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Hypoplastic left heart syndrome: from fetus to fontan

  • Peter P. Roeleveld (a1), David M. Axelrod (a2), Darren Klugman (a3), Melissa B. Jones (a3), Nikhil K. Chanani (a4), Joseph W. Rossano (a5) and John M. Costello (a6)...
Abstract

The care of children with hypoplastic left heart syndrome is constantly evolving. Prenatal diagnosis of hypoplastic left heart syndrome will aid in counselling of parents, and selected fetuses may be candidates for in utero intervention. Following birth, palliation can be undertaken through staged operations: Norwood (or hybrid) in the 1st week of life, superior cavopulmonary connection at 4–6 months of life, and finally total cavopulmonary connection (Fontan) at 2–4 years of age. Children with hypoplastic left heart syndrome are at risk of circulatory failure their entire life, and selected patients may undergo heart transplantation. In this review article, we summarise recent advances in the critical care management of patients with hypoplastic left heart syndrome as were discussed in a focused session at the 12th International Conference of the Paediatric Cardiac Intensive Care Society held on 9 December, 2016, in Miami Beach, Florida.

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Corresponding author
Author for correspondence: P. P. Roeleveld, Department of Pediatric Intensive Care, Leiden University Medical Center, IC Kinderen, J4-32, 2300 RC, Leiden, The Netherlands. Tel: +31(0)715298486; Fax: +31(0)715266966; E-mail: p.p.roeleveld@lumc.nl
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Presented at: the 12th International Conference of the Pediatric Cardiac Intensive Care Society, Miami, Fl, 9 December, 2016.

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References
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1. Ohye, RG, Schranz, D, D’Udekem, Y. Current therapy for hypoplastic left heart syndrome and related single ventricle lesions. Circulation 2016; 134: 12651279.
2. Morris, SA, Ethen, MK, Penny, DJ, et al. Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome. Circulation 2014; 129: 285–292.
3. Atz, AM, Travison, TG, Williams, IA, et al. Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: Screening data from the pediatric heart network single ventricle reconstruction trial(*). J Thorac Cardiovasc Surg 2010; 140: 12451250.
4. van Velzen, CL, Clur, SA, Rijlaarsdam, ME, et al. Prenatal detection of congenital heart disease--results of a national screening programme. BJOG 2016; 123: 400407.
5. Donofrio, MT, Moon-Grady, AJ, Hornberger, LK, et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American heart association. Circulation 2014; 129: 21832242.
6. Patel, A, Hickey, E, Mavroudis, C, et al. Impact of noncardiac congenital and genetic abnormalities on outcomes in hypoplastic left heart syndrome. Ann Thorac Surg 2010; 89: 18051813; discussion 1813–1804.
7. Beroukhim, RS, Gauvreau, K, Benavidez, OJ, Baird, CW, LaFranchi, T, Tworetzky, W. Perinatal outcome after prenatal diagnosis of single-ventricle cardiac defects. Ultrasound Obstet Gynecol 2015; 45: 657663.
8. Rychik, J, Szwast, A, Natarajan, S, et al. Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience. Ultrasound Obstet Gynecol 2010; 36: 465470.
9. Kipps, AK, Feuille, C, Azakie, A, et al. Prenatal diagnosis of hypoplastic left heart syndrome in current era. Am J Cardiol 2011; 108: 421427.
10. Freud, LR, McElhinney, DB, Marshall, AC, et al. Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome: Postnatal outcomes of the first 100 patients. Circulation 2014; 130: 638645.
11. Moon-Grady, AJ, Morris, SA, Belfort, M, et al. International fetal cardiac intervention registry: A worldwide collaborative description and preliminary outcomes. J Am Coll Cardiol 2015; 66: 388399.
12. Emani, SM, McElhinney, DB, Tworetzky, W, et al. Staged left ventricular recruitment after single-ventricle palliation in patients with borderline left heart hypoplasia. J Am Coll Cardiol 2012; 60: 19661974.
13. Marshall, AC, Levine, J, Morash, D, et al. Results of in utero atrial septoplasty in fetuses with hypoplastic left heart syndrome. Prenat Diagn 2008; 28: 10231028.
