The principles of management, and outcomes for, patients with functionally univentricular hearts

Paediatric Cardiology

Volumn , Issue , 2010, Pages 687-696

  Hutter, Damian ^a   Redington, Andrew N ^b,c  

^a NATIONAL INSTITUTE ON AGING   (United States)

^b HOSPITAL FOR SICK CHILDREN   (Canada)

^c UNIVERSITY OF TORONTO   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 84865483606     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1016/B978-0-7020-3064-2.00035-7     Document Type: Chapter

References (114)

1. Peacock T.B. Malformations of the heart. On Malformations of the Human Heart: With Original Cases 1858, 10-102. John Churchill, London, UK, (Chemineau: Sur la structure extraordinaire due coeur d'un foetus humain. Hist Acad Ray 37-38, 1699.). T.B. Peacock (Ed.).
2. Van Praagh R., Ongely P.A., Swan H.J. Anatomic types of single or common ventricle in man: Morphologic and geometric aspects of 60 necrobiopsied cases. Am J Cardiol 1964, 13:367-386.
3. Anderson R.H., Becker A.E., Tynan M., et al. The univentricular atrioventricular connection: Getting to the root of a thorny problem. Am J Cardiol 1984, 54:822-828.
4. Jacobs M.L., Mayer J.E. Congenital Heart Surgery Nomenclature and Database Project: Single ventricle. Ann Thorac Surg 2000, 69(4 Suppl):197-204.
5. Jacobs M.L., Anderson R.H. Nomenclature of the functionally univentricular heart. Cardiol Young 2006, 16(Suppl 1):3-8.
6. Moodie D.S., Ritter D.G., Tajik A.J., O'Fallon W.M. Long-term follow-up in the unoperated univentricular heart. Am J Cardiol 1984, 53:1124-1128.
7. Franklin R.C.G., Spiegelhalter D.J., Anderson R.H., et al. Double-inlet ventricle presenting in infancy. II. Results of palliative operations. J Thorac Cardiovasc Surg 1991, 101:767-776.
8. Restino G., Dirksen M.S., de Roos A. Long-term survival in a case of unoperated single ventricle. Int J Cardiovasc Imaging 2004, 20:221-225.
9. Koito H., Ohkubo N., Suzuki J., et al. Prolonged survival in a patient with a single ventricle without pulmonary stenosis. Chest 1994, 106:971-972.
10. Vitarelli A., Gabbarini F. Holmes heart in the adult: Transesophageal echocardiographic findings and long-term natural survival. Int J Cardiol 1996, 56:301-305.
11. Ammash N.M., Warnes C.A. Survival into adulthood of patients with unoperated single ventricle. Am J Cardiol 1996, 77:542-544.
12. PerloffJ.K., Rosove M.H., Child J.S., Wright G.B. Adults with cyanotic congenital heart disease: Hematologic management. Ann Intern Med 1988, 109:406-413.
13. Oechslin E. Hematological management of the cyanotic adult with congenital heart disease. Int J Cardiol 2004, 1997(Suppl 1):109-115.
14. Lill M.C., PerloffJ.K., Child J.S. Pathogenesis of thrombocytopenia in cyanotic congenital heart disease. Am J Cardiol 2006, 98:254-258.
15. PerloffJ.K., Marelli A.J., Miner P.D. Risk of stroke in adults with cyanotic congenital heart disease. Circulation 1993, 87:1954-1959.
16. McLaughlin G.E., McCarty D.J., Downing D.F. Hypertrophic osteoarthropathy associated with cyanotic congenital heart disease. Ann Intern Med 1967, 67:579-587.
17. Ammash N.M., Connolly H.M., Abel M.D., Warnes C.A. Noncardiac surgery in Eisenmenger syndrome. J Am Coll Cardiol 1999, 33:222-227.
18. Liberthson R.R. Eisenmenger's physiology, pulmonary vascular obstruction. Congenital Heart Disease: Diagnosis and Management in Children and Adults 1989, 87-93. Little, Brown, Boston. R.R. Liberthson (Ed.).
19. Humes R.A., Mair D.D., Porter C.B., et al. Results of the modified Fontan operation in adults. Am J Cardiol 1988, 61:602-604.
20. Veldtman G.R., Nishimoto A., Siu S., et al. The Fontan procedure in adults. Heart 2001, 86:330-335.
21. Kopf G.S., Laks H., Stansel H.C., et al. Thirty-year follow-up of superior vena cava-pulmonary artery (Glenn) shunts. J Thorac Cardiovasc Surg 1990, 100:662-671.
