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Long-Term Outcomes in Congenital Heart Disease

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Pediatric Critical Care Medicine

Abstract

The long-term survival of children with congenital heart disease (CHD) continues to improve due to advancements in cardiac surgery and perioperative care and enhancements in cardiovascular diagnostic and interventional capabilities. As the mortality rate associated with congenital heart defects has continued to decline, the number of adults with CHD has increased exponentially. In fact, it is estimated that there are now more than one million adults living with CHD. While ongoing improvement in short-term results remains important, the clinical focus of medical caregivers has expanded to evaluating late outcomes. Understanding the long-term outcomes of our CHD survivors allows for intervention opportunities to prevent these late complications and treat them if they are present. The late outcomes encompass not only cardiac specific physiologic and anatomic abnormalities, but also important non-cardiac sequelae such as neurodevelopmental and psychosocial morbidity on the individual and their families.

This chapter will describe the long-term outcomes of specific CHD lesions. The specific CHD lesions are grouped into broad categories including left-to-right shunts, right and left-sided obstructive lesions, mixing lesions, and single ventricle anatomies. The evaluation of neurodevelopmental outcomes is discussed as we attempt to better understand brain development in CHD patients and minimize brain injury that may result from the underlying CHD lesion or the therapies required to repair or palliate the defect. Each section begins with an overview of the anatomy and physiology of the lesion followed by a brief description of the surgical intervention. Complications and long-term outcomes are then described for each lesion.

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References

  1. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. N Engl J Med. 2000;342:256–63.

    CAS  PubMed  Google Scholar 

  2. Kirklin JW, Barratt-Boyes BG. Cardiac surgery. New York: Wiley; 1986.

    Google Scholar 

  3. Trusler GA, Kazenelson G, Freedom RM, et al. Late results following repair of partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. J Thorac Cardiovasc Surg. 1980;79:776–81.

    CAS  PubMed  Google Scholar 

  4. Nicholson IA, Chard RB, Nunn GR, et al. Transcaval repair of the sinus venosus syndrome. J Thorac Cardiovasc Surg. 2000;119:741–4.

    CAS  PubMed  Google Scholar 

  5. Murphy JG, Gersh BJ, McGoon MD, et al. Long-term outcome after surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years. N Engl J Med. 1990;323:1645–50.

    CAS  PubMed  Google Scholar 

  6. Stewart RD, Bailliard F, Kelle AM, et al. Evolving surgical strategy for sinus venosus atrial septal defect: effect on sinus node function and late venous obstruction. Ann Thorac Surg. 2007;84:1651–5.

    PubMed  Google Scholar 

  7. Craig RJ, Selzer A. Natural history and prognosis of atrial septal defect. Circulation. 1968;37:805–15.

    CAS  PubMed  Google Scholar 

  8. Berger F, Vogel M, Kramer A, et al. Incidence of atrial flutter/fibrillation in adults with atrial septal defect before and after surgery. Ann Thorac Surg. 1999;68:75–8.

    CAS  PubMed  Google Scholar 

  9. Kidd L, Driscoll DJ, Gersony WM, et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation. 1993;87:I38–51.

    CAS  PubMed  Google Scholar 

  10. Hoffman JIE, Rudolph A. The natural history of ventricular septal defects in infancy. Am J Cardiol. 1965;16:634–53.

    CAS  PubMed  Google Scholar 

  11. Alpert BS, Cook DH, Varghese PJ, Rowe RD. Spontaneous closure of small ventricular septal defects: ten-year follow-up. Pediatrics. 1979;63:204–6.

    CAS  PubMed  Google Scholar 

  12. Moe DG, Guntheroth WG. Spontaneous closure of uncomplicated ventricular septal defect. Am J Cardiol. 1987;60:674–8.

    CAS  PubMed  Google Scholar 

  13. Roos-Hesselink JW, Meijboom FJ, Spitaels SE, et al. Outcome of patients after surgical closure of ventricular septal defect at young age: longitudinal follow-up of 22–34 years. Eur Heart J. 2004;25:1057–62.

    CAS  PubMed  Google Scholar 

  14. Nicholson IA, Nunn GR, Sholler GF, et al. Simplified single patch technique for the repair of atrioventricular septal defect. J Thorac Cardiovasc Surg. 1999;118:642–6.

    CAS  PubMed  Google Scholar 

  15. Atz AM, Hawkins JA, Lu M, et al. Surgical management of complete atrioventricular septal defect: associations with surgical technique, age, and trisomy 21. J Thorac Cardiovasc Surg. 2010;141:1371–9.

    PubMed Central  PubMed  Google Scholar 

  16. Hoohenkerk GJF, Bruggemans EF, Rijlaarsdam M, et al. More than 30 years’ experience with surgical correction of atrioventricular septal defects. Ann Thorac Surg. 2010;90:1554–61.

    PubMed  Google Scholar 

  17. Stulak JM, Burkhart HM, Dearani JA, et al. Reoperations after initial repair of complete atrioventricular septal defects. Ann Thorac Surg. 2009;87:1872–7. discussion 1877–1878.

    PubMed  Google Scholar 

  18. Hoohenkerk GJF, Bruggemans EF, Koolbergen DR, et al. Long-term results of reoperation for left atrioventricular valve regurgitation after correction of atrioventricular septal defects. Ann Thorac Surg. 2012;93:849–55.

    PubMed  Google Scholar 

  19. Hals J, Hagemo PS, Thaulow E, et al. Pulmonary vascular resistance in complete atrioventricular septal defect: a comparison between children with and without Down syndrome. Acta Paediatr. 1993;82:595–8.

    CAS  PubMed  Google Scholar 

  20. Pozzi M, Remig J, Fimmers R, et al. Atrioventricular septal defect: analysis of short and mid-term results. J Thorac Cardiovasc Surg. 1991;101:138–42.

    CAS  PubMed  Google Scholar 

  21. Nagao GI, et al. Cardiovascular anomalies associated with tetralogy of Fallot. Am J Cardiol. 1967;20:206–15.

    CAS  PubMed  Google Scholar 

  22. Stamm C, Friehs I, Zurakowski D, et al. Outcome after reconstruction of discontinuous pulmonary arteries. J Thorac Cardiovasc Surg. 2002;123:246–57.

    PubMed  Google Scholar 

  23. Gupta D, et al. Detection of coronary artery anomalies in tetralogy of Fallot using a specific angiographic protocol. Am J Cardiol. 2001;87:241–4.

    CAS  PubMed  Google Scholar 

  24. Kirklin JW, et al. Surgical treatment for the tetralogy of Fallot by open intracardiac repair. J Thorac Surg. 1959;37:22–51.

    CAS  PubMed  Google Scholar 

  25. Landolt CC, et al. Importance of coronary artery anomalies in operations for congenital heart disease. Ann Thorac Surg. 1986;41:351–5.

    CAS  PubMed  Google Scholar 

  26. McManus BM, et al. The case for preoperative coronary angiography in patients with tetralogy of Fallot and other complex congenital heart diseases. Am Heart J. 1982;103:451–6.

    CAS  PubMed  Google Scholar 

  27. Murphy JG, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993;329:593–9.

    CAS  PubMed  Google Scholar 

  28. Kuehne T, et al. Sequential magnetic resonance monitoring of pulmonary flow with endovascular stents placed across the pulmonary valve in growing swine. Circulation. 2001;104:2363–8.

    CAS  PubMed  Google Scholar 

  29. Kuehne T. Effects of pulmonary insufficiency on biventricular function in the developing heart of growing swine. Circulation. 2003;108:2007–13.

    PubMed  Google Scholar 

  30. Lam W, Friedman RA. Electrophysiology issues in adult congenital heart disease. Methodist Debakey Cardiovasc J. 2011;7:13–7.

