The Journal of Heart and Lung Transplantation
Volume 23, Issue 12 , Pages 1313-1333 , December 2004

International Society for Heart and Lung Transplantation: Practice guidelines for management of heart failure in children

  • David Rosenthal, MD (Chair)

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
    • Corresponding Author InformationReprint requests: David Rosenthal, MD, (W) 725 Welch Road, room 1751, Stanford, California 94304, Telephone: 650 497-8676, Fax: 650 497-8422.
  • ,
  • Maryanne R.K. Chrisant, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Erik Edens, MD, PhD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Lynn Mahony, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Charles Canter, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Steven Colan, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Anne Dubin, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Jacque Lamour, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Robert Ross, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Robert Shaddy, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Linda Addonizio, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Lee Beerman, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Stuart Berger, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Daniel Bernstein, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Elizabeth Blume, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Mark Boucek, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Paul Checchia, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Anne Dipchand, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Jonathan Drummond-Webb, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Jay Fricker, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Richard Friedman, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Sara Hallowell, RN

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Robert Jaquiss, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Seema Mital, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Elfriede Pahl, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Bennett Pearce, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Larry Rhodes, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Kathy Rotondo, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Paolo Rusconi, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Janet Scheel, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Tajinder Pal Singh, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas
  • ,
  • Jeffrey Towbin, MD

      Affiliations

    • International Society for Heart and Lung Transplantation, Addison, Texas

References 

  1. Lipshultz SE , Sleeper LA , Towbin JA , et al.   The incidence of pediatric cardiomyopathy in two regions of the United States . N Engl J Med . 2003;348:1647–1655
  2. Boucek MM , Edwards LB , Keck BM , et al.   The Registry of the International Society for Heart and Lung Transplantation (Sixth Official Pediatric Report–2003) . J Heart Lung Transplant . 2003;22:636–652
  3. Heart Failure Society of America (HFSA) practice guidelines . HFSA guidelines for management of patients with heart failure caused by left ventricular systolic dysfunction-pharmacological approaches . J Card Fail . 1999;5:357–382
  4. Katz AM . Heart Failure Pathophysiology, Molecular Biology and Clinical Management . Philadelphia, Pa: Lippincott, Williams & Wilkins; 2000;
  5. Ross RD , Daniels SR , Schwartz DC , Hannon DW , Shukla R , Kaplan S . Plasma norepinephrine levels in infants and children with congestive heart failure . Am J Cardiol . 1987;59:911–914
  6. Johnstone DE , Abdulla A , Arnold JM , et al.   Diagnosis and management of heart failure. Canadian Cardiovascular Society . Can J Cardiol . 1994;10:613–631 35–54
  7. Wu JR , Chang HR , Huang TY , Chiang CH , Chen SS . Reduction in lymphocyte beta-adrenergic receptor density in infants and children with heart failure secondary to congenital heart disease . Am J Cardiol . 1996;77:170–174
  8. Ross RD , Bollinger RO , Pinsky WW . Grading the severity of congestive heart failure in infants . Pediatr Cardiol . 1992;13:72–75
  9. Connolly D , Rutkowski M , Auslender M , Artman M . The New York University Pediatric Heart Failure Index (a new method of quantifying chronic heart failure severity in children) . J Pediatr . 2001;138:644–648
  10. Ross RD . Grading the graders of congestive heart failure in children . J Pediatr . 2001;138:618–620
  11. Ohuchi H , Takasugi H , Ohashi H , et al.   Stratification of pediatric heart failure on the basis of neurohormonal and cardiac autonomic nervous activities in patients with congenital heart disease . Circulation . 2003;108:2368–2376
  12. Hunt SA , Baker DW , Chin MH , et al.   ACC/AHA guidelines for the evaluation and management of chronic heart failure in the adult: executive summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to revise the 1995 Guidelines for the Evaluation and Management of Heart Failure) . J Am Coll Cardiol . 2001;38:2101–2113