14. Kalish, BT, Tworetzky, W, Benson, CB, et al. Technical challenges of atrial septal stent placement in fetuses with hypoplastic left heart syndrome and intact atrial septum. Catheter Cardiovasc Interv 2014; 84: 7785.
15. Lara, DA, Morris, SA, Maskatia, SA, et al. Pilot study of chronic maternal hyperoxygenation and effect on aortic and mitral valve annular dimensions in fetuses with left heart hypoplasia. Ultrasound Obstet Gynecol 2016; 48: 365372.
16. Sun, L, Macgowan, CK, Sled, JG, et al. Reduced fetal cerebral oxygen consumption is associated with smaller brain size in fetuses with congenital heart disease. Circulation 2015; 131: 13131323.
17. Limperopoulos, C, Tworetzky, W, McElhinney, DB, et al. Brain volume and metabolism in fetuses with congenital heart disease: Evaluation with quantitative magnetic resonance imaging and spectroscopy. Circulation 2010; 121: 2633.
18. Jones, HN, Olbrych, SK, Smith, KL, et al. Hypoplastic left heart syndrome is associated with structural and vascular placental abnormalities and leptin dysregulation. Placenta 2015; 36: 10781086.
19. Williams, RV, Ravishankar, C, Zak, V, et al. Birth weight and prematurity in infants with single ventricle physiology: Pediatric heart network infant single ventricle trial screened population. Congenit Heart Dis 2010; 5: 96103.
20. Costello, JM, Pasquali, SK, Jacobs, JP, et al. Gestational age at birth and outcomes after neonatal cardiac surgery: An analysis of the society of thoracic surgeons congenital heart surgery database. Circulation 2014; 129: 25112517.
21. Costello, JM, Polito, A, Brown, DW, et al. Birth before 39 weeks’ gestation is associated with worse outcomes in neonates with heart disease. Pediatrics 2010; 126: 277284.
22. Calderon, J, Stopp, C, Wypij, D, et al. Early-term birth in single-ventricle congenital heart disease after the fontan procedure: Neurodevelopmental and psychiatric outcomes. J Pediatr 2016; 179: 96103.
23. Brown, DW, Cohen, KE, O’Brien, P, et al. Impact of prenatal diagnosis in survivors of initial palliation of single ventricle heart disease: analysis of the national pediatric cardiology quality improvement collaborative database. Pediatr Cardiol 2015; 36: 314321.
24. Allen, RH, Benson, CB, Haug, LW. Pregnancy outcome of fetuses with a diagnosis of hypoplastic left ventricle on prenatal sonography. J Ultrasound Med 2005; 24: 11991203.
25. Thakur, V, Munk, N, Mertens, L, Nield, LE. Does prenatal diagnosis of hypoplastic left heart syndrome make a difference? – a systematic review. Prenat Diagn 2016; 36: 854863.
26. Hornik, CP, He, X, Jacobs, JP, et al. Relative impact of surgeon and center volume on early mortality after the norwood operation. Ann Thorac Surg 2012; 93: 19921997.
27. Pasquali, SK, Jacobs, JP, He, X, et al. The complex relationship between center volume and outcome in patients undergoing the norwood operation. Ann Thorac Surg 2012; 93: 15561562.
28. Norwood, WI, Lang, P, Casteneda, AR, Campbell, DN. Experience with operations for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1981; 82: 511519.
29. Karamlou, T, Peyvandi, S. Hybrid versus norwood: “Fifty shades of grey”. J Thorac Cardiovasc Surg 2015; 150: 456457.
30. Caldarone, CA, Benson, L, Holtby, H, Li, J, Redington, AN, Van Arsdell, GS. Initial experience with hybrid palliation for neonates with single-ventricle physiology. Ann Thorac Surg 2007; 84: 12941300.
31. O’Brien, SM, Clarke, DR, Jacobs, JP, et al. An empirically based tool for analyzing mortality associated with congenital heart surgery. J Thorac Cardiovasc Surg 2009; 138: 11391153.