    PubMed  Google Scholar 

  31. Davlouros PA, Kilner PJ, Hornung TS, et al. Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging. J Am Coll Cardiol. 2002;40(11):2044–52.

    PubMed  Google Scholar 

  32. Kempny A, Diller GP, Orwat S, et al. Right ventricular–left ventricular interaction in adults with tetralogy of Fallot: a combined cardiac magnetic resonance and echocardiographic speckle tracking study. Int J Cardiol. 2012;154:259–64.

    PubMed  Google Scholar 

  33. Therrien J, et al. Pulmonary valve replacement in adults late after repair of tetralogy of fallot: are we operating too late? J Am Coll Cardiol. 2000;36:1670–5.

    CAS  PubMed  Google Scholar 

  34. van Huysduynen BH, et al. Reduction of QRS duration after pulmonary valve replacement in adult Fallot patients is related to reduction of right ventricular volume. Eur Heart J. 2005;26:928–32.

    PubMed  Google Scholar 

  35. Karamlou T, et al. Outcomes after late reoperation in patients with repaired tetralogy of Fallot: the impact of arrhythmia and arrhythmia surgery. Ann Thorac Surg. 2006;81:1786–93. discussion 1793.

    PubMed  Google Scholar 

  36. Geva T. Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. J Cardiovasc Magn Reson. 2011;13:9.

    PubMed Central  PubMed  Google Scholar 

  37. Deanfield JE, McKenna WJ, Presbitero P, et al. Ventricular arrhythmia in unrepaired and repaired tetralogy of Fallot. Relation to age, timing of repair, and haemodynamic status. Br Heart J. 1984;52:77–81.

    CAS  PubMed Central  PubMed  Google Scholar 

  38. Garson A, Nihill MR, McNamara DG, et al. Status of the adult and adolescent after repair of tetralogy of Fallot. Circulation. 1979;59:1232–40.

    PubMed  Google Scholar 

  39. Gatzoulis MA, Till JA, Sommerville J, et al. Mechanoelectrical interaction in tetralogy of Fallot; QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation. 1995;92:231–7.

    CAS  PubMed  Google Scholar 

  40. Gross GJ, Chiu CC, Hamilton RM, et al. Natural history of postoperative heart block in congenital heart disease: implications for pacing intervention. Heart Rhythm. 2006;3:601–4.

    PubMed  Google Scholar 

  41. Haworth SG. Collateral arteries in pulmonary atresia with ventricular septal defect: a precarious blood supply. Br Heart J. 1980;44:5.

    CAS  PubMed Central  PubMed  Google Scholar 

  42. Liao PK, Edwards WD, Julsrud PR, et al. Pulmonary blood supply in patients with pulmonary atresia with ventricular septal defect. J Am Coll Cardiol. 1985;6:1343.

    CAS  PubMed  Google Scholar 

  43. McElhinney DB, Reddy VM, Hanley FL. Tetralogy of Fallot with major aortopulmonary collaterals: early total repair. Pediatr Cardiol. 1998;19:289–96. 51.

    CAS  PubMed  Google Scholar 

  44. Reddy VM, Petrossian E, McElhinney DB, et al. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed? J Thorac Cardiovasc Surg. 1997;113:858–68. 52.

    CAS  PubMed  Google Scholar 

  45. Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation. 2000;101:1826–32.

    CAS  PubMed  Google Scholar 

  46. Cho JM, Puga FJ, Danielson GK, et al. Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2002;124:70–81. 48.

    PubMed  Google Scholar 

  47. Lev M, Eckner FAO. The pathologic anatomy of tetralogy of Fallot and its variations. Chest. 1964;45:251–61. 79.

    CAS  Google Scholar 

  48. Donofrio MT, Jacobs ML, Rychik J. Tetralogy of Fallot with absent pulmonary valve: echocardiographic morphometric features of the right-sided structures and their relationship to presentation and outcome. J Am Soc Echocardiogr. 1997;10:556–61. 114.

    CAS  PubMed  Google Scholar 

  49. McDonnell BE, Raff GW, Gaynor JW, et al. Outcome after repair of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg. 1999;67:1391–5. discussion 1395–1396.

    CAS  PubMed  Google Scholar 

  50. Dearani JA, Danielson GK. Congenital heart surgery nomenclature and database project: Ebstein’s anomaly and tricuspid valve disease. Ann Thorac Surg. 2000;69:S106–17.

    CAS  PubMed  Google Scholar 

  51. Benson DW, Silberbach GM, Kavanaugh-McHugh A, et al. Mutations in the cardiac transcription factor NKX2.5 affect diverse cardiac developmental pathways. J Clin Invest. 1999;104:1567–73.

    CAS  PubMed Central  PubMed  Google Scholar 

  52. Knott-Craig CJ, Goldberg SP, Overholt ED, et al. Repair of neonates and young infants with Ebstein’s anomaly and related disorders. Ann Thorac Surg. 2007;84:587–93.

    PubMed  Google Scholar 

  53. Reemtsen BL, Fagan BT, Wells WJ, Starnes VA. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg. 2006;132:1285–90.

    PubMed  Google Scholar 

  54. Attie F, Rosas M, Rijlaarsdam M, et al. The adult patient with Ebstein anomaly. Outcome in 72 unoperated patients. Medicine (Baltimore). 2000;7:27–36.

    Google Scholar 

  55. Brown ML, Dearani JA, Danielson GK. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg. 2008;135:1120–36.

    PubMed  Google Scholar 

  56. Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol. 1970;26:72–83.

    CAS  PubMed  Google Scholar 

  57. Larson EC, Edwards WD. Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol. 1984;53:849–55.

    CAS  PubMed  Google Scholar 

  58. Nistri S, Sorbo MD, Marin M, et al. Aortic root dilatation in young men with normally functioning bicuspid aortic valves. Heart. 1999;82:19–22.

    CAS  PubMed Central  PubMed  Google Scholar 

  59. Braunwald E, Goldblatt A, Aygen MM, et al. Congenital aortic stenosis. I. Clinical and hemodynamic findings in 100 patients. II. Surgical and the results of operation. Circulation. 1963;27:426–62.

    CAS  PubMed  Google Scholar 

  60. Nadas AS. Report from the joint study on the natural history of congenital heart defects. IV. Clinical course. Introduction. Circulation. 1977;56:I36–8.

    CAS  PubMed  Google Scholar 

  61. McElhinney DB, Lock JE, Keane JF, et al. Left heart growth, function and reintervention after aortic valvuloplasty for neonatal aortic stenosis. Circulation. 2005;111:451.

    PubMed  Google Scholar 

  62. Moore P, Egito E, Mowrey H, et al. Midterm results of balloon dilation of congenital aortic stenosis: predictors of success. J Am Coll Cardiol. 1996;27:1257.

    CAS  PubMed  Google Scholar 

  63. Bonow RO, Carabello BA, Chatterjee K, et al. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2008;23(52):e1–142.

    Google Scholar 

  64. Brown JW, Ruzmetov M, Vijay P, et al. The Ross-Konno procedure in children: outcomes, autograft and allograft function, and reoperations. Ann Thorac Surg. 2006;82:1301–6.

    PubMed  Google Scholar 

  65. Ohye RG, Gomez CA, Ohye BJ, et al. The Ross/Konno procedure in neonates and infants: intermediate-term survival and autograft function. Ann Thorac Surg. 2001;72:823–30.

    CAS  PubMed  Google Scholar 

  66. Elkins RC, Lane MM, McCue C. Ross operation in children: late results. J Heart Valve Dis. 2001;10:736–41.

    CAS  PubMed  Google Scholar 

  67. Wright GB, Keane JF, Nadas AS, et al. Fixed subaortic stenosis in the young: medical and surgical course in 83 patients. Am J Cardiol. 1983;52:830–5.