  13. Bonnet D , de Lonlay P , Gautier I , et al.   Efficiency of metabolic screening in childhood cardiomyopathies . Eur Heart J . 1998;19:790–793
  14. Schwartz ML , Cox GF , Lin AE , et al.   Clinical approach to genetic cardiomyopathy in children . Circulation . 1996;94:2021–2038
  15. Kohlschutter A , Hausdorf G . Primary (genetic) cardiomyopathies in infancy. A survey of possible disorders and guidelines for diagnosis . Eur J Pediatr . 1986;145:454–459
  16. Krishnan US , Gersony WM , Berman-Rosenzweig E , Apfel HD . Late left ventricular function after surgery for children with chronic symptomatic mitral regurgitation . Circulation . 1997;96:4280–4285
  17. Skudicky D , Essop MR , Sareli P . Time-related changes in left ventricular function after double valve replacement for combined aortic and mitral regurgitation in a young rheumatic population. Predictors of postoperative left ventricular performance and role of chordal preservation . Circulation . 1997;95:899–904
  18. Packer M , Gheorghiade M , Young JB , et al.   Withdrawal of digoxin from patients with chronic heart failure treated with angiotensin-converting-enzyme inhibitors. RADIANCE Study . N Engl J Med . 1993;329:1–7
  19. Adams KF , Gheorghiade M , Uretsky BF , Patterson JH , Schwartz TA , Young JB . Clinical benefits of low serum digoxin concentrations in heart failure . J Am Coll Cardiol . 2002;39:946–953
  20. Rathore SS , Curtis JP , Wang Y , Bristow MR , Krumholz HM . Association of serum digoxin concentration and outcomes in patients with heart failure . Jama . 2003;289:871–878
  21. Pitt B , Zannad F , Remme WJ , et al.   The effect of spironolactone on morbidity and mortality in patients with severe heart failure. Randomized Aldactone Evaluation Study Investigators . N Engl J Med . 1999;341:709–717
  22. Pitt B , Remme W , Zannad F , et al.   Eplerenone, a selective aldosterone blocker, in patients with left ventricular dysfunction after myocardial infarction . N Engl J Med . 2003;348:1309–1321
  23. Brunner-La Rocca HP , Vaddadi G , Esler MD . Recent insight into therapy of congestive heart failure (focus on ACE inhibition and angiotensin-II antagonism) . J Am Coll Cardiol . 1999;33:1163–1173
  24. Braunwald E , Bristow MR . Congestive heart failure (fifty years of progress) . Circulation . 2000;102:IV14–IV23
  25. Swedberg K , Eneroth P , Kjekshus J , Wilhelmsen L  CONSENSUS Trial Study Group . Hormones regulating cardiovascular function in patients with severe congestive heart failure and their relation to mortality . Circulation . 1990;82:1730–1736
  26. Gilbert EM , Sandoval A , Larrabee P , Renlund DG , O’Connell JB , Bristow MR . Lisinopril lowers cardiac adrenergic drive and increases beta-receptor density in the failing human heart . Circulation . 1993;88:472–480
  27. The CONSENSUS Trial Study Group . Effects of enalapril on mortality in severe congestive heart failure. Results of the Cooperative North Scandinavian Enalapril Survival Study (CONSENSUS) . N Engl J Med . 1987;316:1429–1435
  28. Cohn JN , Johnson G , Ziesche S , et al.   A comparison of enalapril with hydralazine-isosorbide dinitrate in the treatment of chronic congestive heart failure . N Engl J Med . 1991;325:303–310
  29. Effect of enalapril on survival in patients with reduced left ventricular ejection fractions and congestive heart failure. The SOLVD Investigators . N Engl J Med . 1991;325:293–302
  30. Effect of enalapril on mortality and the development of heart failure in asymptomatic patients with reduced left ventricular ejection fractions. The SOLVD Investigattors . N Engl J Med . 1992;327:685–691
  31. Packer M . Do angiotensin-converting enzyme inhibitors prolong life in patients with heart failure treated in clinical practice . J Am Coll Cardiol . 1996;28:1323–1327
  32. Uretsky BF , Shaver JA , Liang CS , et al.   Modulation of hemodynamic effects with a converting enzyme inhibitor (acute hemodynamic dose-response relationship of a new angiotensin converting enzyme inhibitor, lisinopril, with observations on long-term clinical, functional, and biochemical responses) . Am Heart J . 1988;116:480–488
  33. Pacher R , Globits S , Bergler-Klein J , et al.   Clinical and neurohumoral response of patients with severe congestive heart failure treated with two different captopril dosages . Eur Heart J . 1993;14:273–278
  34. Riegger GA . Effects of quinapril on exercise tolerance in patients with mild to moderate heart failure . Eur Heart J . 1991;12:705–711
  35. Packer M , Poole-Wilson PA , Armstrong PW , et al.  ATLAS Study Group   Comparative effects of low and high doses of the angiotensin-converting enzyme inhibitor, lisinopril, on morbidity and mortality in chronic heart failure . Circulation . 1999;100:2312–2318