32. Kogon, BE, Kanter, K, Alsoufi, B, Maher, K, Oster, ME. Outcomes and hospital costs associated with the norwood operation: Beyond morbidity and mortality. Cardiol Young 2015; 25: 853859.
33. McHugh, KE, Pasquali, SK, Hall, MA, Scheurer, MA. Impact of postoperative complications on hospital costs following the norwood operation. Cardiol Young 2016; 26: 13031309.
34. Noonan, JA, Nadas, AS. The hypoplastic left heart syndrome; an analysis of 101 cases. Pediatr Clin North Am 1958; 5: 10291056.
35. Norwood, WI, Lang, P, Hansen, DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med 1983; 308: 2326.
36. Egan, MJ, Hill, SL, Boettner, BL, Holzer, RJ, et al. Predictors of retrograde aortic arch obstruction after hybrid palliation of hypoplastic left heart syndrome. Pediatr Cardiol 2011; 32: 6775.
37. Barnea, O, Santamore, WP, Rossi, A, Salloum, E, Chien, S, Austin, EH. Estimation of oxygen delivery in newborns with a univentricular circulation. Circulation 1998; 98: 14071413.
38. Pizarro, C, Malec, E, Maher, KO, et al. Right ventricle to pulmonary artery conduit improves outcome after stage i norwood for hypoplastic left heart syndrome. Circulation 2003; 108 (Suppl 1): II155II160.
39. Wright, GE, Crowley, DC, Charpie, JR, Ohye, RG, Bove, EL, Kulik, TJ. High systemic vascular resistance and sudden cardiovascular collapse in recovering norwood patients. Ann Thorac Surg 2004; 77: 4852.
40. Ohye, RG, Ludomirsky, A, Devaney, EJ, Bove, EL. Comparison of right ventricle to pulmonary artery conduit and modified Blalock-Taussig shunt hemodynamics after the norwood operation. Ann Thorac Surg 2004; 78: 10901093.
41. Sano, S, Ishino, K, Kawada, M, Arai, S, et al. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2003; 126: 504509; discussion 509–510.
42. Karamlou, T, Overman, D, Hill, KD, et al. Stage 1 hybrid palliation for hypoplastic left heart syndrome – assessment of contemporary patterns of use: An analysis of the society of thoracic surgeons congenital heart surgery database. J Thorac Cardiovasc Surg 2015; 149: 195201; 202 e191.
43. Gibbs, JL, Rothman, MT, Rees, MR, Parsons, JM, Blackburn, ME, Ruiz, CE. Stenting of the arterial duct: a new approach to palliation for pulmonary atresia. Br Heart J 1992; 67: 240245.
44. Ohye, RG, Gaynor, JW, Ghanayem, NS, et al. Design and rationale of a randomized trial comparing the blalock-taussig and right ventricle-pulmonary artery shunts in the norwood procedure. J Thorac Cardiovasc Surg 2008; 136: 968975.
45. Ohye, RG, Sleeper, LA, Mahony, L, et al . Comparison of shunt types in the norwood procedure for single-ventricle lesions. N Engl J Med 2010; 362: 19801992.
46. Newburger, JW, Sleeper, LA, Frommelt, PC, et al. Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial. Circulation 2014; 129: 20132020.
47. Vida, VL, Bacha, EA, Larrazabal, A, et al. Surgical outcome for patients with the mitral stenosis-aortic atresia variant of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2008; 135: 339346.
48. Siehr, SL, Maeda, K, Connolly, AA, et al. Mitral stenosis and aortic atresia--a risk factor for mortality after the modified norwood operation in hypoplastic left heart syndrome. Ann Thorac Surg 2016; 101: 162167.
49. Sata, S, Sinzobahamvya, N, Arenz, C, Zartner, P, Asfour, B, Hraska, V. Restrictive atrial septum defect becomes a risk factor for norwood palliation of hypoplastic left heart syndrome only when it is combined with mitral or aortic atresia. Thorac Cardiovasc Surg 2015; 63: 354359.
50. Alsoufi, B, Mori, M, Gillespie, S, et al. Impact of patient characteristics and anatomy on results of norwood operation for hypoplastic left heart syndrome. Ann Thorac Surg 2015; 100: 591598.