    CAS  PubMed  Google Scholar 

  68. McMahon CJ, Gauvreau K, Edwards JC, et al. Risk factors for aortic valve dysfunction in children with discrete subvalvar aortic stenosis. Am J Cardiol. 2004;94:459–64.

    PubMed  Google Scholar 

  69. Firpo C, Maitre Azcarate MJ, Quero Jimenez M, et al. Discrete subaortic stenosis (DSS) in childhood: a congenital or acquired disease? Follow-up in 65 patients. Eur Heart J. 1990;11:1033–40.

    CAS  PubMed  Google Scholar 

  70. Lupinetti FM, Pridjain AK, Callow LB, et al. Optimum treatment of discrete subaortic stenosis. Ann Thorac Surg. 1992;54:467–70.

    CAS  PubMed  Google Scholar 

  71. Parry AJ, Kovalchin JP, Suda K, et al. Resection of subaortic stenosis; can a more aggressive approach be justified? Eur J Cardiothorac Surg. 1999;15:631–8.

    CAS  PubMed  Google Scholar 

  72. Wood AE, Javadpour H, Duff D, et al. Is extended arch aortoplasty the operation of choice for infant aortic coarctation? Results of 15 years’ experience in 181 patients. Ann Thorac Surg. 2004;77:1353–7.

    PubMed  Google Scholar 

  73. Moene RJ, Gittenberger-de Groot AC, Oppenheimer-Dekker A, et al. Anatomic characteristics of ventricular septal defect associated with coarctation of the aorta. Am J Cardiol. 1987;59:952–5.

    CAS  PubMed  Google Scholar 

  74. Shone JD, Sellers RD, Anderson RC, et al. The development complex of “parachute mitral valve”, supravalvar ring of left atrium, subaortic stenosis and coarctation of the aorta. Am J Cardiol. 1963;11:714–25.

    CAS  PubMed  Google Scholar 

  75. Wright GE, Nowak CA, Goldberg CS, et al. Extended resection and end-to-end anastomosis for aortic coarctation in infants: results of a tailored surgical approach. Ann Thorac Surg. 2005;80:1453–9.

    PubMed  Google Scholar 

  76. Puchalski MD, Williams RV, Hawkins JA, et al. Follow-up of aortic coarctation repair in neonates. J Am Coll Cardiol. 2004;44:188–91.

    PubMed  Google Scholar 

  77. Kaushal S, Backer CL, Patel JN, et al. Coarctation of the aorta: midterm outcomes of resection with extended end-to-end anastomosis. Ann Thorac Surg. 2009;88:1932–8.

    PubMed  Google Scholar 

  78. Parks WJ, Mgo TD, Plauth WH, et al. Incidence of aneurysm formation after Dacron patch aortoplasty repair for coarctation of the aorta: long-term results and assessment utilizing magnetic resonance angiography with three-dimensional surface rendering. J Am Coll Cardiol. 1995;26:266–71.

    CAS  PubMed  Google Scholar 

  79. Bromberg BI, Beekman RH, Rocchini AP, et al. Aortic aneurysm after patch aortoplasty repair of coarctation: a prospective analysis of prevalence, screening tests and risks. J Am Coll Cardiol. 1989;14:734–41.

    CAS  PubMed  Google Scholar 

  80. Del Nido P, Williams W, Wilson G, et al. Synthetic patch angioplasty for repair of coarctation of the aorta: experience with aneurysm formation. Circulation. 1986;74:I32–6.

    PubMed  Google Scholar 

  81. Rubay JE, Sluysmans T, Alexandrescu V, et al. Surgical repair of coarctation of the aorta in infants under one year of age: long-term results in 146 patients comparing subclavian flap angioplasty and modified end-to-end anastomosis. J Cardiovasc Surg. 1992;33:216–22.

    CAS  Google Scholar 

  82. Merill WH, Hoff SJ, Stewart JR, et al. Operative risk factors and durability of repair of coarctation of the aorta in the neonate. Ann Thorac Surg. 1994;58:399–402.

    Google Scholar 

  83. Ibarra-Perez C, Castaneda AR, Varco RL, et al. Recoarctation of the aorta: nineteen year clinical experience. Am J Cardiol. 1969;23:778–84.

    CAS  PubMed  Google Scholar 

  84. Beekman RH, Rocchini AP, Behrendt DM, et al. Long-term outcome after repair of coarctation in infancy: subclavian angioplasty does not reduce the need for reoperation. J Am Coll Cardiol. 1986;8:1406–11.

    CAS  PubMed  Google Scholar 

  85. Brouwer RM, Erasmus ME, Ebels T, et al. Influence of age on survival, late hypertension, and recoarctation in elective aortic coarctation repair. J Thorac Cardiovasc Surg. 1994;108:525–31.

    CAS  PubMed  Google Scholar 

  86. Kaemmerer H, Oelert F, Bahlmann J, et al. Arterial hypertension in adults after surgical treatment of aortic coarctation. Thorac Cardiovasc Surg. 1998;46:121–5.

    CAS  PubMed  Google Scholar 

  87. O’Sullivan JJ, Derrick G, Darnell R. Prevalence of hypertension in children after early repair of coarctation of the aorta: a cohort study using casual and 24 hour blood pressure measurement. Heart. 2002;88:163–6.

    PubMed Central  PubMed  Google Scholar 

  88. Hijazi ZM, Fahey JT, Kleinman CS, et al. Balloon angioplasty for recurrent coarctation of the aorta. Circulation. 1991;84:1150–6.

    CAS  PubMed  Google Scholar 

  89. Yetman AT, Nykanen D, McCrindle BW, et al. Balloon angioplasty of recur-rent coarctation: a 12-year review. J Am Coll Cardiol. 1997;30:811–6.

    CAS  PubMed  Google Scholar 

  90. Mendelshon AM, Lloyd TR, Crowley DC, et al. Late follow-up of balloon angioplasty in children with a native coarctation of the aorta. Am J Cardiol. 1994;74:696–700.

    Google Scholar 

  91. Fletcher SE, Nihill MR, Grifka RG, et al. Balloon angioplasty of native coarctation of the aorta: mid-term follow-up and prognostic factors. J Am Coll Cardiol. 1995;25:730–4.

    CAS  PubMed  Google Scholar 

  92. Kirklin JW, Barratt-Boyes BG. Complete transposition of the great arteries. In: Kirklin JW, Barratt-Boyes BG, editors. Cardiac surgery. New York: Churchill Livingston; 1993. p. 1383–467.

    Google Scholar 

  93. Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Transposition of the great arteries. In: Moss and Adams’ heart disease in infants, children, and adolescents. Philadelphia: Lippincott Williams & Wilkins; 2013. p. 1098–106.

    Google Scholar 

  94. Roubertie F, Thambo JB, Bretonneau A, et al. Late outcome of 132 Senning procedures after 20 years of follow-up. Ann Thorac Surg. 2011;92:2206–13.

    PubMed  Google Scholar 

  95. Puley G, Siu S, Connelly M, et al. Arrhythmia and survival in patients >18 years of age after the mustard procedure for transposition of the great arteries. Am J Cardiol. 1999;83:1080–4.

    CAS  PubMed  Google Scholar 

  96. 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;29:194–201.

    CAS  PubMed  Google Scholar 

  97. Wilson NJ, Clarkson PM, Barratt-Boyes BG, et al. Long-term outcome after the Mustard repair for simple transposition of the great arteries. J Am Coll Cardiol. 1998;32:758–65.

    CAS  PubMed  Google Scholar 

  98. Meijboom F, Szatmari A, Deckers JW, et al. Long-term follow-up (10 to 17 years) after Mustard repair for transposition of the great arteries. J Thorac Cardiovasc Surg. 1996;111:1158–68.