  36. Lin M , Chiang HT , Lin SL , et al.   Vasodilator therapy in chronic asymptomatic aortic regurgitation (enalapril versus hydralazine therapy) . J Am Coll Cardiol . 1994;24:1046–1053
  37. Pitt B , Konstam MA . Overview of angiotensin II-receptor antagonists . Am J Cardiol . 1998;82:47S–49S
  38. Pitt B , Segal R , Martinez FA , et al.   Randomised trial of losartan versus captopril in patients over 65 with heart failure (Evaluation of Losartan in the Elderly Study, ELITE) . Lancet . 1997;349:747–752
  39. Pitt B , Poole-Wilson P , Segal R , et al.   Effects of losartan versus captopril on mortality in patients with symptomatic heart failure (rationale, design, and baseline characteristics of patients in the Losartan Heart Failure Survival Study—ELITE II) . J Card Fail . 1999;5:146–154
  40. Hamroff G , Katz SD , Mancini D , et al.   Addition of angiotensin II receptor blockade to maximal angiotensin-converting enzyme inhibition improves exercise capacity in patients with severe congestive heart failure . Circulation . 1999;99:990–992
  41. Bengur AR , Beekman RH , Rocchini AP , Crowley DC , Schork MA , Rosenthal A . Acute hemodynamic effects of captopril in children with a congestive or restrictive cardiomyopathy . Circulation . 1991;83:523–527
  42. Leversha AM , Wilson NJ , Clarkson PM , Calder AL , Ramage MC , Neutze JM . Efficacy and dosage of enalapril in congenital and acquired heart disease . Arch Dis Child . 1994;70:35–39
  43. Seguchi M , Nakazawa M , Momma K . Effect of enalapril on infants and children with congestive heart failure . Cardiol Young . 1992;2:14–19
  44. Stern H , Weil J , Genz T , Vogt W , Buhlmeyer K . Captopril in children with dilated cardiomyopathy (acute and long-term effects in a prospective study of hemodynamic and hormonal effects) . Pediatr Cardiol . 1990;11:22–28
  45. Schiffman H , Gawlik L , Wessel A . Treatment effects of captopril in neonates, infants and young children with congestive heart failure . Z Kardiol . 1996;85:709
  46. Lewis AB , Chabot M . The effect of treatment with angiotensin-converting enzyme inhibitors on survival of pediatric patients with dilated cardiomyopathy . Pediatr Cardiol . 1993;14:9–12
  47. Mori Y , Nakazawa M , Tomimatsu H , Momma K . Long-term effect of angiotensin-converting enzyme inhibitor in volume overloaded heart during growth (a controlled pilot study) . J Am Coll Cardiol . 2000;36:270–275
  48. Marino MR , Vachharajani NN . Pharmacokinetics of irbesartan are not altered in special populations . J Cardiovasc Pharmacol . 2002;40:112–122
  49. Smith KM , Macmillan JB , McGrath JC . Investigation of alpha1-adrenoceptor subtypes mediating vasoconstriction in rabbit cutaneous resistance arteries . Br J Pharmacol . 1997;122:825–832
  50. Elhawary AM , Pang CC . Alpha 1b-adrenoceptors mediate renal tubular sodium and water reabsorption in the rat . Br J Pharmacol . 1994;111:819–824
  51. Communal C , Singh K , Pimentel DR , Colucci WS . Norepinephrine stimulates apoptosis in adult rat ventricular myocytes by activation of the beta-adrenergic pathway . Circulation . 1998;98:1329–1334
  52. Packer M . Current role of beta-adrenergic blockers in the management of chronic heart failure . Am J Med . 2001;110(Suppl 7A):81S–94S
  53. Bruns LA , Chrisant MK , Lamour JM , et al.   Carvedilol as therapy in pediatric heart failure (an initial multicenter experience) . J Pediatr . 2001;138:505–511
  54. Rusconi P , Gomez-Marin O , Rossique-Gonzalez M , et al.   Carvedilol in children with cardiomyopathy (3 years experience in a single institution) . J Heart Lung Transplant . 2003; in press
  55. Packer M , Bristow MR , Cohn JN , et al.  U.S. Carvedilol Heart Failure Study Group   The effect of carvedilol on morbidity and mortality in patients with chronic heart failure . N Engl J Med . 1996;334:1349–1355
  56. Waagstein F , Caidahl K , Wallentin I , Bergh CH , Hjalmarson A . Long-term beta-blockade in dilated cardiomyopathy. Effects of short- and long-term metoprolol treatment followed by withdrawal and readministration of metoprolol . Circulation . 1989;80:551–563
  57. Epstein SE , Braunwald E . The effect of beta adrenergic blockade on patterns of urinary sodium excretion. Studies in normal subjects and in patients with heart disease . Ann Intern Med . 1966;65:20–27
  58. Weil JV , Chidsey CA . Plasma volume expansion resulting from interference with adrenergic function in normal man . Circulation . 1968;37:54–61
  59. Waagstein F , Bristow MR , Swedberg K , et al.  Metoprolol in Dilated Cardiomyopathy (MDC) Trial Study Group   Beneficial effects of metoprolol in idiopathic dilated cardiomyopathy . Lancet . 1993;342:1441–1446
  60. Effects of metoprolol CR in patients with ischemic and dilated cardiomyopathy (the randomized evaluation of strategies for left ventricular dysfunction pilot study) . Circulation . 2000;101:378–384