22. Mott A.R., Feltes T.F., McKenzie E.D., et al. Improved early results with the Fontan operation in adults with functional single ventricle. Ann Thorac Surg 2004, 77:1334-1340.
23. Williams J.A., Bansal A.K., Kim B.J., et al. Two thousand Blalock-Taussig shunts: A six-decade experience. Ann Thorac Surg 2007, 84:2070-2075.
24. Macé L., Dervanian P., Losay J., et al. Bidirectional inferior vena cava-pulmonary artery shunt. Ann Thorac Surg 1997, 63:1321-1325.
25. Freedom R.M., Sondheimer H., Sische R., Rowe R.D. Development of "subaortic stenosis" after pulmonary arterial banding for common ventricle. Am J Cardiol 1977, 39:78-83.
26. Cheung H.C., Lincoln C., Anderson R.H., et al. Options for surgical repair in hearts with univentricular atrioventricular connection and subaortic stenosis. J Thorac Cardiovasc Surg 1990, 100:672-681.
27. DeLeon S.Y., Ilbawi M.N., Tubeszewski K., et al. The Damus-Stansel-Kaye procedure: Anatomical determinants and modifications. Ann Thorac Surg 1991, 52:680-687.
28. Glenn W.W.L. Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal pulmonary artery: Report of clinical application. N Engl J Med 1958, 259:117-120.
29. Robicsek F., Sanger P.W., Gollucci V., et al. Long-term circulatory exclusion of the right heart. Surgery 1966, 59:431-437.
30. Fontan F., Baudet E. Surgical repair of tricuspid atresia. Thorax 1971, 26:240-248.
31. Sade R.M., Castaneda A.R. The dispensable right ventricle. Surgery 1975, 77:624-631.
32. Kurocynski W. Development of the Fontan procedure as therapeutic concept for children with complex congenital heart disease. Herz 2007, 32:241-247.
33. Bjork V.O., Olin C.L., Bjarke B.B., et al. Right atrial-right ventricular anastomosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg 1979, 77:452-458.
34. Doty W.J.M., Lauer R.M. Modified Fontan procedure: Methods to achieve direct anastomosis of right atrium to pulmonary artery. J Thorac Cardiovasc Surg 1981, 81:470-475.
35. Hopkins R.A., Armstrong B.E., Serwer G.A., et al. Physiological rationale for a bidirectional cavopulmonary shunt. A versatile complement to the Fontan principle. J Thorac Cardiovasc Surg 1985, 90:391-398.
36. Humes R.A., Feldt R.H., Porter C.J., et al. The modified Fontan operation for asplenia and polysplenia syndromes. J Thorac Cardiovasc Surg 1988, 96:212-218.
37. Lee C.-N., SchaffH.V., Danielson G.K., et al. Comparison of atriopulmonary versus atrioventricular connections for modified Fontan/Kreutzer repair of tricuspid valve atresia. J Thorac Cardiovasc Surg 1986, 92:1038-1048.
38. Levalde M.R., Kilner P., Gewillig M., et al. Total cavopulmonary connection: A logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988, 96:682-695.
39. Marcelletti C., Corno A., Giannico S., et al. Inferior vena cava-pulmonary artery extracardiac conduit. J Thorac Cardiovasc Surg 1990, 100:228-232.
40. Sievers H.-H., Gerdes A., Kunze J., et al. Superior hydrodynamics of a modified cavopulmonary connection for the Norwood operation. Ann Thorac Surg 1998, 65:1741-1745.
41. Fontan F., Kirklin J.W., Fernandez G., et al. Outcome of the perfect Fontan operation. Circulation 1990, 81:1520-1536.
42. Stamm C., Friehs I., Mayer J.E., et al. Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg 2001, 121:28-41.
43. Kumar S.P., Rubinstein C.S., Simsic J.M., et al. Lateral tunnel versus extracardiac conduit Fontan procedure: A concurrent comparison. Ann Thorac Surg 2003, 76:1389-1397.
44. Giannico S., Hammad F., Amodeo A., et al. Clinical outcome of 193 extracardiac Fontan patients. J Am Coll Cardiol 2006, 47:2065-2073.
45. D'Udekem Y., Iyengar A.J., Cochrane A.D., et al. The Fontan procedure: Contemporary techniques have improved long-term outcomes. Circulation 2007, 116(Suppl 11):I157-I164.