51. Kalfa, D, Krishnamurthy, G, Levasseur, S, et al. Norwood stage i palliation in patients less than or equal to 2.5 kg: Outcomes and risk analysis. Ann Thorac Surg 2015; 100: 167173.
52. Shamszad, P, Gospin, TA, Hong, BJ, McKenzie, ED, Petit, CJ. Impact of preoperative risk factors on outcomes after norwood palliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2014; 147: 897901.
53. Tabbutt, S, Ghanayem, N, Ravishankar, C, et al. Risk factors for hospital morbidity and mortality after the norwood procedure: A report from the pediatric heart network single ventricle reconstruction trial. J Thorac Cardiovasc Surg 2012; 144: 882895.
54. Alsoufi, B, Manlhiot, C, Al-Ahmadi, M, et al. Older children at the time of the norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection. J Thorac Cardiovasc Surg 2011; 142: 142147 e142.
55. Lynch, JM, Buckley, EM, Schwab, PJ, et al. Time to surgery and preoperative cerebral hemodynamics predict postoperative white matter injury in neonates with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2014; 148: 21812188.
56. Taeed, R, Schwartz, SM, Pearl, JM, et al. Unrecognized pulmonary venous desaturation early after norwood palliation confounds gp:Gs assessment and compromises oxygen delivery. Circulation 2001; 103: 26992704.
57. Gupta, P, Jacobs, JP, Pasquali, SK, et al. Epidemiology and outcomes after in-hospital cardiac arrest after pediatric cardiac surgery. Ann Thorac Surg 2014; 98: 21382143; discussion 2144.
58. Marino, BS, Tabbutt, S, MacLaren, G, et al. Cardiopulmonary resuscitation in infants and children with cardiac disease: A scientific statement from the American heart association. Circulation 2018; 137: e691e782.
59. Sherwin, ED, Gauvreau, K, Scheurer, MA, et al. Extracorporeal membrane oxygenation after stage 1 palliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2012; 144: 13371343.
60. Allan, CK, Thiagarajan, RR, del Nido, PJ, Roth, SJ, Almodovar, MC, Laussen, PC. Indication for initiation of mechanical circulatory support impacts survival of infants with shunted single-ventricle circulation supported with extracorporeal membrane oxygenation. J Thorac Cardiovasc Surg 2007; 133: 660667.
61. Jaggers, JJ, Forbess, JM, Shah, AS, et al. Extracorporeal membrane oxygenation for infant postcardiotomy support: Significance of shunt management. Ann Thorac Surg 2000; 69: 14761483.
62. Roeleveld, PP, Wilde, R, Hazekamp, M, Rycus, PT, Thiagarajan, RR. Extracorporeal membrane oxygenation in single ventricle lesions palliated via the hybrid approach. World J Pediatr Congenit Heart Surg 2014; 5: 393397.
63. Polimenakos, AC, Rizzo, V, El-Zein, CF, Ilbawi, MN. Post-cardiotomy rescue extracorporeal cardiopulmonary resuscitation in neonates with single ventricle after intractable cardiac arrest: Attrition after hospital discharge and predictors of outcome. Pediatr Cardiol 2017; 38: 314323.
64. Friedland-Little, JM, Uzark, K, Yu, S, Lowery, R, Aiyagari, R, Hirsch-Romano, JC. Functional status and quality of life in survivors of extracorporeal membrane oxygenation after the norwood operation. Ann Thorac Surg 2017; 103: 19501955.
65. Elias, MD, Achuff, BJ, Ittenbach, RF, et al. Long-term outcomes of pediatric cardiac patients supported by extracorporeal membrane oxygenation. Pediatr Crit Care Med 2017; 18: 787794.
66. Poh, CL, Chiletti, R, Zannino, D, et al. Ventricular assist device support in patients with single ventricles: The melbourne experience. Interact Cardiovasc Thorac Surg. 2017; 25: 310–316.
67. Pasquali, SK, Ohye, RG, Lu, M, et al. Variation in perioperative care across centers for infants undergoing the norwood procedure. J Thorac Cardiovasc Surg 2012; 144: 915921.