  61. Hjalmarson A , Goldstein S , Fagerberg B , et al.  MERIT-HF Study Group   Effects of controlled-release metoprolol on total mortality, hospitalizations, and well-being in patients with heart failure (the Metoprolol CR/XL Randomized Intervention Trial in congestive heart failure (MERIT-HF)) . Jama . 2000;283:1295–1302
  62. Colucci WS , Packer M , Bristow MR , et al.  US Carvedilol Heart Failure Study Group   Carvedilol inhibits clinical progression in patients with mild symptoms of heart failure . Circulation . 1996;94:2800–2806
  63. Packer M , Colucci WS , Sackner-Bernstein JD , et al.   Double-blind, placebo-controlled study of the effects of carvedilol in patients with moderate to severe heart failure. The PRECISE Trial. Prospective Randomized Evaluation of Carvedilol on Symptoms and Exercise . Circulation . 1996;94:2793–2799
  64. Bristow MR , Gilbert EM , Abraham WT , et al.   Carvedilol produces dose-related improvements in left ventricular function and survival in subjects with chronic heart failure. MOCHA Investigators . Circulation . 1996;94:2807–2816
  65. Packer M , Coats AJ , Fowler MB , et al.   Effect of carvedilol on survival in severe chronic heart failure . N Engl J Med . 2001;344:1651–1658
  66. Shaddy RE , Tani LY , Gidding SS , et al.   Beta-blocker treatment of dilated cardiomyopathy with congestive heart failure in children (a multi-institutional experience) . J Heart Lung Transplant . 1999;18:269–274
  67. Azeka E , Franchini Ramires JA , Valler C , Alcides Bocchi E . Delisting of infants and children from the heart transplantation waiting list after carvedilol treatment . J Am Coll Cardiol . 2002;40:2034–2038
  68. Grossman W . Diastolic dysfunction in congestive heart failure . N Engl J Med . 1991;325:1557–1564
  69. Grossman W . Defining diastolic dysfunction . Circulation . 2000;101:2020–2021
  70. Sweitzer NK , Stevenson LW . Diastolic heart failure (miles to go before we sleep) . Am J Med . 2000;109:683–685
  71. Vasan RS , Benjamin EJ , Levy D . Prevalence, clinical features and prognosis of diastolic heart failure (an epidemiologic perspective) . J Am Coll Cardiol . 1995;26:1565–1574
  72. Vasan RS , Levy D . Defining diastolic heart failure (a call for standardized diagnostic criteria) . Circulation . 2000;101:2118–2121
  73. Tardif JC , Rouleau JL . Diastolic dysfunction . Can J Cardiol . 1996;12:389–398
  74. Guidelines for the evaluation and management of heart failure. Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Evaluation and Management of Heart Failure) . Circulation . 1995;92:2764–2784
  75. Lewis AB . Clinical profile and outcome of restrictive cardiomyopathy in children . Am Heart J . 1992;123:1589–1593
  76. Rivenes SM , Kearney DL , Smith EO , Towbin JA , Denfield SW . Sudden death and cardiovascular collapse in children with restrictive cardiomyopathy . Circulation . 2000;102:876–882
  77. Kushwaha SS , Fallon JT , Fuster V . Restrictive cardiomyopathy . N Engl J Med . 1997;336:267–276
  78. Chen SC , Balfour IC , Jureidini S . Clinical spectrum of restrictive cardiomyopathy in children . J Heart Lung Transplant . 2001;20:90–92
  79. Lee RT , Lord CP , Plappert T , Sutton MS . Effects of nifedipine on transmitral Doppler blood flow velocity profile in patients with concentric left ventricular hypertrophy . Am Heart J . 1990;119:1130–1136
  80. Lorell BH , Grossman W . Cardiac hypertrophy (the consequences for diastole) . J Am Coll Cardiol . 1987;9:1189–1193
  81. Devereux RB , Palmieri V , Sharpe N , et al.   Effects of once-daily angiotensin-converting enzyme inhibition and calcium channel blockade-based antihypertensive treatment regimens on left ventricular hypertrophy and diastolic filling in hypertension (the prospective randomized enalapril study evaluating regression of ventricular enlargement (preserve) trial) . Circulation . 2001;104:1248–1254
  82. Warner JG , Metzger DC , Kitzman DW , Wesley DJ , Little WC . Losartan improves exercise tolerance in patients with diastolic dysfunction and a hypertensive response to exercise . J Am Coll Cardiol . 1999;33:1567–1572
  83. Paulus WJ , Vantrimpont PJ , Shah AM . Acute effects of nitric oxide on left ventricular relaxation and diastolic distensibility in humans. Assessment by bicoronary sodium nitroprusside infusion . Circulation . 1994;89:2070–2078
  84. Redington AN , Rigby ML , Shinebourne EA , Oldershaw PJ . Changes in the pressure-volume relation of the right ventricle when its loading conditions are modified . Br Heart J . 1990;63:45–49
  85. Hagler DJ , Ritter DG , Mair DD , et al.   Right and left ventricular function after the Mustard procedure in transposition of the great arteries . Am J Cardiol . 1979;44:276–283
  86. Benson LN , Bonet J , McLaughlin P , et al.   Assessment of right ventricular function during supine bicycle exercise after Mustard’s operation . Circulation . 1982;65:1052–1059
