adults late after repair of Tetralogy of Fallot. Circulation 2002; 106: 13741378.Google Scholar
25.Mongeon, FP, Gurvitz, MZ, Broberg, CS, et al. Aortic root dilatation in adults with surgically repaired Tetralogy of Fallot: a multicenter cross-sectional study. Circulation 2013; 127: 172179.CrossRefGoogle ScholarPubMed
26.Rudski, LG, Lai, WW, Afilalo, J, Hua, L, Handschumacher, MD, Chandrasekaran, K, Solomon, SD, Louie, EK, Schiller, NB. Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 2010; 23: 685713.CrossRefGoogle Scholar
27.Shors, SM, Fung, CW, Finn, PJ, Fieno, D. Accurate quantification of right ventricular mass at MR imaging by using cine true fast imaging with steady-state precession: study in dogs. Radiology 2004; 230: 383388.Google Scholar
28.Grothues, F, Moon, JC, Bellenger, NG, et al. Interstudy reproducibility of right ventricular volumes, function, and mass with cardiovascular magnetic resonance. Am Heart J 2004; 43: 10681074.Google Scholar
29.Lee, C, Kim, YM, Lee, C-H, et al. Outcomes of pulmonary valve replacement in 170 patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction: implications for optimal timing of pulmonary valve replacement. J Am Coll Cardiol 2012; 60: 10051014.CrossRefGoogle ScholarPubMed
30.Holmes, K. Timing of pulmonary valve replacement in Tetralogy of Fallot using cardiac magnetic resonance imaging J Am Coll Cardiol 2012; 60: 10151017.Google Scholar
31.Raman, SV, Cook, SC, McCarthy, B, Ferketich, AK. Usefulness of multidetector row computed tomography to quantify right ventricular size and function in adults with either Tetralogy of Fallot or transposition of the great arteries. Am J Cardiol 2005; 95: 683686.CrossRefGoogle ScholarPubMed
32.American College of Cardiology Foundation Task Force on Expert Consensus Documents, Mark, DB, Berman, DS, Budoff, MJ, Carr, JJ, Gerber, TC, Hecht, HS, Hlatky, MA, Hodgson, JM, Lauer, MS, Miller, JM, Morin, RL, Mukherjee, D, Poon, M, Rubin, GD, Schwartz, RS. ACCF/ACR/AHA/NASCI/SAIP/SCAI/SCCT 2010 expert consensus document on coronary computed tomographic angiography: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents. Circulation 2010; 121: 25092543.Google Scholar
33.Giardini, A, Specchia, S, Coutsoumbas, G, Donti, A, Formigari, R, Fattori, R, Oppido, G, Gargiulo, G, Picchio, FM. Impact of pulmonary regurgitation and right ventricular dysfunction on oxygen uptake recovery kinetics in repaired Tetralogy of Fallot. Eur J Heart Fail 2006; 8: 736743.CrossRefGoogle ScholarPubMed
34.Gatzoulis, MA, Balaji, S, Webber, SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of Tetralogy of Fallot: a multicentre study. Lancet 2000; 356: 975981.CrossRefGoogle ScholarPubMed
35.Khairy, P, Aboulhosn, J, Gurvitz, MZ, Opotowsky, AR, Mongeon, FP, Kay, J, Valente, AM, Earing, MG, Lui, G, Gersony, DR, Cook, S, Ting, JG, Nickolaus, MJ, Webb, G, Landzberg, MJ, Broberg, CS; Alliance for Adult Research in Congenital Cardiology (AARCC). Arrhythmia burden in adults with surgically repaired Tetralogy of Fallot: a multi-institutional study. Circulation 2010; 122: 868875.Google Scholar
36.Bricker, JT. Sudden death and Tetralogy of Fallot, risks, markers, and causes. Circulation 1995; 92: 158159.Google Scholar