68. Feinstein, JA, Benson, DW, Dubin, AM, et al. Hypoplastic left heart syndrome: Current considerations and expectations. J Am Coll Cardiol 2012; 59: S1S42.
69. Jeffries, HE, Wells, WJ, Starnes, VA, Wetzel, RC, Moromisato, DY. Gastrointestinal morbidity after norwood palliation for hypoplastic left heart syndrome. Ann Thorac Surg 2006; 81: 982987.
70. Oster, ME, Chen, S, Dagincourt, N, et al. Development and impact of arrhythmias after the norwood procedure: A report from the pediatric heart network. J Thorac Cardiovasc Surg 2017; 153 (638-645): e632.
71. Kogon, B, Jain, A, Oster, M, Woodall, K, Kanter, K, Kirshbom, P. Risk factors associated with readmission after pediatric cardiothoracic surgery. Ann Thorac Surg 2012; 94: 865873.
72. Ugonabo, N, Hirsch-Romano, JC, Uzark, K. The role of home monitoring in interstage management of infants following the norwood procedure. World J Pediatr Congenit Heart Surg 2015; 6: 266273.
73. Pridjian, AK, Mendelsohn, AM, Lupinetti, FM, et al. Usefulness of the bidirectional Glenn procedure as staged reconstruction for the functional single ventricle. Am J Cardiol 1993; 71: 959962.
74. Kirklin, JK, Blackstone, EH, Kirklin, JW, Pacifico, AD, Bargeron, LM Jr. The fontan operation. Ventricular hypertrophy, age, and date of operation as risk factors. J Thorac Cardiovasc Surg 1986; 92: 10491064.
75. Seliem, MA, Baffa, JM, Vetter, JM, Chen, SL, Chin, AJ, Norwood, WI Jr. Changes in right ventricular geometry and heart rate early after hemi-fontan procedure. Ann Thorac Surg 1993; 55: 15081512.
76. Fogel, MA, Weinberg, PM, Chin, AJ, Fellows, KE, Hoffman, EA. Late ventricular geometry and performance changes of functional single ventricle throughout staged fontan reconstruction assessed by magnetic resonance imaging. J Am Coll Cardiol 1996; 28: 212221.
77. Scheurer, MA, Hill, EG, Vasuki, N, et al. Survival after bidirectional cavopulmonary anastomosis: Analysis of preoperative risk factors. J Thorac Cardiovasc Surg 2007; 134: 8289; 89 e81–82.
78. Galantowicz, M, Cheatham, JP, Phillips, A, et al. Hybrid approach for hypoplastic left heart syndrome: Intermediate results after the learning curve. Ann Thorac Surg 2008; 85: 20632070; discussion 2070–2061.
79. Brown, DW, Gauvreau, K, Moran, AM, et al. Clinical outcomes and utility of cardiac catheterization prior to superior cavopulmonary anastomosis. J Thorac Cardiovasc Surg 2003; 126: 272281.
80. Dave, H, Rosser, B, Knirsch, W, Hubler, M, Pretre, R, Kretschmar, O. Hybrid approach for hypoplastic left heart syndrome and its variants: the fate of the pulmonary arteries. Eur J Cardiothorac Surg 2014; 46: 1419.
81. Davies, RR, Radtke, WA, Klenk, D, Pizarro, C. Bilateral pulmonary arterial banding results in an increased need for subsequent pulmonary artery interventions. J Thorac Cardiovasc Surg 2014; 147: 706712.
82. Eagam, M, Loomba, RS, Pelech, AN, Tweddell, JS, Kirkpatrick, E. Predicting the need for neoaortic arch intervention in infants with hypoplastic left heart syndrome through the Glenn procedure. Pediatr Cardiol 2017; 38: 7076.
83. Brown, DW, Gauvreau, K, Powell, AJ, et al. Cardiac magnetic resonance versus routine cardiac catheterization before bidirectional Glenn anastomosis: Long-term follow-up of a prospective randomized trial. J Thorac Cardiovasc Surg 2013; 146: 11721178.