    CAS  PubMed  Google Scholar 

  99. Moons P, Gewillig M, Sluysmans T, et al. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart. 2004;90:307–13.

    CAS  PubMed Central  PubMed  Google Scholar 

  100. Roos-Hesselink JW, Meijboom FJ, Spitaels SEC, et al. Decline in ventricular function and clinical condition after mustard repair for transposition of the great arteries (a prospective study of 22–29 years). Eur Heart J. 2004;25:1264–70.

    CAS  PubMed  Google Scholar 

  101. Silka MJ, Hardy BG, Menashe VD, et al. A population based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects. J Am Coll Cardiol. 1998;32:245–51.

    CAS  PubMed  Google Scholar 

  102. Janousek J, Paul T, Luhmer I, et al. Atrial baffle procedures for complete transposition of the great arteries: natural course of sinus node dysfunction and risk factors for dysrhythmias and sudden death. Z Kardiol. 1994;83:933–8.

    CAS  PubMed  Google Scholar 

  103. Rudra HS, Mavroudis C, Backer CL, et al. The arterial switch operation: 25-year experience with 258 patients. Ann Thorac Surg. 2011;92:1742–6.

    PubMed  Google Scholar 

  104. Hutter PA, Kreb DL, Mantel SF, et al. Twenty-five years’ experience with the arterial switch operation. Thorac Cardiovasc Surg. 2002;124:790.

    CAS  Google Scholar 

  105. Tobler D, Williams WG, Jegatheeswaran A, et al. Cardiac outcomes in young adult survivors of the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol. 2010;56:58.

    PubMed  Google Scholar 

  106. Losay J, Touchot A, Serraf A, et al. Late outcome after arterial switch operation for transposition of the great arteries. Circulation. 2001;104:1121–6.

    Google Scholar 

  107. Mussatto K, Wernovsky G. Challenges facing the child, adolescent, and young adult after the arterial switch operation. Cardiol Young. 2005;15:111–21.

    PubMed  Google Scholar 

  108. Marino BS, Wernovsky G, McElhinney DB, et al. Neo-aortic valvar function after the arterial switch. Cardiol Young. 2006;16:481–9.

    PubMed  Google Scholar 

  109. Losay J, Touchot A, Capderou A, et al. Aortic valve regurgitation after arterial switch operation for transposition of the great arteries – Incidence, risk factors, and outcome. J Am Coll Cardiol. 2006;47:2057–62.

    PubMed  Google Scholar 

  110. Hutter PA, Kreb DL, Mantel SF, et al. Twenty-five years’ experience with the arterial switch operation. J Thorac Cardiovasc Surg. 2002;124:790–7.

    CAS  PubMed  Google Scholar 

  111. Vouhe PR, Tamisier D, Leca F, et al. Transposition of the great-arteries, ventricular septal defect, and pulmonary outflow tract obstruction – Rastelli Or Lecompte Procedure. J Thorac Cardiovasc Surg. 1992;103:428–36.

    CAS  PubMed  Google Scholar 

  112. Sharma R, Choudhary SK, Bhan A, et al. Late outcome after arterial switch operation for complete transposition of great arteries with left ventricular outflow tract obstruction. Ann Thorac Surg. 2002;74:1986–91.

    PubMed  Google Scholar 

  113. Connelly MS, Liu PP, Williams WG, et al. Congenitally corrected trans-position of the great arteries in the adult: functional status and complications. J Am Coll Cardiol. 1996;27:1238–43.

    CAS  PubMed  Google Scholar 

  114. Hornung TS, Calder L. Congenitally corrected transposition of the great arteries. Heart. 2010;96:1154–61.

    PubMed  Google Scholar 

  115. Graham Jr TP, Bernard YD, Mellen BG, et al. Long-term outcome in con-genitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol. 2000;36:255–61.

    PubMed  Google Scholar 

  116. Quinn DW, McGuirk SP, Metha C, et al. The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction. J Thorac Cardiovasc Surg. 2008;135:1137–44.

    PubMed  Google Scholar 

  117. Butto F, Lucas RV, Edwards JE. Persistent truncus arteriosus: pathologic anatomy in 54 cases. Pediatr Cardiol. 1986;7:95–101.

    CAS  PubMed  Google Scholar 

  118. Nath P, Zollikofer C, Castandeda-Zuniga W. Persistent truncus arteriosus associated with interruption of the aortic arch. Br J Radiol. 1980;53:853–9.

    CAS  PubMed  Google Scholar 

  119. Rajasinghe HA, McElhinney DB, Reddy VM, et al. Long term follow-up of truncus arteriosus repaired in infancy: a twenty year experience. Thorac Cardiovasc Surg. 1997;113:869–79.

    CAS  Google Scholar 

  120. Thompson LD, McElhinney DB, Reddy VM, et al. Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg. 2001;72:391–5.

    CAS  PubMed  Google Scholar 

  121. Brown JW, Ruzmetov M, Okada Y, et al. Truncus arteriosus repair, outcomes, risk factors, reoperation and management. Eur J Cardiothrac Surg. 2001;20:221–7.

    CAS  Google Scholar 

  122. Seale AN, Uemura H, Webber SA, et al. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 2010;122:2718–26.

    PubMed  Google Scholar 

  123. Korbmacher B, Buttgen S, Schulte HD, et al. Long term results after repair of total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 2001;49(2):101–6.

    CAS  Google Scholar 

  124. Azakie A, Russell JL, McCrindle BW, et al. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early patterns of survival, patterns of ventricular recover and late outcome. Ann Thorac Surg. 2003;75:1535–41.

    PubMed  Google Scholar 

  125. Imamura M, Dossey AM, Jaquiss RDB. Reoperation and mechanical circulatory support after repair of anomalous origin of the left coronary artery from the pulmonary artery: a twenty-year experience. Ann Thorac Surg. 2011;92:167–73.

    PubMed  Google Scholar 

  126. Yong MS, d’Udekem Y, Konstantinov IE, et al. Surgical management of pulmonary artery sling in children. J Thorac Cardiovasc Surg. 2013;145:1033–9.

    PubMed  Google Scholar 

  127. Backer CL, Ilbawi MN, DeLeon SY. Vascular anomalies causing tracheoesophageal compression: review of experience in children. J Thorac Cardiovasc Surg. 1989;97:725–31.

    CAS  PubMed  Google Scholar 

  128. Goldstein B, Bergersen L, Lang P, et al. Long-term outcome of surgically repaired unilateral anomalous pulmonary artery origin. Pediatr Cardiol. 2010;31:944–51.

    PubMed  Google Scholar 

  129. Binet JP, Langlois J. Aortic arch anomalies in children and infants. Thorac Cardiovasc Surg. 1977;73:248–52.

    CAS  Google Scholar 

  130. Roesler M, De LM, Chrispin A, Stark J. Surgical management of vascular ring. Ann Surg. 1983;197:139–46.

    CAS  PubMed Central  PubMed  Google Scholar 

  131. Moes CAF. Vascular rings and anomalies of the aortic arch. In: Keith JD, Rowe DR, Vlad P, editors. Heart disease in infancy and childhood. New York: Macmillan; 1978. p. 856–81.

    Google Scholar 

  132. van Son JA, Julsrud PR, Hagler DJ, et al. Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc. 1993;68:1056–63.

    PubMed  Google Scholar 

  133. Alsenaidi K, Gurofsky R, McCrindle B. Management and outcomes of double aortic arch in 81 patients. Pediatrics. 2006;118:1336–41.

    Google Scholar 

  134. Anand R, Dooley KJ, Vincent RN, et al. Follow-up of surgical correction of vascular anomalies causing tracheobronchial compression. Pediatr Cardiol. 1994;15:58–61.