46. Nakanishi T. Cardiac catheterization is necessary before bidirectional Glenn and Fontan procedures in single ventricle physiology. Pediatr Cardiol 2005, 26:159-161.
47. Redington A.N., Knight B., Oldershaw P.J., et al. Left ventricular function in double inlet left ventricle before the Fontan operation: Comparison with tricuspid atresia. Br Heart J 1988, 60:324-331.
48. Gaynor J.W., Bridges N.D., Cohen M.I., et al. Predictors of outcome after the Fontan operation: Is hypoplastic left heart syndrome still a risk factor?. J Thorac Cardiovasc Surg 2002, 123:237-245.
49. Gentles T.L., Mayer J.E., Gauvreau K., et al. Fontan operation in five hundred consecutive patients: Factors influencing early and late outcome. J Thorac Cardiovasc Surg 1997, 114:376-391.
50. Sluysmans T., Sanders S.P., 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.
51. Gewillig M., Daenen W., Aubert A., Van der Hauwaert L. Abolishment of chronic volume overload. Implications for diastolic function of the systemic ventricle immediately after Fontan repair. Circulation 1992, 86(Suppl 5):II93-II99.
52. Penny D.J., Lincoln C., Shore D.F., et al. The early response of the systemic ventricle during transition to the Fontan circulation: An acute hypertrophic cardiomyopathy?. Cardiol Young 1992, 2:78-84.
53. Penny D., Redington A.N. Diastolic ventricular function after the Fontan operation. Am J Cardiol 1992, 69:974-975.
54. Border W.L., Syed A.U., Michelfelder E.C., et al. Impaired systemic ventricular relaxation affects postoperative short-term outcome in Fontan patients. J Thorac Cardiovasc Surg 2003, 126:1760-1764.
55. Penny D.J., Redington A.N. Angiographic demonstration of incoordinate motion of the ventricular wall after the Fontan operation. Br Heart J 1991, 66:456-459.
56. Penny D.J., Rigby M.L., Redington A.N. Abnormal patterns of intraventricular flow and diastolic filling after the Fontan operation: Evidence for incoordinate ventricular wall motion. Br Heart J 1991, 66:375-378.
57. Cheung Y.F., Penny D.J., Redington A.N. Serial assessment of left ventricular diastolic function after Fontan procedure. Heart 2000, 83:420-424.
58. Cheitlin M.D. Cardiovascular physiology-changes with aging. Am J Geriatr Cardiol 2003, 12:9-13.
59. Erenberg F.G., Banerjee A. Systolic and diastolic properties of univentricular hearts in children: Insights from physiologic indices that reflect calcium cycling. Pediatr Res 2003, 54:885-891.
60. Cheung M., Smallhorn J.F., Van Arsdell G.S., Redington A.N. Systemic right ventricular force-frequency relationships are superior to those of the left ventricle in the univentricular circulation. Circulation 2003, 108(Suppl 3). 1286.
61. Alpert N.R., Leavitt B.J., Ittleman F.P., et al. A mechanistic analysis of the force-frequency relation in non-failing and progressively failing human myocardium. Basic Res Cardiol 1998, 93(Suppl 1):23-32. Cited by Scopus (32).
62. Bolger A.P., Sharma R., Li W., et al. Neurohormonal activation and the chronic heart failure syndrome in adults with congenital heart disease. Circulation 2002, 106:92-99.
63. Sanders S.P., Wright G.B., Keane J.F., et al. Clinical and hemodynamic results of the Fontan operation for tricuspid atresia. Am J Cardiol 1982, 49:1733-1740.
64. 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, 105:2885-2892.
65. Kouatli A.A., Garcia J.A., Zellers T.M., et al. Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation 1997, 96:1507-1512.
66. Rosenthal M., Bush A., Deanfield J., Redington A.N. Comparison of cardiopulmonary adaptation during exercise in children after the atriopulmonary and total cavopulmonary connection Fontan procedures. Circulation 1995, 91:372-378.
67. Redington A.N., Penny D.J., Shinebourne E.A. Pulmonary blood flow after total cavopulmonary shunt. Br Heart J 1991, 65:213-217.
68. Penny D.J., Redington A.N. Doppler echocardiographic evaluation of pulmonary blood flow after the Fontan operation: The role of the lungs. Br Heart J 1991, 66:372-374.
69. Fogel M.A., Weinberg P.M., Rychik J., et al. Caval contribution to flow in the branch pulmonary arteries of Fontan patients with a novel application of magnetic resonance presaturation pulse. Circulation 1999, 99:1215-1221.
