Staged single-ventricle palliation in 2011: Outcomes and expectations

Congenital Heart Disease

Volumn 6, Issue 5, 2011, Pages 406-416

  Petit, Christopher J ^a  

^a BAYLOR COLLEGE OF MEDICINE   (United States)

Author keywords

Fontan; Hypoplastic left heart; Norwood; Single ventricle

Indexed keywords

AORTOPULMONARY SHUNT; CAVOPULMONARY CONNECTION; CORONARY ARTERY BLOOD FLOW; FONTAN PROCEDURE; GLENN SHUNT; HEART HEMODYNAMICS; HEART SINGLE VENTRICLE; HUMAN; NORWOOD PROCEDURE; PALLIATIVE THERAPY; PRIORITY JOURNAL; QUALITY OF LIFE; REVIEW; SURVIVAL; TREATMENT OUTCOME; VEIN BLOOD FLOW;

CARDIAC SURGICAL PROCEDURES; HEART DEFECTS, CONGENITAL; HEART VENTRICLES; HEMODYNAMICS; HUMANS; PALLIATIVE CARE; RISK ASSESSMENT; RISK FACTORS; TREATMENT OUTCOME;

EID: 80052831043     PISSN: 1747079X     EISSN: 17470803     Source Type: Journal    
DOI: 10.1111/j.1747-0803.2011.00560.x     Document Type: Review

References (74)