    CAS  PubMed  Google Scholar 

  135. Chun K, Colombani PM, Dudgeon DL, Haller Jr JA. Diagnosis and management of congenital vascular rings: a 22-year experience. Ann Thorac Surg. 1992;53:597–602.

    CAS  PubMed  Google Scholar 

  136. Shinkawa T, Greenberg B, Imamura M. Primary translocation of aberrant left subclavian artery for children with symptomatic vascular ring. Ann Thorac Surg. 2012;93:1262–5.

    PubMed  Google Scholar 

  137. Li S, Luo G, Norwitz E, et al. Prenatal diagnosis of congenital vascular rings and slings: sonographic features and perinatal outcome in 81 consecutive cases. Prenat Diagn. 2011;31:334–46.

    CAS  PubMed  Google Scholar 

  138. Backer CL, Hillman N, Holinger LD, et al. Resection of Kommerell’s diverticulum and left subclavian artery transfer for recurrent symptoms after vascular ring division. Eur J Cardiothorac Surg. 2002;22:64–9.

    CAS  PubMed  Google Scholar 

  139. Kouchoukos NT, Masetti P. Aberrant subclavian artery and Kommerell aneurysm: surgical treatment with a standard approach. J Thorac Cardiovasc Surg. 2007;133:888–92.

    PubMed  Google Scholar 

  140. Backer CL, Mavroudis C, Holinger LD, et al. Trends in vascular ring surgery. J Thorac Cardiovasc Surg. 2005;129:1339–47.

    PubMed  Google Scholar 

  141. Hennein HA. The Fontan operation for hypoplastic left heart syndrome. In: Hennein HA, Bove EL, editors. Hypoplastic left heart syndrome. Armonk: Futura Publishing; 2002. p. 155–78.

    Google Scholar 

  142. Mair DD, Puga FJ, Danielson GK. The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients. J Am Coll Cardiol. 2001;37:933–9.

    CAS  PubMed  Google Scholar 

  143. Bove EL, de Leval MR, Migliavacca F, et al. Computational fluid dynamics in the evaluation of hemodynamic performance of cavopulmonary connections after the Norwood procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126:1040–7.

    PubMed  Google Scholar 

  144. Nurnberg JH, Ovroutski S, Alexi-Meskishvilli V, et al. New onset arrhythmias after the extracardiac conduit Fontan operation compared with the intraatrial lateral tunnel procedure: early and midterm results. Ann Thorac Surg. 2004;78:1979–88.

    PubMed  Google Scholar 

  145. Azakie A, McCrindle BW, Van Arsdell G, et al. Extracardiac conduit versus lateral tunnel cavopulmonary connections at a single institution: impact on outcomes. J Thorac Cardiovasc Surg. 2001;122:1219–28.

    CAS  PubMed  Google Scholar 

  146. Bae E, Lee J, Noh C, et al. Sinus node dysfunction after Fontan modifications – influence of surgical method. Int J Cardiol. 2003;88:285–91.

    PubMed  Google Scholar 

  147. Cohen MI, Bridges ND, Gaynor JW, et al. Modifications to the cavopulmonary anastomosis do not eliminate early sinus node dysfunction. J Thorac Cardiovasc Surg. 2000;120:891–901.

    CAS  PubMed  Google Scholar 

  148. Kumar SP, Rubinstein CS, Simsic JM, et al. Lateral tunnel versus extracardiac conduit Fontan procedure: a concurrent comparison. Ann Thorac Surg. 2003;76:1389–96.

    PubMed  Google Scholar 

  149. Gaynor JW, Bridges ND, Cohen MI, et al. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg. 2002;123:237–45.

    PubMed  Google Scholar 

  150. Burkhart HM, Dearani JA, Mair DD, et al. The modified Fontan procedure: early and late results in 132 adult patients. J Thorac Cardiovasc Surg. 2003;125:1252–8.

    PubMed  Google Scholar 

  151. Khaity P, Fernandes SM, Mayer Jr JE, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117:85–92.

    Google Scholar 

  152. Feinstein JA, Benson DW, Martin GR, et al. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol. 2012;59:S1–42.

    PubMed  Google Scholar 

  153. Mahle WT, Coon PD, Wernovsky G, et al. Quantitative echocardiographic assessment of the performance of the functional single ventricle after the Fontan operation. Cardiol Young. 2001;11:399–406.

    CAS  PubMed  Google Scholar 

  154. Cheung YF, Penny DJ, Redington AN. Serial assessment of left ventricular diastolic function after Fontan procedure. Heart. 2000;83:420–4.

    CAS  PubMed Central  PubMed  Google Scholar 

  155. Marino BS. Outcomes after the Fontan procedure. Curr Opin Pediatr. 2002;14:620–6.

    PubMed  Google Scholar 

  156. Senzaki H, Masutani S, Kobayashi J, et al. Ventricular afterload and ventricular work in Fontan circulation: comparison with normal two-ventricle circulation and single-ventricle circulation with Blalock-Taussig shunts. Circulation. 2002;105:2885–92.

    PubMed  Google Scholar 

  157. Gentles TL, Gauvreau K, Mayer JE, et al. Functional outcome after the Fontan operation: factors influencing late morbidity. J Thorac Cardiovasc Surg. 1997;114:392–403.

    CAS  PubMed  Google Scholar 

  158. Fredriksen PM, Therrien J, Veldtman G, et al. Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart. 2001;85:295–9.

    CAS  PubMed Central  PubMed  Google Scholar 

  159. Gewillig M. The Fontan circulation: late functional results. Semin Thorac Cardiovasc Surg. 1994;6:56–63.

    CAS  PubMed  Google Scholar 

  160. Triedman JK, Bridges ND, Mayer Jr JE, et al. Prevalence and risk factors for aortopulmonary collateral vessels after Fontan and bidirectional Glenn procedures. J Am Coll Cardiol. 1993;22:207–15.

    CAS  PubMed  Google Scholar 

  161. Feldt RH, Driscoll DJ, Offord KP, et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996;122:672–80.

    Google Scholar 

  162. Powell AJ, Gauvreau K, Jenkins KJ, et al. Perioperative risk factors for development of protein-losing enteropathy following a Fontan procedure. Am J Cardiol. 2001;88:1206–9.

    CAS  PubMed  Google Scholar 

  163. Rychik J, Spray TL. Strategies to treat protein-losing enteropathy. Semin Thorac Cardiovasc Pediatr Cardiac Surg Annu. 2002;5:3–11.

    Google Scholar 

  164. Mertens L, Hagler DJ, Sauer U, et al. Protein-losing enteropathy after the Fontan operation: an international multicenter study. J Thorac Cardiovasc Surg. 1998;115:1063–73.

    CAS  PubMed  Google Scholar 

  165. Parsons MK, Moreau GA, Graham Jr TP, et al. Echocardiographic estimation of critical left ventricular size in infants with isolated aortic valve stenosis. J Am Coll Cardiol. 1991;18:1049–55.

    CAS  PubMed  Google Scholar 

  166. Lenz D, Hambsch J, Schneider P, et al. Protein-losing enteropathy in patients with Fontan circulation: is it triggered by infection? Crit Care. 2003;7:185–90.

    PubMed Central  PubMed  Google Scholar 

  167. Therrien J, Webb GD, Gatzoulis MA. Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation? Heart. 1999;82:241–3.

    CAS  PubMed Central  PubMed  Google Scholar 

  168. Coon PD, Rychik J, Novello RT, et al. Thrombus formation after the Fontan operation. Ann Thorac Surg. 2001;71:1990–4.

    CAS  PubMed  Google Scholar 

  169. Mongale P, Karl TR. Thromboembolic problems after the Fontan operation. Semin Thorac Cardiovasc Surg. 2002;5:36–47.

    Google Scholar 

  170. Cromme-Dijkhuis AH, Hess J, Hahlen K, et al. Specific sequelae after Fontan operation at mid- and long-term follow up. Arrhythmia, liver dysfunction, and coagulation disorders. J Thorac Cardiovasc Surg. 1993;106:1126–32.