70. Hsia T.Y., Khambadkone S., Redington A.N., et al. Effects of respiration and gravity on infradiaphragmatic venous flow in normal and Fontan patients. Circulation 2000, 102(19 Suppl. 3):III148-III153.
71. Williams D.B., Kiernan P.D., Metke M.P., et al. Hemodynamic response to positive end-expiratory pressure following right atrium-pulmonary artery bypass (Fontan procedure). J Thorac Cardiovasc Surg 1984, 87:856-861.
72. Shekerdemian L.S., Bush A., Shore D.F., et al. Cardiopulmonary interactions after Fontan operations: Augmentation of cardiac output using negative pressure ventilation. Circulation 1997, 96:3934-3942.
73. Kreutzer J., Keane J.F., Lock J.E., et al. Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis. J Thorac Cardiovasc Surg 1996, 111:1169-1176.
74. Jahangiri M., Ross D.B., Redington A.N., et al. Thromboembolism after the Fontan procedure and its modifications. Ann Thorac Surg 1994, 58:1409-1413.
75. Varma C., Warr M.R., Hendler A.L., et al. Prevalence of "silent" pulmonary emboli in adults after the Fontan operation. J Am Coll Cardiol 2003, 41:2252-2258.
76. Bartmus D.A., Driscoll D.J., Offord K.P., et al. The modified Fontan operation for children less than 4 years old. J Am Coll Cardiol 1990, 15:429-435.
77. Mair D.D., Hagler D.J., Puga F.J., et al. Fontan operation in 176 patients with tricuspid atresia. Results and a proposed new index for patient selection. Circulation 1990, 82(Suppl 5):IV164-IV169.
78. Cohen M.I., Wernovsky G., Vetter V.L., et al. Sinus node function after a systematically staged Fontan procedure. Circulation 1998, 98(19 Suppl):II352-II358.
79. Triedman J.K., Alexander M.E., Berul C.I., et al. Electroanatomic mapping of entrained and exit zones in patients with repaired congenital heart disease and intra-atrial reentrant tachycardia. Circulation 2001, 103:2060-2065.
80. Triedman J.K., DeLucca J.M., Alexander M.E., et al. Prospective trial of electroanatomically guided, irrigated catheter ablation of atrial tachycardia in patients with congenital heart disease. Heart Rhythm 2005, 2:700-705.
81. Mavroudis C., Deal B.J., Backer C.L. The beneficial effects of total cavopulmonary conversion and arrhythmia surgery for the failed Fontan. Semin Thorac Cardiovasc Surg Pediatr Cardiol Surg Annu 2002, 5:12-24.
82. Jahangiri M., Kreutzer J., Zurakowski D., et al. Evaluation of hemostatic and coagulation factor abnormalities in patients undergoing the Fontan operation. J Thorac Cardiovasc Surg 2000, 120:778-782.
83. Ravn H.B., Hjortdal V.E., Stenbog E.V., et al. Increased platelet reactivity and significant changes in coagulation markers after cavopulmonary connection. Heart 2001, 85:61-65.
84. Lill M.C., PerloffJ.K., Child J.S. Pathogenesis of thrombocytopenia in cyanotic congenital heart disease. Am J Cardiol 2006, 98:254-258. (Please refer to references of the natural history of the univentricular heart.
85. Barker P.C., Nowak C., King K., et al. Risk factors for cerebrovascular events following Fontan palliation in patients with a functional single ventricle. Am J Cardiol 2005, 96:587-591.
86. Seipelt R.G., Franke A., Vazquez-Jimenez J.F., et al. Thromboembolic complications after Fontan procedures: Comparison of different therapeutic approaches. Ann Thorac Surg 2002, 74:556-562.
87. Kaulitz R., Ziemer G., Rauch R., et al. Prophylaxis of thromboembolic complications after the Fontan operation (total cavopulmonary anastomosis). J Thorac Cardiovasc Surg 2005, 129:569-575.
88. Mahnke C.B., Boyle G.J., Janosky J.E., et al. Anticoagulation and incidence of late cerebrovascular accidents following the Fontan procedure. Pediatr Cardiol 2005, 26:56-61.
89. Seipelt R.G., Franke A., Vazquez-Jimenez J.F., et al. Thromboembolic complications after Fontan procedures: Comparison of different therapeutic approaches. Ann Thorac Surg 2002, 74:556-562.
90. Cheung Y.F., Chay G.W., Chiu C.S., Cheng L.C. Long-term anticoagulation therapy and thromboembolic complications after the Fontan procedure. Int J Cardiol 2005, 102:509-513.