  87. Murphy JH , Barlai-Kovach MM , Mathews RA , et al.   Rest and exercise right and left ventricular function late after the Mustard operation (assessment by radionuclide ventriculography) . Am J Cardiol . 1983;51:1520–1526
  88. Mathews RA , Fricker FJ , Beerman LB , et al.   Exercise studies after the Mustard operation in transposition of the great arteries . Am J Cardiol . 1983;51:1526–1529
  89. Ensing GJ , Heise CT , Driscoll DJ . Cardiovascular response to exercise after the Mustard operation for simple and complex transposition of the great vessels . Am J Cardiol . 1988;62:617–622
  90. Paridon SM , Humes RA , Pinsky WW . The role of chronotropic impairment during exercise after the Mustard operation . J Am Coll Cardiol . 1991;17:729–732
  91. Reybrouck T , Gewillig M , Dumoulin M , van der Hauwaert LG . Cardiorespiratory exercise performance after Senning operation for transposition of the great arteries . Br Heart J . 1993;70:175–179
  92. Matthys D , De Wolf D , Verhaaren H . Lack of increase in stroke volume during exercise in asymptomatic adolescents in sinus rhythm after intraatrial repair for simple transposition of the great arteries . Am J Cardiol . 1996;78:595–596
  93. Fogel MA , Weinberg PM , Fellows KE , Hoffman EA . A study in ventricular-ventricular interaction. Single right ventricles compared with systemic right ventricles in a dual-chamber circulation . Circulation . 1995;92:219–230
  94. Millane T , Bernard EJ , Jaeggi E , et al.   Role of ischemia and infarction in late right ventricular dysfunction after atrial repair of transposition of the great arteries . J Am Coll Cardiol . 2000;35:1661–1668
  95. Lubiszewska B , Gosiewska E , Hoffman P , et al.   Myocardial perfusion and function of the systemic right ventricle in patients after atrial switch procedure for complete transposition (long-term follow-up) . J Am Coll Cardiol . 2000;36:1365–1370
  96. Singh TP , Humes RA , Muzik O , Kottamasu S , Karpawich PP , Di Carli MF . Myocardial flow reserve in patients with a systemic right ventricle after atrial switch repair . J Am Coll Cardiol . 2001;37:2120–2125
  97. Graham TP , Bernard YD , Mellen BG , et al.   Long-term outcome in congenitally corrected transposition of the great arteries (a multi-institutional study) . J Am Coll Cardiol . 2000;36:255–261
  98. Imamura M , Drummond-Webb JJ , Murphy DJ , et al.   Results of the double switch operation in the current era . Ann Thorac Surg . 2000;70:100–105
  99. Hechter SJ , Fredriksen PM , Liu P , et al.   Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure . Am J Cardiol . 2001;87:660–663 A11
  100. Sluysmans T , Sanders SP , van der Velde M , et al.   Natural history and patterns of recovery of contractile function in single left ventricle after Fontan operation . Circulation . 1992;86:1753–1761
  101. Tanoue Y , Sese A , Ueno Y , Joh K , Hijii T . Bidirectional Glenn procedure improves the mechanical efficiency of a total cavopulmonary connection in high-risk fontan candidates . Circulation . 2001;103:2176–2180
  102. Berman NB , Kimball TR . Systemic ventricular size and performance before and after bidirectional cavopulmonary anastomosis . J Pediatr . 1993;122:S63–S67
  103. Graham TP , Johns JA . Pre-operative assessment of ventricular function in patients considered for Fontan procedure . Herz . 1992;17:213–219
  104. Matsuda H , Kawashima Y , Kishimoto H , et al.   Problems in the modified Fontan operation for univentricular heart of the right ventricular type . Circulation . 1987;76:III45–III52
  105. Uemura H , Yagihara T , Kawashima Y , et al.   What factors affect ventricular performance after a Fontan-type operation . J Thorac Cardiovasc Surg . 1995;110:405–415
  106. Mahle WT , Coon PD , Wernovsky G , Rychik J . Quantitative echocardiographic assessment of the performance of the functionally single right ventricle after the Fontan operation . Cardiol Young . 2001;11:399–406
  107. Julsrud PR , Weigel TJ , Van Son JA , et al.   Influence of ventricular morphology on outcome after the Fontan procedure . Am J Cardiol . 2000;86:319–323
  108. Hjortdal VE , Stenbog EV , Ravn HB , et al.   Neurohormonal activation late after cavopulmonary connection . Heart . 2000;83:439–443
  109. Mainwaring RD , Lamberti JJ , Moore JW , Billman GF , Nelson JC . Comparison of the hormonal response after bidirectional Glenn and Fontan procedures . Ann Thorac Surg . 1994;57:59–63 discussion 64
  110. Buheitel G , Hofbeck M , Gerling S , Koch A , Singer H . Similarities and differences in the exercise performance of patients after a modified Fontan procedure compared to patients with complete transposition following a Senning operation . Cardiol Young . 2000;10:201–207
  111. Miyairi T , Kawauchi M , Takamoto S , Morizuki O , Furuse A . Oxygen utilization and hemodynamic response during exercise in children after Fontan procedure . Jpn Heart J . 1998;39:659–669
  112. Chua TP , Iserin L , Somerville J , Coats AJ . Effects of chronic hypoxemia on chemosensitivity in patients with univentricular heart . J Am Coll Cardiol . 1997;30:1827–1834