    CAS  PubMed  Google Scholar 

  171. Rosenthal DN, Friedman AH, Kleinman CS, et al. Thromboembolic complications after Fontan operations. Circulation. 1995;92:287–93.

    Google Scholar 

  172. Monagle P, Cochrane A, Roberts R, et al. A Multicenter randomized trial comparing Heparin/warfarin versus aspiring as primary thromboprophylaxis for two years after Fontan procedure in children. Circulation. 2008;118:S651.

    Google Scholar 

  173. Meyer D, Zamora G, Wernovsky G, et al. Outcomes of the Fontan procedure using cardiopulmonary bypass with aortic cross-clamping. Ann Thorac Surg. 2006;82:1611–8.

    PubMed  Google Scholar 

  174. Day RW, Boyer RS, Tait VF, et al. Factors associated with stroke following the Fontan procedure. Pediatr Cardiol. 1995;16:270–5.

    CAS  PubMed  Google Scholar 

  175. Mair DD, Puga FJ, Danielson GK. Late functional status of survivors of the Fontan procedure performed during the 1970s. Circulation. 1992;86:106–9.

    Google Scholar 

  176. Deal BJ, Mavroudis C, Backer CL, et al. Diagnosis and management of late atrial arrhythmias following Fontan surgery. In: Hennein HA, Bove EL, editors. Hypoplastic left heart syndrome. Armonk: Futura Publishing; 2002. p. 253–63.

    Google Scholar 

  177. Amodeo A, Galletti L, Marianeschi S, et al. Extracardiac Fontan operation for complex cardiac anomalies: seven years” experience. J Thorac Cardiovasc Surg. 1997;114:1020–30.

    CAS  PubMed  Google Scholar 

  178. Ono M, Boethig D, Breymann T. Clinical outcome of patients 20 years after Fontan operation – effect of fenestration on late morbidity. Eur J Cardiothorac Surg. 2006;30:923–9.

    PubMed  Google Scholar 

  179. Bokenkamp A, Domanetzki M, Zinck R, Schumann G, Brodehl J. Reference values for cystatin C serum concentrations in children. Pediatr Nephrol. 1998;12:125–9.

    CAS  PubMed  Google Scholar 

  180. Filler G, Priem F, Vollmer I, Gellermann J, Jung K. Diagnostic sensitivity of serum cystatin for impaired glomerular filtration rate. Pediatr Nephrol. 1999;13:501–5.

    CAS  PubMed  Google Scholar 

  181. Camposilvan S, Milanesi O, Stellin G, Pettenazzo A, Zancan L, D’Antiga L. Liver and cardiac function in the long term after Fontan operation. Ann Thorac Surg. 2008;86:177–82.

    PubMed  Google Scholar 

  182. van Nieuwenhuizen RC, Peters M, Lubbers LJ, Trip MD, Tijssen JG, Mulder BJ. Abnormalities in liver function and coagulation profile following the Fontan procedure. Heart. 1999;82:40–6.

    PubMed Central  PubMed  Google Scholar 

  183. Green A. Outcomes of congenital heart disease: a review. Pediatr Nurs. 2004;30:280–4.

    PubMed  Google Scholar 

  184. Dearani JA, Connolly HM, Martinez R, Fontanet H, Webb GD. Caring for adults with congenital cardiac disease: successes and challenges for 2007 and beyond. Cardiol Young. 2007;17 Suppl 2:87–96.

    PubMed  Google Scholar 

  185. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JI, Somerville J, Williams RG, Webb GD. Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol. 2001;37:1170–5.

    CAS  PubMed  Google Scholar 

  186. Marino BS, Tomlinson RS, Wernovsky G, Drotar D, Newburger JW, Mahony L, Mussatto K, Tong E, Cohen M, Andersen C, Shera D, Khoury PR, Wray J, Gaynor JW, Helfaer MA, Kazak AE, Shea JA, Pediatric Cardiac Quality of Life Inventory Testing Study Consortium. Validation of the pediatric cardiac quality of life inventory. Pediatrics. 2010;126(3):498–508.

    PubMed Central  PubMed  Google Scholar 

  187. Marino BS, Tomlinson RS, Drotar D, Claybon ES, Aguirre A, Ittenbach R, Welkom JS, Helfaer MA, Wernovsky G, Shea JA. Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease. Pediatrics. 2009;123(4):e708–15.

    PubMed  Google Scholar 

  188. Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, Mussatto KA, Uzark K, Goldberg CS, Johnson WH Jr, Li J, Smith SE, Bellinger DC, Mahle WT, on behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, and Stroke Council. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012;126:1143–72.

    Google Scholar 

  189. Marino BS. New concepts in predicting, evaluating, and managing neurodevelopmental outcomes in children with congenital heart disease. Curr Opin Pediatr. 2013;25:574–84.

    Google Scholar 

  190. Byrne A, MacDonald J, Buckley S. Reading, language and memory skills: a comparative longitudinal study of children with Down syndrome and their mainstream peers. Br J Educ Psychol. 2002;72(Pt 4):513–29.

    PubMed  Google Scholar 

  191. Brugge KL, Nichols SL, Salmon DP, Hill LR, Delis DC, Aaron L, Trauner DA. Cognitive impairment in adults with Down’s syndrome: similarities to early cognitive changes in Alzheimer’s disease. Neurology. 1994;44:232–8.

    CAS  PubMed  Google Scholar 

  192. Moss EM, Batshaw ML, Solot CB, Gerdes M, McDonald-McGinn DM, Driscoll DA, Emanuel BS, Zackai EH, Wang PP. Psychoeducational profile of the 22q11.2 microdeletion: a complex pattern. J Pediatr. 1999;134:193–8.

    CAS  PubMed  Google Scholar 

  193. Bearden CE, Woodin MF, Wang PP, Moss E, McDonald-McGinn D, Zackai E, Emannuel B, Cannon TD. The neurocognitive phenotype of the 22q11.2 deletion syndrome: selective deficit in visual-spatial memory. J Clin Exp Neuropsychol. 2001;23:447–64.

    CAS  PubMed  Google Scholar 

  194. Swillen A, Fryns JP, Kleczkowska A, Massa G, Vanderschueren-Lodeweyckx M, Van den Berghe H. Intelligence, behaviour and psychosocial development in turner syndrome. A cross-sectional study of 50 pre-adolescent and adolescent girls (4–20 years). Genet Couns. 1993;4:7–18.

    CAS  PubMed  Google Scholar 

  195. Temple CM, Carney RA. Intellectual functioning of children with turner syndrome: a comparison of behavioural phenotypes. Dev Med Child Neurol. 1993;35:691–8.

    CAS  PubMed  Google Scholar 

  196. Grossfeld PD, Mattina T, Lai Z, Favier R, Jones KL, Cotter F, Jones C. The 11q terminal deletion disorder: a prospective study of 110 cases. Am J Med Genet A. 2004;129A:51–61.

    PubMed  Google Scholar 

  197. Raqbi F, Le Bihan C, Morisseau-Durand MP, Dureau P, Lyonnet S, Abadie V. Early prognostic factors for intellectual outcome in CHARGE syndrome. Dev Med Child Neurol. 2003;45:483–8.

    CAS  PubMed  Google Scholar 

  198. Bellinger DC, Wypij D, Kuban KC, Rappaport LA, Hickey PR, Wernovsky G, Jonas RA, Newburger JW. Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. Circulation. 1999;100:526–32.

    CAS  PubMed  Google Scholar 

  199. Mahle WT, Clancy RR, Moss EM, Gerdes M, Jobes DR, Wernovsky G. Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics. 2000;105:1082–9.