84. Han, BK, Vezmar, M, Lesser, JR, et al. Selective use of cardiac computed tomography angiography: An alternative diagnostic modality before second-stage single ventricle palliation. J Thorac Cardiovasc Surg 2014; 148: 15481554.
85. Menon, SC, McCandless, RT, Mack, GK, et al. Clinical outcomes and resource use for infants with hypoplastic left heart syndrome during bidirectional Glenn: Summary from the joint council for congenital heart disease national pediatric cardiology quality improvement collaborative registry. Pediatr Cardiol 2013; 34: 143148.
86. Baker-Smith, CM, Goldberg, SW, Rosenthal, GL. Predictors of prolonged hospital length of stay following stage ii palliation of hypoplastic left heart syndrome (and variants): Analysis of the national pediatric cardiology quality improvement collaborative (npc-qic) database. Pediatr Cardiol 2015; 36: 16301641.
87. Lee, TM, Aiyagari, R, Hirsch, JC, Ohye, RG, Bove, EL, Devaney, EJ. Risk factor analysis for second-stage palliation of single ventricle anatomy. Ann Thorac Surg 2012; 93: 614618. discussion 619.
88. Redington, AN, Penny, D, Shinebourne, EA. Pulmonary blood flow after total cavopulmonary shunt. Br Heart J 1991; 65: 213217.
89. Lofland, GK. The enhancement of hemodynamic performance in fontan circulation using pain free spontaneous ventilation. Eur J Cardiothorac Surg 2001; 20: 114118; discussion 118–119.
90. Huang, J, Zhou, Y, Zhu, D. Systemic haemodynamics and regional tissue oxygen saturation after bidirectional cavopulmonary shunt: Positive pressure ventilation versus spontaneous breathing. Interact Cardiovasc Thorac Surg 2016; 23: 235239.
91. Bronicki, RA, Anas, NG. Cardiopulmonary interaction. Pediatr Crit Care Med 2009; 10: 313322.
92. Bronicki, RA, Penny, DJ, Anas, NG, Fuhrman, B. Cardiopulmonary interactions. Pediatr Crit Care Med 2016; 17: S182S193.
93. Li, J, Hoskote, A, Hickey, C, Stephens, D, et al. Effect of carbon dioxide on systemic oxygenation, oxygen consumption, and blood lactate levels after bidirectional superior cavopulmonary anastomosis. Crit Care Med 2005; 33: 984989.
94. Jolley, M, Thiagarajan, RR, Barrett, CS, et al. Extracorporeal membrane oxygenation in patients undergoing superior cavopulmonary anastomosis. J Thorac Cardiovasc Surg 2014; 148: 15121518.
95. Booth, KL, Roth, SJ, Thiagarajan, RR, Almodovar, MC, del Nido, PJ, Laussen, PC. Extracorporeal membrane oxygenation support of the fontan and bidirectional Glenn circulations. Ann Thorac Surg 2004; 77: 13411348.
96. Gomez, D, Duffy, V, Hersey, D, et al. Extracorporeal membrane oxygenation outcomes after the comprehensive stage ii procedure in patients with single ventricles. Artif Organs 2017; 41: 6670.
97. Niebler, RA, Shah, TK, Mitchell, ME, et al. Ventricular assist device in single-ventricle heart disease and a superior cavopulmonary anastomosis. Artif Organs 2016; 40: 180184.
98. Fontan, F, Baudet, E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240248.
99. Lemler, MS, Scott, WA, Leonard, SR, Stromberg, D, Ramaciotti, C. Fenestration improves clinical outcome of the fontan procedure: A prospective, randomized study. Circulation 2002; 105: 207212.
100. Sinha, P, Zurakowski, D, He, D, et al. Intra/extracardiac fenestrated modification leads to lower incidence of arrhythmias after the fontan operation. J Thorac Cardiovasc Surg 2013; 145: 678682.
101. Forsdick, V, Iyengar, AJ, Carins, T, et al. Unsatisfactory early and late outcomes after fontan surgery delayed to adolescence and adulthood. Semin Thorac Cardiovasc Surg 2015; 27: 168174.