91. GoffD.A., Blume E.D., Gavreau K., et al. Clinical outcome of fenestrated Fontan patients after closure. The first 10 years. Circulation 2000, 102:2094-2099.
92. Mertens L., Hagler D.J., Sauer U., et al. Protein-losing enteropathy after the Fontan operaton: An international multicenterstudy. J Thorac Cardiovasc Surg 1998, 115:1063-1073.
93. Earing M.G., Cetta F., Driscoll D.J., et al. Long-term results of the Fontan operation for double-inlet left ventricle. Am J Cardiol 2005, 96:291-298.
94. Masetti P., Marianeschi S.M., Cipriani A., et al. Reversal of protein-losing enteropathy after ligation of systemic-pulmonary shunt. Ann Thorac Surg 1999, 67:235-236.
95. Haas G.S., Hess H., Black M., et al. Extracardiac conduit Fontan procedure: Early and intermediate results. Eur J Cardiothorac Surg 2000, 17:648-654.
96. Mavroudis C., Backer C.L., Deal B.J., et al. Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoablation. J Thorac Cardiovasc Surg 1998, 115:547-556.
97. Chaudhari M., Stumper O. Plastic bronchitis after Fontan operation: Treatment with stent fenestration of the Fontan circuit. Heart 2004, 90:801.
98. Tzifa A., Robards M., Simpson J.M. Plastic bronchitis; a serious complication of the Fontan operation. Int J Cardiol 2005, 101:513-514.
99. Stiller B., Riedel F., Paul K., van Landeghem F.K. Plastic bronchitis in children with Fontan palliation: Analogue to protein losing enteropathy?. Pediatr Cardiol 2002, 23:90-94.
100. Mendoza Soto A., Galletti L., Gómez de Quero P., et al. Plastic bronchitis. A case report and review of the literature. An Pediatr (Barc) 2005, 62:72-75.
101. Costello J.M., Steinhorn D., McColley S., et al. Treatment of plastic bronchitis in a Fontan patient with tissue plasminogen activator: A case report and review of the literature. Pediatrics 2002, 109. e67.
102. Wilson J., Russell J., Williams W., Benson L. Fenestration of the Fontan circuit as treatment for plastic bronchitis. Pediatr Cardiol 2005, 26:717-719.
103. Barber B.J., Burch G.H., Tripple D., Balaji S. Resolution of plastic bronchitis with atrial pacing in a patient with Fontan physiology. Pediatr Cardiol 2004, 25:73-76.
104. Apostolopoulou S.C., Papagiannis J., Rammos S. Bosentan induces clinical, exercise and hemodynamic improvement in a pre-transplant patient with plastic bronchitis after Fontan operation. J Heart Lung Transplant 2005, 24:1174-1176.
105. Haseyama K., Satomi G., Yasukochi S., et al. Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2006, 132:1232-1233.
106. Wakeham M.K., Van Bergen A.H., Torero L.E., Akhter J. Long-term treatment of plastic bronchitis with aerosolized tissue plasminogen activator in a Fontan patient. Pediatr Crit Care Med 2005, 6:76-78.
107. Zaccagni H.J., Kirchner L., Brownlee J., Bloom K. A case of plastic bronchitis presenting 9 years after Fontan. Pediatr Cardiol 2008, 29:157-159.
108. Shah S.S., Drinkwater D.C., Christian K.G. Plastic bronchitis: Is thoracic duct ligation a real surgical option?. Ann Thorac Surg 2006, 81:2281-2283.
109. Mavroudis C., Backer C.L., Deal B.J., et al. Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation. J Thorac Cardiovasc Surg 2001, 122:863-871.
110. Deal B.J., Mavroudis C., Backer C.L. Arrhythmia management in the Fontan patient. Pediatr Cardiol 2007, 28:448-456.
111. Nurnberg J.H., Ovroutski S., Alexi-Meskishvili 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, 78:1979-1988.
112. Michielon G., Parisi F., Di Carlo D., et al. Orthotopic heart transplantation for failing single ventricle physiology. Eur J Cardiothorac Surg 2003, 24:502-510.
113. Jayakumar K.A., Addonizio L.J., Kichuk-Chrisant M.R., et al. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol 2004, 44:2065-2072.
114. Bernstein D., Naftel D., Chin C., et al. Outcome of listing for cardiac transplantation for failed Fontan. A multi-institutional study. Circulation 2006, 114:273-280.