  113. Durongpisitkul K , Driscoll DJ , Mahoney DW , et al.   Cardiorespiratory response to exercise after modified Fontan operation (determinants of performance) . J Am Coll Cardiol . 1997;29:785–790
  114. Fredriksen PM , Therrien J , Veldtman G , Warsi MA , et al.   Lung function and aerobic capacity in adult patients following modified Fontan procedure . Heart . 2001;85:295–299
  115. Ohuchi H , Tasato H , Sugiyama H , Arakaki Y , Kamiya T . Responses of plasma norepinephrine and heart rate during exercise in patients after Fontan operation and patients with residual right ventricular outflow tract obstruction after definitive reconstruction . Pediatr Cardiol . 1998;19:408–413
  116. Ohuchi H , Yasuda K , Hasegawa S , Miyazaki A , et al.   Influence of ventricular morphology on aerobic exercise capacity in patients after the Fontan operation . J Am Coll Cardiol . 2001;37:1967–1974
  117. Ohuchi H , Hasegawa S , Yasuda K , Yamada O , Ono Y , Echigo S . Severely impaired cardiac autonomic nervous activity after the Fontan operation . Circulation . 2001;104:1513–1518
  118. Iserin L , Chua TP , Chambers J , Coats AJ , Somerville J . Dyspnoea and exercise intolerance during cardiopulmonary exercise testing in patients with univentricular heart. The effects of chronic hypoxaemia and Fontan procedure . Eur Heart J . 1997;18:1350–1356
  119. Ohuchi H , Arakaki Y , Hiraumi Y , Tasato H , Kamiya T . Cardiorespiratory response during exercise in patients with cyanotic congenital heart disease with and without a Fontan operation and in patients with congestive heart failure . Int J Cardiol . 1998;66:241–251
  120. Ghai A , Harris L , Harrison DA , Webb GD , Siu SC . Outcomes of late atrial tachyarrhythmias in adults after the Fontan operation . J Am Coll Cardiol . 2001;37:585–592
  121. Cohen MI , Rhodes LA , Wernovsky G , Gaynor JW , Spray TL , Rychik J . Atrial pacing (an alternative treatment for protein-losing enteropathy after the Fontan operation) . J Thorac Cardiovasc Surg . 2001;121:582–583
  122. Kouatli AA , Garcia JA , Zellers TM , Weinstein EM , Mahony L . Enalapril does not enhance exercise capacity in patients after Fontan procedure . Circulation . 1997;96:1507–1512
  123. Mahle WT , Wernovsky G , Bridges ND , Linton AB , Paridon SM . Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology . J Am Coll Cardiol . 1999;34:1637–1643
  124. Felker GM , O’Connor CM . Inotropic therapy for heart failure (an evidence-based approach) . Am Heart J . 2001;142:393–401
  125. Lowes BD , Simon MA , Tsvetkova TO , Bristow MR . Inotropes in the beta-blocker era . Clin Cardiol . 2000;23:III11–III16
  126. Wessel DL . Managing low cardiac output syndrome after congenital heart surgery . Crit Care Med . 2001;29:S220–S230
  127. Tabbutt S . Heart failure in pediatric septic shock (utilizing inotropic support) . Crit Care Med . 2001;29:S231–S236
  128. Chang AC , Atz AM , Wernovsky G , Burke RP , Wessel DL . Milrinone (systemic and pulmonary hemodynamic effects in neonates after cardiac surgery) . Crit Care Med . 1995;23:1907–1914
  129. Hoffman TM , Wernovsky G , Atz AM , et al.   Prophylactic intravenous use of milrinone after cardiac operation in pediatrics (PRIMACORP) study. Prophylactic Intravenous Use of Milrinone After Cardiac Operation in Pediatrics . Am Heart J . 2002;143:15–21
  130. Baruch L , Patacsil P , Hameed A , Pina I , Loh E . Pharmacodynamic effects of milrinone with and without a bolus loading infusion . Am Heart J . 2001;141:266–273
  131. SoRelle R . Cardiovascular news. VMAC . Circulation . 2000;102:E9050–E9051
  132. Colucci WS , Elkayam U , Horton DP , et al.  Nesiritide Study Group   Intravenous nesiritide, a natriuretic peptide, in the treatment of decompensated congestive heart failure . N Engl J Med . 2000;343:246–253
  133. Colucci WS . Nesiritide for the treatment of decompensated heart failure . J Card Fail . 2001;7:92–100
  134. Rosenzweig EB , Starc TJ , Chen JM , et al.   Intravenous arginine-vasopressin in children with vasodilatory shock after cardiac surgery . Circulation . 1999;100:II182–II186
  135. Reinhartz O , Keith FM , El-Banayosy A , et al.   Multicenter experience with the thoratec ventricular assist device in children and adolescents . J Heart Lung Transplant . 2001;20:439–448
  136. Hetzer R , Loebe M , Potapov EV , et al.   Circulatory support with pneumatic paracorporeal ventricular assist device in infants and children . Ann Thorac Surg . 1998;66:1498–1506
  137. Weyand M , Kececioglu D , Kehl HG , et al.   Neonatal mechanical bridging to total orthotopic heart transplantation . Ann Thorac Surg . 1998;66:519–522
  138. Konertz W , Hotz H , Schneider M , Redlin M , Reul H . Clinical experience with the MEDOS HIA-VAD system in infants and children (a preliminary report) . Ann Thorac Surg . 1997;63:1138–1144