37.Bouzas, B, Kilner, PJ, Gatzoulis, MA. Pulmonary regurgitation: not a benign lesion Eur Heart J 2005; 26: 433439.Google Scholar
38.Frigiola, A, Redington, AN, Cullen, S. Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired Tetralogy of Fallot. Circulation 2004; 110 (Suppl 1): 153157.CrossRefGoogle ScholarPubMed
39.Ammash, NM, Dearani, JA, Burkhart, HM, et al. Pulmonary regurgitation after Tetralogy of Fallot: clinical features, sequelae, and timing of pulmonary valve replacement. Cong Heart Dis 2007; 2: 386403.Google Scholar
40.Davroulos, PA, Karatza, AA, Gatzoulis, MA, Shore, DF. Timing and type of surgery for severe pulmonary regurgitation late after repair of Tetralogy of Fallot. Int J Cardiol 2004; 97: 91101.Google Scholar
41.Bove, EL, Kavey, RE, Byrum, CJ, et al. Improved right ventricular function following late pulmonary valve replacement for residual pulmonary insufficiency or stenosis. J Thorac Cardiovasc Surg 1985; 90: 5055.Google Scholar
42.Therrien, J, Siu, SC, McLaughlin, PR, Liu, PP, Williams, WG, Webb, GD. Pulmonary valve replacement in adults late after repair of Tetralogy of Fallot: are we operating too late? J Am Coll Cardiol 2000; 36: 16701675.Google Scholar
43.Therrien, J, Siu, SC, Harris, L, et al. Impact of pulmonary valve replacement on arrhythmia propensity late after repair of Tetralogy of Fallot. Circulation 2001; 103: 24892494.Google Scholar
44.Hawkins, JA, Sower, CT, Lambert, LM, et al. Stentless porcine valves in the right ventricular outflow tract: improved durability. Eur J Cardiothorac Surg 2009; 35: 600604; discussion 604–605.Google Scholar
45.Kanter, KR, Budde, JM, Parks, WJ, et al. One hundred pulmonary valve replacements in children after relief of right ventricular outflow tract obstruction. Ann Thorac Surg 2002; 73: 18011806; discussion 1806–1807.Google Scholar
46.Fiore, AC, Rodefeld, M, Turrentine, M, et al. Pulmonary valve replacement: comparison of three biologic valves. Ann Thorac Surg 2008; 85: 17121718.Google Scholar
47.Hawkins, JA, Breinholt, JP, Lambert, LM, et al. Class I and class II anti-HLA antibodies after implantation of cryopreserved allograft material in pediatric patients. J Thorac Cardiovasc Surg 2000; 119: 324330.Google Scholar
48.Waterbolk, TW, Hoendermis, ES, den Hamer, IJ, Ebels, T. Pulmonary valve replacement with a mechanical prosthesis. Promising results of 28 procedures in patients with congenital heart disease. Eur J Cardiothorac Surg 2006; 30: 2832.CrossRefGoogle ScholarPubMed
49.Quintessenza, J, Jacobs, JP, Morell, VO, et al. Initial experience with a bicuspid PTFE pulmonary valve in 41 children: a new option for right ventricular outflow tract reconstruction. Ann Thorac Surg 2005; 79: 924931.CrossRefGoogle ScholarPubMed
50.Quintessenza, JA, Jacobs, JP, Chai, PJ, Morell, VO, Lindberg, H. Polytetrafluoroethylene bicuspid pulmonary valve implantation: experience with 126 patients. World J Pediatr Congenit Heart Surg 2010; 1: 2027.Google Scholar
51.Frigiola, A, Nordmeyer, J, Bonhoeffer, P, et al. Percutaneous pulmonary valve replacement. Coron Artery Dis 2009; 20: 189191.Google Scholar
52.Toshiharu, S, Dominique, ST, Peter, X. Creation of viable pulmonary artery autografts through tissue engineering. J Thorac Cardiovasc Surg 1998; 115: 536546.Google Scholar