    CAS  PubMed  Google Scholar 

  200. Wernovsky G, Stiles KM, Gauvreau K, Gentles TL, duPlessis AJ, Bellinger DC, Walsh AZ, Burnett J, Jonas RA, Mayer Jr JE, Newburger JW. Cognitive development after the Fontan operation. Circulation. 2000;102:883–9.

    CAS  PubMed  Google Scholar 

  201. Forbess JM, Visconti KJ, Bellinger DC, Jonas RA. Neurodevelopmental outcomes in children after the Fontan operation. Circulation. 2001;104(12 Suppl 1):I127–32.

    CAS  PubMed  Google Scholar 

  202. Hovels-Gurich HH, Konrad K, Skorzenski D, Nacken C, Minkenberg R, Messmer BJ, Seghaye MC. Long-term neurodevelopmental outcome and exercise capacity after corrective surgery for tetralogy of Fallot or ventricular septal defect in infancy. Ann Thorac Surg. 2006;81:958–66.

    PubMed  Google Scholar 

  203. Bellinger DC, Wypij D, DuPlessis AJ, Rappaport LA, Jonas RA, Wernovsky G, Newburger JW. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg. 2003;126:1385–96.

    PubMed  Google Scholar 

  204. Kirshbom PM, Flynn TB, Clancy RR, Ittenbach RF, Hartman DM, Paridon SM, Wernovsky G, Spray TL, Gaynor JW. Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 2005;129:1091–7.

    PubMed  Google Scholar 

  205. Antshel KM, Faraone SV, Fremont W, Monuteaux MC, Kates WR, Doyle A, Mick E, Biederman J. Comparing ADHD in velocardiofacial syndrome to idiopathic ADHD. J Atten Disord. 2007;11:64–73.

    PubMed  Google Scholar 

  206. Niklasson L, Rasmussen P, Oskarsdottir S, Gillberg C. Autism, ADHD, mental retardation and behavior problems in 100 individuals with 22q11 deletion syndrome. Res Dev Disabil. 2009;30:763–73.

    PubMed  Google Scholar 

  207. Sznajer Y, Keren B, Baumann C, Pereira S, Alberti C, Elion J, Cave H, Verloes A. The spectrum of cardiac anomalies in Noonan syndrome as a result of mutations in the PTPN11 gene. Pediatrics. 2007;119:e1325–31.

    PubMed  Google Scholar 

  208. Bellinger DC. Are children with congenital cardiac malformations at increased risk of deficits in social cognition? Cardiol Young. 2008;18:3–9.

    PubMed  Google Scholar 

  209. Brune M, Brune-Cohrs U. Theory of mind: evolution, ontogeny, brain mechanisms and psychopathology. Neurosci Biobehav Rev. 2006;30:437–55.

    PubMed  Google Scholar 

  210. Rappaport LA, Wypij D, Bellinger DC, Helmers SL, Holmes GL, Barnes PD, Wernovsky G, Kuban KC, Jonas RA, Newburger JW. Relation of seizures after cardiac surgery in early infancy to neurodevelopmental outcome: Boston Circulatory Arrest Study Group. Circulation. 1998;97:773–9.

    CAS  PubMed  Google Scholar 

  211. Bellinger DC, Bernstein JH, Kirkwood MW, Rappaport LA, Newburger JW. Visual-spatial skills in children after open-heart surgery. J Dev Behav Pediatr. 2003;24:169–79.

    PubMed  Google Scholar 

  212. Shillingford AJ, Glanzman MM, Ittenbach RF, Clancy RR, Gaynor JW, Wernovsky G. Inattention, hyperactivity, and school performance in a population of school-age children with complex congenital heart disease. Pediatrics. 2008;121:e759–67.

    PubMed  Google Scholar 

  213. Hovels-Gurich HH, Konrad K, Skorzenski D, Herpertz-Dahlmann B, Messmer BJ, Seghaye MC. Attentional dysfunction in children after corrective cardiac surgery in infancy. Ann Thorac Surg. 2007;83:1425–30.

    PubMed  Google Scholar 

  214. Hoffman GM, Mussatto KA, Brosig CL, Ghanayem NS, Musa N, Fedderly RT, Jaquiss RD, Tweddell JS. Systemic venous oxygen saturation after the Norwood procedure and childhood neurodevelopmental outcome. J Thorac Cardiovasc Surg. 2005;130:1094–100.

    PubMed  Google Scholar 

  215. Brosig CL, Mussatto KA, Kuhn EM, Tweddell JS. Neurodevelopmental outcome in preschool survivors of complex congenital heart disease: implications for clinical practice. J Pediatr Health Care. 2007;21:3–12.

    PubMed  Google Scholar 

  216. Miatton M, De Wolf D, François K, Thiery E, Vingerhoets G. Neuropsychological performance in school-aged children with surgically corrected congenital heart disease. J Pediatr. 2007;151:73–8, 78.e1.

    PubMed  Google Scholar 

  217. Miatton M, De Wolf D, François K, Thiery E, Vingerhoets G. Intellectual, neuropsychological, and behavioral functioning in children with tetralogy of Fallot. J Thorac Cardiovasc Surg. 2007;133:449–55.

    PubMed  Google Scholar 

  218. Marino BS, Beebe D, Cassedy A, Riedel M, Burger M, Medek S, Finan S, Andersen C, Uzark K, Ross J, Ittenbach RF, Drotar D. Executive functioning, gross motor ability and mood are key drivers of poorer quality of life in child and adolescent survivors with complex congenital heart disease. J Am Coll Cardiol. 2011;57:E421.

    Google Scholar 

  219. Williams DL, Gelijns AC, Moskowitz AJ, Weinberg AD, Ng JH, Crawford E, Hayes CJ, Quaegebeur JM. Hypoplastic left heart syndrome: valuing the survival. J Thorac Cardiovasc Surg. 2000;119:720–31.

    CAS  PubMed  Google Scholar 

  220. Goldberg CS, Schwartz EM, Brunberg JA, Mosca RS, Bove EL, Schork MA, Stetz SP, Cheatham JP, Kulik TJ. Neurodevelopmental outcome of patients after the Fontan operation: a comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions. J Pediatr. 2000;137:646–52.

    CAS  PubMed  Google Scholar 

  221. Hovels-Gurich HH, Konrad K, Skorzenski D, Minkenberg R, Herpertz-Dahlmann B, Messmer BJ, Seghaye MC. Long-term behavior and quality of life after corrective cardiac surgery in infancy for tetralogy of Fallot or ventricular septal defect. Pediatr Cardiol. 2007;28:346–54.

    CAS  PubMed  Google Scholar 

  222. Birkeland AL, Rydberg A, Hagglof B. The complexity of the psychosocial situation in children and adolescents with heart disease. Acta Paediatr. 2005;94:1495–501.

    PubMed  Google Scholar 

  223. Karsdorp PA, Everaerd W, Kindt M, Mulder BJ. Psychological and cognitive functioning in children and adolescents with congenital heart disease: a meta-analysis. J Pediatr Psychol. 2007;32:527–41.

    PubMed  Google Scholar 

  224. Lambert LM, Minich LL, Newburger JW, Lu M, Pemberton VL, McGrath EA, Atz AM, Xu M, Radojewski E, Servedio D, McCrindle BW, Pediatric Heart Network Investigators. Parent-versus child reported functional health status after the Fontan procedure. Pediatrics. 2009;124:e942–9.

    PubMed Central  PubMed  Google Scholar 

  225. Hovels-Gurich H, Konrad K, Wiesner M, Minkenberg R, Herpertz-Dahlmann B, Messmer B, Von Bernuth G. Long term behavioural outcome after neonatal arterial switch operation for transposition of the great arteries. Arch Dis Child. 2002;87:506–10.