102. Garofalo, CA, Cabreriza, SE, Quinn, TA, et al. Ventricular diastolic stiffness predicts perioperative morbidity and duration of pleural effusions after the fontan operation. Circulation 2006; 114: I56I61.
103. Fontan, F, Fernandez, G, Costa, F, et al. The size of the pulmonary arteries and the results of the fontan operation. J Thorac Cardiovasc Surg 1989; 98: 711719; discussion 719–724.
104. Knott-Craig, CJ, Julsrud, PR, Schaff, HV, Puga, FJ, Danielson, GK. Pulmonary artery size and clinical outcome after the modified fontan operation. Ann Thorac Surg 1993; 55: 646651.
105. Fogel, MA, Pawlowski, TW, Whitehead, KK, et al. Cardiac magnetic resonance and the need for routine cardiac catheterization in single ventricle patients prior to fontan: a comparison of 3 groups: Pre-fontan cmr versus cath evaluation. J Am Coll Cardiol 2012; 60: 10941102.
106. Azakie, A, Merklinger, SL, Williams, WG, Van Arsdell, GS, Coles, JG, Adatia, I. Improving outcomes of the fontan operation in children with atrial isomerism and heterotaxy syndromes. Ann Thorac Surg 2001; 72: 16361640.
107. Bartz, PJ, Driscoll, DJ, Dearani, JA, et al. Early and late results of the modified fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol 2006; 48: 23012305.
108. Gentles, TL, Mayer, JE Jr., Gauvreau, K, et al. Fontan operation in five hundred consecutive patients: Factors influencing early and late outcome. J Thorac Cardiovasc Surg 1997; 114: 376391.
109. Ballweg, JA, Dominguez, TE, Ravishankar, C, et al. A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at fontan completion. J Thorac Cardiovasc Surg 2010; 140: 537544.
110. Hakacova, N, Lakomy, M, Kovacikova, L. Arrhythmias after fontan operation: comparison of lateral tunnel and extracardiac conduit. J Electrocardiol 2008; 41: 173177.
111. Shekerdemian, LS, Bush, A, Shore, DF, Lincoln, C, Redington, AN. Cardiopulmonary interactions after fontan operations: Augmentation of cardiac output using negative pressure ventilation. Circulation 1997; 96: 39343942.
112. Penny, DJ, Redington, AN. Doppler echocardiographic evaluation of pulmonary blood flow after the fontan operation: The role of the lungs. Br Heart J 1991; 66: 372374.
113. Mutsuga, M, Quinonez, LG, Mackie, AS, et al. Fast-track extubation after modified fontan procedure. J Thorac Cardiovasc Surg 2012; 144: 547552.
114. Salvin, JW, Scheurer, MA, Laussen, PC, et al. Factors associated with prolonged recovery after the fontan operation. Circulation 2008; 118: S171S176.
115. Imielski, BR, Niebler, RA, Kindel, SJ, Woods, RK. Heartware ventricular assist device implantation in patients with fontan physiology. Artif Organs 2017; 41: 4046.
116. Throckmorton, AL, Lopez-Isaza, S, Downs, EA, Chopski, SG, Gangemi, JJ, Moskowitz, W. A viable therapeutic option: Mechanical circulatory support of the failing fontan physiology. Pediatr Cardiol 2013; 34: 13571365.
117. Woods, RK, Ghanayem, NS, Mitchell, ME, Kindel, S, Niebler, RA. Mechanical circulatory support of the fontan patient. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2017; 20: 2027.
118. Arnaoutakis, GJ, Blitzer, D, Fuller, S, et al. Mechanical circulatory support as bridge to transplantation for the failing single ventricle. Ann Thorac Surg 2017; 103: 193197.
119. Giridharan, GA, Ising, M, Sobieski, MA, et al. Cavopulmonary assist for the failing fontan circulation: Impact of ventricular function on mechanical support strategy. ASAIO J 2014; 60: 707715.
120. Rychik, J. Forty years of the fontan operation: a failed strategy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2010; 13: 96100.