  139. Stiller B , Dahnert I , Weng YG , Hennig E , Hetzer R , Lange PE . Children may survive severe myocarditis with prolonged use of biventricular assist devices . Heart . 1999;82:237–240
  140. Sidiropoulos A , Hotz H , Konertz W . Pediatric circulatory support . J Heart Lung Transplant . 1998;17:1172–1176
  141. del Nido PJ , Duncan BW , Mayer JE , Wessel DL , LaPierre RA , Jonas RA . Left ventricular assist device improves survival in children with left ventricular dysfunction after repair of anomalous origin of the left coronary artery from the pulmonary artery . Ann Thorac Surg . 1999;67:169–172
  142. Thuys CA , Mullaly RJ , Horton SB , et al.   Centrifugal ventricular assist in children under 6 kg . Eur J Cardiothorac Surg . 1998;13:130–134
  143. Williams MR , Quaegebeur JM , Hsu DT , Addonizio LJ , Kichuk MR , Oz MC . Biventricular assist device as a bridge to transplantation in a pediatric patient . Ann Thorac Surg . 1996;62:578–580
  144. Helman DN , Addonizio LJ , Morales DL , et al.   Implantable left ventricular assist devices can successfully bridge adolescent patients to transplant . J Heart Lung Transplant . 2000;19:121–126
  145. Goldstein DJ . Worldwide experience with the MicroMed DeBakey Ventricular Assist Device as a bridge to transplantation . Circulation . 2003;108(Suppl 1):II272–II277
  146. Khan A , Gazzaniga AB . Mechanical circulatory assistance in paediatric patients with cardiac failure . Cardiovasc Surg . 1996;4:43–49
  147. Ibrahim AE , Duncan BW , Blume ED , Jonas RA . Long-term follow-up of pediatric cardiac patients requiring mechanical circulatory support . Ann Thorac Surg . 2000;69:186–192
  148. Kulik TJ , Moler FW , Palmisano JM , et al.   Outcome-associated factors in pediatric patients treated with extracorporeal membrane oxygenator after cardiac surgery . Circulation . 1996;94:II63–II68
  149. Mehta U , Laks H , Sadeghi A , et al.   Extracorporeal membrane oxygenation for cardiac support in pediatric patients . Am Surg . 2000;66:879–886
  150. Pizarro C , Davis DA , Healy RM , Kerins PJ , Norwood WI . Is there a role for extracorporeal life support after stage I Norwood . Eur J Cardiothorac Surg . 2001;19:294–301
  151. Jaggers JJ , Forbess JM , Shah AS , et al.   Extracorporeal membrane oxygenation for infant postcardiotomy support (significance of shunt management) . Ann Thorac Surg . 2000;69:1476–1483
  152. Kirshbom PM , Bridges ND , Myung RJ , Gaynor JW , Clark BJ , Spray TL . Use of extracorporeal membrane oxygenation in pediatric thoracic organ transplantation . J Thorac Cardiovasc Surg . 2002;123:130–136
  153. Burnett CM , Duncan JM , Frazier OH , Sweeney MS , Vega JD , Radovancevic B . Improved multiorgan function after prolonged univentricular support . Ann Thorac Surg . 1993;55:65–71 discussion 71
  154. Burch M , Siddiqi SA , Celermajer DS , Scott C , Bull C , Deanfield JE . Dilated cardiomyopathy in children (determinants of outcome) . Br Heart J . 1994;72:246–250
  155. Lewis AB , Chabot M . Outcome of infants and children with dilated cardiomyopathy . Am J Cardiol . 1991;68:365–369
  156. Griffin ML , Hernandez A , Martin TC , et al.   Dilated cardiomyopathy in infants and children . J Am Coll Cardiol . 1988;11:139–144
  157. Friedman RA , Moak JP , Garson A . Clinical course of idiopathic dilated cardiomyopathy in children . J Am Coll Cardiol . 1991;18:152–156
  158. Muller G , Ulmer HE , Hagel KJ , Wolf D . Cardiac dysrhythmias in children with idiopathic dilated or hypertrophic cardiomyopathy . Pediatr Cardiol . 1995;16:56–60
  159. Duster MC , Bink-Boelkens MT , Wampler D , Gillette PC , McNamara DG , Cooley DA . Long-term follow-up of dysrhythmias following the Mustard procedure . Am Heart J . 1985;109:1323–1326
  160. Hayes CJ , Gersony WM . Arrhythmias after the Mustard operation for transposition of the great arteries (a long-term study) . J Am Coll Cardiol . 1986;7:133–137
  161. Garson A , Bink-Boelkens M , Hesslein PS , et al.   Atrial flutter in the young (a collaborative study of 380 cases) . J Am Coll Cardiol . 1985;6:871–878
  162. Sullivan ID , Presbitero P , Gooch VM , Aruta E , Deanfield JE . Is ventricular arrhythmia in repaired tetralogy of Fallot an effect of operation or a consequence of the course of the disease? A prospective study . Br Heart J . 1987;58:40–44
  163. Deanfield JE , Ho SY , Anderson RH , McKenna WJ , Allwork SP , Hallidie-Smith KA . Late sudden death after repair of tetralogy of Fallot (a clinicopathologic study) . Circulation . 1983;67:626–631
  164. Wiles HB , Gillette PC , Harley RA , Upshur JK . Cardiomyopathy and myocarditis in children with ventricular ectopic rhythm . J Am Coll Cardiol . 1992;20:359–362
  165. Dyckner T , Wester PO . Potassium/magnesium depletion in patients with cardiovascular disease . Am J Med . 1987;82:11–17
  166. Rosenthal DN , Dubin AM , Chin C , Falco D , Gamberg P , Bernstein D . Outcome while awaiting heart transplantation in children (a comparison of congenital heart disease and cardiomyopathy) . J Heart Lung Transplant . 2000;19:751–755