    CAS  PubMed Central  PubMed  Google Scholar 

  226. Miatton M, De Wolf D, François K, Thiery E, Vingerhoets G. Behavior and self-perception in children with a surgically corrected congenital heart disease. J Dev Behav Pediatr. 2007;28:294–301.

    PubMed  Google Scholar 

  227. Pierpont ME, Basson CT, Benson 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;115:3015–38.

    PubMed  Google Scholar 

  228. Musewe NN, Alexander DJ, Teshima I, et al. Echocardiographic evaluation of the spectrum of cardiac anomalies associated with trisomy 13 and trisomy 18. J Am Coll Cardiol. 1990;15:673–7.

    CAS  PubMed  Google Scholar 

  229. Lehman CD, Nyberg DA, Winter 3rd TC, et al. Trisomy 13 syndrome: prenatal US findings in a review of 33 cases. Radiology. 1995;194:217–22.

    CAS  PubMed  Google Scholar 

  230. Van Praagh S, Truman T, Firpo A, et al. Cardiac malformations in trisomy 18. A study of 41 postmortem cases. J Am Coll Cardiol. 1989;13:1586–97.

    PubMed  Google Scholar 

  231. Ferencz C, Loffredo CA, Corea-Villasenor A, et al. Genetic and environmental risk factors of major congenital heart defects: the Baltimore-Washington infant study: 1981–1989. Armonk: Futura Publishing; 1997.

    Google Scholar 

  232. Freeman SB, Taft LF, Dooley KJ, et al. Population-based study of congenital heart defects in Down syndrome. Am J Med Genet. 1998;80:213–7.

    CAS  PubMed  Google Scholar 

  233. McElhinney DB, Straka M, Goldmuntz E, et al. Correlation between abnormal cardiac physical examination and echocardiographic findings in neonates with Down syndrome. Am J Med Genet. 2002;113:238–41.

    PubMed  Google Scholar 

  234. Mazzanti L, Cacciari E. Congenital heart disease in patients with Turner syndrome. Italian Study Group for Turner Syndrome (ISGTS). J Pediatr. 1998;133:688–92.

    CAS  PubMed  Google Scholar 

  235. Ho VB, Bakalov VK, Cooley M, et al. Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. Circulation. 2004;110:1694–700.

    PubMed  Google Scholar 

  236. Gravholt CH, Landin-Wilhelmsen K, Stochholm K, et al. Clinical and epidemiological description of aortic dissection in Turner’s syndrome. Cardiol Young. 2006;16:430–6.

    PubMed  Google Scholar 

  237. Bondy CA. The turner syndrome consensus study group: guidelines for the care of girls and women with turner syndrome. J Clin Endocrinol Metab. 2007;92(1):10–25.

    CAS  PubMed  Google Scholar 

  238. Smoot L, Zhang H, Klaiman C, et al. Medical overview and genetics of Williams-Beuren syndrome. Prog Pediatr Cardiol. 2005;20:195–205.

    Google Scholar 

  239. Grossfeld PD, Mattina T, Lai Z, et al. The 11q terminal deletion disorder: a prospective study of 110 cases. Am J Med Genet. 2004;129:51–61.

    Google Scholar 

  240. Berends MJ, Tan-Sindhunata G, Leegte B, et al. Phenotypic variability of cat-eye syndrome. Genet Couns. 2001;12:23–34.

    CAS  PubMed  Google Scholar 

  241. McDonald-McGinn DM, LaRossa D, Goldmuntz E, et al. The 22q11.2 deletion: screening, diagnostic workup, and outcome of results; report on 181 patients. Genet Test. 1997;1:99–108.

    CAS  PubMed  Google Scholar 

  242. Goldmuntz E, Clark BJ, Mitchell LE, et al. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol. 1998;32:492–8.

    CAS  PubMed  Google Scholar 

  243. Spinner NB, Colliton RP, Crosnier C, et al. Jagged1 mutations in Alagille syndrome. Hum Mutat. 2001;17:18–33.

    CAS  PubMed  Google Scholar 

  244. Sweeney E, Fryer A, Walters M. Char syndrome: a new family and review of the literature emphasizing the presence of symphalangism and the variable phenotype. Clin Dysmorphol. 2000;9:177–82.

    CAS  PubMed  Google Scholar 

  245. Lalani SR, Safiullah AM, Fernbach SD, et al. Spectrum of CHD7 mutations in 110 individuals with CHARGE syndrome and genotype-phenotype correlation. Am J Hum Genet. 2006;78:303–14.

    CAS  PubMed Central  PubMed  Google Scholar 

  246. Jackson L, Kline AD, Barr MA, et al. de Lange syndrome: a clinical review of 310 individuals. Am J Med Genet. 1993;47:940–6.

    CAS  PubMed  Google Scholar 

  247. Lin AE, Grossfeld PD, Hamilton RM, et al. Further delineation of cardiac abnormalities in Costello syndrome. Am J Med Genet. 2002;111:115–29.

    PubMed  Google Scholar 

  248. Gripp KW, Stabley DL, Nicholson L, et al. Somatic mosaicism for an HRAS mutation causes Costello syndrome. Am J Med Genet. 2006;140:2163–9.

    PubMed  Google Scholar 

  249. Sletten LJ, Pierpont ME. Variation in severity of cardiac disease in Holt-Oram syndrome. Am J Med Genet. 1996;65:128–32.

    CAS  PubMed  Google Scholar 

  250. Sarkozy A, Conti E, Seripa D, et al. Correlation between PTPN11 gene mutations and congenital heart defects in Noonan and LEOPARD syndromes. J Med Genet. 2003;40:704–8.

    CAS  PubMed Central  PubMed  Google Scholar 

  251. Keren B, Hadchouel A, Saba S, et al. PTPN11 mutations in patients with LEOPARD syndrome: a French multicentric experience. J Med Genet. 2004;41:e117.

    CAS  PubMed Central  PubMed  Google Scholar 

  252. Noonan JA. Noonan syndrome and related disorders. Prog Pediatr Cardiol. 2005;20:177–85.

    Google Scholar 

  253. Zenker M, Lehmann K, Schulz AL, et al. Expansion of the genotypic and phenotypic spectrum in patients with KRAS germline mutations. J Med Genet. 2007;44:131–5.

    CAS  PubMed Central  PubMed  Google Scholar 

  254. Roberts AE, Araki T, Swanson KD, et al. Germlinegain-of-function mutations in SOS1 cause Noonan syndrome. Nat Genet. 2007;29:70–4.

    Google Scholar 

  255. Stevens CA, Bhakta MG. Cardiac abnormalities in the Rubinstein-Taybi syndrome. Am J Med Genet. 1995;59:346–8.

    CAS  PubMed  Google Scholar 

  256. Lin AE, Ardinger HH, Ardinger Jr RH, et al. Cardiovascular malformations in Smith-Lemli-Opitz syndrome. Am J Med Genet. 1997;68:270–8.

    CAS  PubMed  Google Scholar 

  257. Surka WS, Kohlhase J, Neunert CE, et al. Unique family with Townes-Brocks syndrome, SALL1 mutations, and cardiac defects. Am J Med Genet. 2001;102:250–7.

    CAS  PubMed  Google Scholar 

  258. Wernovsky G. Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease. Cardiol Young. 2006;16 Suppl 1:92–104.

    PubMed  Google Scholar 

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Correspondence to Bradley S. Marino MD, MPP, MSCE .

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Heydarian, H.C., Madsen, N.L., Marino, B.S. (2014). Long-Term Outcomes in Congenital Heart Disease. In: Wheeler, D., Wong, H., Shanley, T. (eds) Pediatric Critical Care Medicine. Springer, London. https://doi.org/10.1007/978-1-4471-6356-5_25

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