121. Alsoufi, B, Mahle, WT, Manlhiot, C, et al. Outcomes of heart transplantation in children with hypoplastic left heart syndrome previously palliated with the norwood procedure. J Thorac Cardiovasc Surg 2016; 151: 167174; 175 e161–162.
122. Kulkarni, A, Neugebauer, R, Lo, Y, et al. Outcomes and risk factors for listing for heart transplantation after the norwood procedure: An analysis of the single ventricle reconstruction trial. J Heart Lung Transplant 2016; 35: 306311.
123. Almond, CS, Gauvreau, K, Canter, CE, Rajagopal, SK, Piercey, GE, Singh, TP. A risk-prediction model for in-hospital mortality after heart transplantation in us children. Am J Transplant 2012; 12: 12401248.
124. Weinstein, S, Bello, R, Pizarro, C, et al. The use of the berlin heart excor in patients with functional single ventricle. J Thorac Cardiovasc Surg 2014; 147: 697704; discussion 704-695.
125. Rossano, JW, Woods, RK, Berger, S, et al. Mechanical support as failure intervention in patients with cavopulmonary shunts (mfics): Rationale and aims of a new registry of mechanical circulatory support in single ventricle patients. Congenit Heart Dis 2013; 8: 182186.
126. O’Connor, MJ, Pahl, E, Webber, SA, Rossano, JW. Recent advances in heart transplant immunology: The role of antibodies. Prog Pediatr Cardiol 2016; 43: 8185.
127. Shaddy, RE, Hunter, DD, Osborn, KA, et al. Prospective analysis of hla immunogenicity of cryopreserved valved allografts used in pediatric heart surgery. Circulation 1996; 94: 10631067.
128. Tambur, AR, Pamboukian, SV, Costanzo, MR, et al. The presence of hla-directed antibodies after heart transplantation is associated with poor allograft outcome. Transplantation 2005; 80: 10191025.
129. Rossano, JW, Morales, DL, Zafar, F, et al. Impact of antibodies against human leukocyte antigens on long-term outcome in pediatric heart transplant patients: An analysis of the united network for organ sharing database. The Journal of thoracic and cardiovascular surgery 2010; 140: 694699; 699 e691–692.
130. Tran, A, Fixler, D, Huang, R, Meza, T, Lacelle, C, Das, BB. Donor-specific hla alloantibodies: Impact on cardiac allograft vasculopathy, rejection, and survival after pediatric heart transplantation. J Heart Lung Transplant 2016; 35: 8791.
131. Colvin, MM, Cook, JL, Chang, P, et al. Antibody-mediated rejection in cardiac transplantation: Emerging knowledge in diagnosis and management: A scientific statement from the american heart association. Circulation 2015; 131: 16081639.
132. Everitt, MD, Boyle, GJ, Schechtman, KB, et al. Early survival after heart transplant in young infants is lowest after failed single-ventricle palliation: a multi-institutional study. J Heart Lung Transplant 2012; 31: 509516.
133. Kovach, JR, Naftel, DC, Pearce, FB, et al. Comparison of risk factors and outcomes for pediatric patients listed for heart transplantation after bidirectional Glenn and after Fontan: an analysis from the pediatric heart transplant study. J Heart Lung Transplant 2012; 31: 133139.
134. Griffiths, ER, Kaza, AK, Wyler von Ballmoos, MC, et al. Evaluating failing fontans for heart transplantation: Predictors of death. Ann Thorac Surg 2009; 88: 558563; discussion 563–554.
135. Simpson, KE, Pruitt, E, Kirklin, JK, et al. Fontan patient survival after pediatric heart transplantation has improved in the current era. Ann Thorac Surg 2017; 103: 13151320.
136. Miller, JR, Simpson, KE, Epstein, DJ, et al. Improved survival after heart transplant for failed fontan patients with preserved ventricular function. J Heart Lung Transplant 2016; 35: 877883.
137. Mehra, MR, Canter, CE, Hannan, MM, et al. The 2016 international society for heart lung transplantation listing criteria for heart transplantation: a 10-year update. J Heart Lung Transplant 2016; 35: 123.
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Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
  • URL: /core/journals/cardiology-in-the-young
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