  167. Bossaert L , Van Hoeyweghen R  The Cerebral Resuscitation Study Group . Bystander cardiopulmonary resuscitation (CPR) in out-of-hospital cardiac arrest . Resuscitation . 1989;17(Suppl):S55–S69 discussion S199–206
  168. Nichol G , Stiell IG , Laupacis A , Pham B , De Maio VJ , Wells GA . A cumulative meta-analysis of the effectiveness of defibrillator-capable emergency medical services for victims of out-of-hospital cardiac arrest . Ann Emerg Med . 1999;34:517–525
  169. Perry JC , Knilans TK , Marlow D , Denfield SW , Fenrich AL , Friedman RA . Intravenous amiodarone for life-threatening tachyarrhythmias in children and young adults . J Am Coll Cardiol . 1993;22:95–98
  170. Figa FH , Gow RM , Hamilton RM , Freedom RM . Clinical efficacy and safety of intravenous Amiodarone in infants and children . Am J Cardiol . 1994;74:573–577
  171. Coumel P , Fidelle J . Amiodarone in the treatment of cardiac arrhythmias in children (one hundred thirty-five cases) . Am Heart J . 1980;100:1063–1069
  172. Triedman JK , Bergau DM , Saul JP , Epstein MR , Walsh EP . Efficacy of radiofrequency ablation for control of intraatrial reentrant tachycardia in patients with congenital heart disease . J Am Coll Cardiol . 1997;30:1032–1038
  173. Deal BJ , Mavroudis C , Backer CL , Buck SH , Johnsrude C . Comparison of anatomic isthmus block with the modified right atrial maze procedure for late atrial tachycardia in Fontan patients . Circulation . 2002;106:575–579
  174. Deal BJ , Mavroudis C , Backer CL , Johnsrude CL , Rocchini AP . Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia . Am J Cardiol . 1999;83:563–568
  175. Fogoros RN . The effect of the implantable cardioverter defibrillator on sudden death and on total survival . Pacing Clin Electrophysiol . 1993;16:506–510
  176. Moss AJ , Hall WJ , Cannom DS , et al.   Improved survival with an implanted defibrillator in patients with coronary disease at high risk for ventricular arrhythmia. Multicenter Automatic Defibrillator Implantation Trial Investigators . N Engl J Med . 1996;335:1933–1940
  177. A comparison of antiarrhythmic-drug therapy with implantable defibrillators in patients resuscitated from near-fatal ventricular arrhythmias. The Antiarrhythmics versus Implantable Defibrillators (AVID) Investigators . N Engl J Med . 1997;337:1576–1583
  178. Bocker D , Bansch D , Heinecke A , et al.   Potential benefit from implantable cardioverter-defibrillator therapy in patients with and without heart failure . Circulation . 1998;98:1636–1643
  179. Lorga-Filho A , Geelen P , Vanderheyden M , et al.   Early benefit of implantable cardioverter defibrillator therapy in patients waiting for cardiac transplantation . Pacing Clin Electrophysiol . 1998;21:1747–1750
  180. Dubin A , Berul CI , Bevilacqua LM , et al.   The use of implantable cardioverter defibrillators in pediatric patients awaiting heart transplantaion . J Card Fail . 2003; in press
  181. Dubin AM , Van Hare GF , Collins KK , Bernstein D , Rosenthal DN . Survey of current practices in use of amiodarone and implantable cardioverter defibrillators in pediatric patients with end-stage heart failure . Am J Cardiol . 2001;88:809–810
  182. Link MS , Hill SL , Cliff DL , et al.   Comparison of frequency of complications of implantable cardioverter-defibrillators in children versus adults . Am J Cardiol . 1999;83:263–266 A5–6
  183. Berul CI , Triedman JK , Forbess J , et al.   Minimally invasive cardioverter defibrillator implantation for children (an animal model and pediatric case report) . Pacing Clin Electrophysiol . 2001;24:1789–1794
  184. Abraham WT , Fisher WG , Smith AL , et al.   Cardiac resynchronization in chronic heart failure . N Engl J Med . 2002;346:1845–1853
  185. Cazeau S , Leclercq C , Lavergne T , et al.   Effects of multisite biventricular pacing in patients with heart failure and intraventricular conduction delay . N Engl J Med . 2001;344:873–880
  186. Nelson GS , Berger RD , Fetics BJ , et al.   Left ventricular or biventricular pacing improves cardiac function at diminished energy cost in patients with dilated cardiomyopathy and left bundle-branch block . Circulation . 2000;102:3053–3059
  187. Nelson GS . In: Erratum in (Circulation) . 103:2001;p. 476
  188. Dubin AM , Feinstein JA , Reddy VM , Hanley FL , Van Hare GF , Rosenthal DN . Electrical resynchronization (a novel therapy for the failing right ventricle) . Circulation . 2003;107:2287–2289
  189. Janousek J , Vojtovic P , Hucin B , et al.   Resynchronization pacing is a useful adjunct to the management of acute heart failure after surgery for congenital heart defects . Am J Cardiol . 2001;88:145–152

PII: S1053-2498(04)00200-1

doi: 10.1016/j.healun.2004.03.018

The Journal of Heart and Lung Transplantation
Volume 23, Issue 12 , Pages 1313-1333 , December 2004