1. Norwood WI, Lang P, Casteneda AR, Campbell DN. Experience with operations for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 1981;82:511-519.
2. Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med. 1983;308:23-26.
3. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:240-248.
4. Kreutzer G, Galindez E, Bono H, De Palma C, Laura JP. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg. 1973;66:613-621.
5. Starr I, Jeffers W, Jr. RM. The absence of conspicuous increments of venous pressure after severe damage to the right ventricle of the dog, with a discussion of the relation between clinical congestive failure and heart disease. Am Heart J. 1943;26:291.
6. Rose J, Cosimano S, Hufnagel C, Mussallo E. The effects of exclusion of the right ventricle from the circulation in dogs. J Clin Invest. 1955;34:1625-1631.
7. Puga F, McGoon D. Exclusion of the right ventricle from the circulation: hemodynamic observations. Surgery. 1973;73:607-613.
8. Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley FL, Silverman NH. Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome. Circulation. 2001;103:1269-1273.
9. Atz AM, Travison TG, Williams IA, etal. 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;140:1245-1250.
10. Mahle WT, Clancy RR, McGaurn SP, Goin JE, Clark BJ. Impact of prenatal diagnosis on survival and early neurologic morbidity in neonates with the hypoplastic left heart syndrome. Pediatrics. 2001;107:1277-1282.
11. Andrews R, Tulloh R, Sharland G, etal. Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis. Arch Dis Child. 2001;85:474-477.
12. Simpson JM. Fetal cardiac interventions: worth it? Heart. 2009;95:1653-1655.
13. Gardiner HM. The case for fetal cardiac intervention. Heart. 2009;95:1648-1652.
14. McElhinney DB, Marshall AC, Wilkins-Haug LE, etal. Predictors of technical success and postnatal biventricular outcome after in utero aortic valvuloplasty for aortic stenosis with evolving hypoplastic left heart syndrome. Circulation. 2009;120:1482-1490.
15. Glatz JA, Tabbutt S, Gaynor JW, etal. Hypoplastic left heart syndrome with atrial level restriction in the era of prenatal diagnosis. Ann Thorac Surg. 2007;84:1633-1638.
16. Rychik J, Rome JJ, Collins MH, DeCampli WM, Spray TL. The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol. 1999;34:554-560.
17. Atz AM, Feinstein JA, Jonas RA, Perry SB, Wessel DL. Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect. Am J Cardiol. 1999;83:1224-1228.
18. Vida VL, Bacha EA, Larrazabal A, etal. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center. Ann Thorac Surg. 2007;84:581-585. discussion 86.
19. Sano S, Ishino K, Kawada M, etal. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126:504-509. discussion 09-10.
20. Pruetz JD, Badran S, Dorey F, Starnes VA, Lewis AB. Differential branch pulmonary artery growth after the Norwood procedure with right ventricle-pulmonary artery conduit versus modified Blalock-Taussig shunt in hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2009;137:1342-1348.
21. Caspi J, Pettitt TW, Mulder T, Stopa A. Development of the pulmonary arteries after the Norwood procedure: comparison between Blalock-Taussig shunt and right ventricular-pulmonary artery conduit. Ann Thorac Surg. 2008;86:1299-1304.
22. Rumball EM, McGuirk SP, Stumper O, etal. The RV-PA conduit stimulates better growth of the pulmonary arteries in hypoplastic left heart syndrome. Eur J Cardiothorac Surg. 2005;27:801-806.
23. Ballweg JA, Dominguez TE, Ravishankar C, etal. A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at stage 2 reconstruction. J Thorac Cardiovasc Surg. 2007;134:297-303.
24. Lai L, Laussen PC, Cua CL, etal. Outcomes after bidirectional Glenn operation: Blalock-Taussig shunt versus right ventricle-to-pulmonary artery conduit. Ann Thorac Surg. 2007;83:1768-1773.
25. Ohye RG, Sleeper LA, Mahony L, etal. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med. 2010;362:1980-1992.
26. Gibbs JL, Wren C, Watterson KG, Hunter S, Hamilton JR. Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome. Br Heart J. 1993;69:551-555.
27. Akintuerk H, Michel-Behnke I, Valeske K, etal. Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage I and II repair in hypoplastic left heart. Circulation. 2002;105:1099-1103.
28. Galantowicz M, Cheatham JP. Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol. 2005;26:190-199.
29. Bacha EA, Daves S, Hardin J, etal. Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy. J Thorac Cardiovasc Surg. 2006;131:163-171. e2.
30. Ghanayem NS, Hoffman GM, Mussatto KA, etal. Home surveillance program prevents interstage mortality after the Norwood procedure. J Thorac Cardiovasc Surg. 2003;126:1367-1377.
31. Hehir DA, Dominguez TE, Ballweg JA, etal. Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants. J Thorac Cardiovasc Surg. 2008;136:94-99. 99 e1-3.
32. Mahle WT, Spray TL, Gaynor JW, Clark BJ 3rd. Unexpected death after reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg. 2001;71:61-65.
33. Petit C, Fraser C, Mattamal R, Slesnick T, Cephus C, Ocampo E. The impact of a dedicated single ventricle home monitoring program on interstage somatic growth, interstage attrition, and one-year survival. J Thorac Cardiovasc Surg. 2011 June 22 [Epub ahead of print].
34. Bartram U, Grunenfelder J, Van Praagh R. Causes of death after the modified Norwood procedure: a study of 122 postmortem cases. Ann Thorac Surg. 1997;64:1795-1802.
35. Anderson JB, Beekman RH 3rd, Border WL, etal. Lower weight-for-age z score adversely affects hospital length of stay after the bidirectional Glenn procedure in 100 infants with a single ventricle. J Thorac Cardiovasc Surg. 2009;138:397-404.
36. Srinivasan C, Jaquiss RD, Morrow WR, etal. Impact of staged palliation on somatic growth in patients with hypoplastic left heart syndrome. Congenit Heart Dis. 2010;5:546-551.
37. Johnson JN, Ansong AK, Li JS, etal. Celiac artery flow pattern in infants with single right ventricle following the Norwood procedure with a modified Blalock-Taussig or right ventricle to pulmonary artery shunt. Pediatr Cardiol. 2011;32:479-486.
38. Jaquiss RD, Ghanayem NS, Hoffman GM, etal. Early cavopulmonary anastomosis in very young infants after the Norwood procedure: impact on oxygenation, resource utilization, and mortality. J Thorac Cardiovasc Surg. 2004;127:982-989.
39. Norwood WI, Jacobs ML. Fontan's procedure in two stages. Am J Surg. 1993;166:548-551.
40. Young WG Jr, Sealy WC, Houck WS Jr, Whalen RE, Spach MS, Canent RV Jr. Superior vena cava-right pulmonary artery anastomosis in cyanotic heart disease. Ann Surg. 1963;157:894-901.
41. Chang AC, Hanley FL, Wernovsky G, etal. Early bidirectional cavopulmonary shunt in young infants. Postoperative course and early results. Circulation. 1993;88:II149-II158.
42. Bradley SM, Mosca RS, Hennein HA, Crowley DC, Kulik TJ, Bove EL. Bidirectional superior cavopulmonary connection in young infants. Circulation. 1996;94:II5-I11.
43. Maher KO, Pizarro C, Gidding SS, etal. Hemodynamic profile after the Norwood procedure with right ventricle to pulmonary artery conduit. Circulation. 2003;108:782-784.
44. Wallace MC, Jaggers J, Li JS, etal. Center variation in patient age and weight at fontan operation and impact on postoperative outcomes. Ann Thorac Surg. 2011;91:1445-1452.
45. Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation. 1990;82:1681-1689.
46. Bridges ND, Mayer JE, Jr, Lock JE, etal. Effect of baffle fenestration on outcome of the modified Fontan operation. Circulation. 1992;86:1762-1769.
47. Laks H. The partial Fontan procedure. A new concept and its clinical application. Circulation. 1990;82:1866-1867.
48. McElhinney DB, Petrossian E, Reddy VM, Hanley FL. Extracardiac conduit Fontan procedure without cardiopulmonary bypass. Ann Thorac Surg. 1998;66:1826-1828.
49. Salazar JD, Zafar F, Siddiqui K Jr, etal. Fenestration during Fontan palliation: now the exception instead of the rule. J Thorac Cardiovasc Surg. 2010;140:129-136.
50. Dasi LP, Krishnankuttyrema R, Kitajima HD, etal. Fontan hemodynamics: importance of pulmonary artery diameter. J Thorac Cardiovasc Surg. 2009;137:560-564.
51. Harris MA, Cosulich MT, Gillespie MJ, etal. Pre-Fontan cardiac magnetic resonance predicts post-Fontan length of stay and avoids ionizing radiation. J Thorac Cardiovasc Surg. 2009;138:941-947.
52. Rychik J. Forty years of the Fontan operation: a failed strategy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2010;13:96-100.
53. Diller GP, Giardini A, Dimopoulos K, etal. Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients. Eur Heart J. 2010;31:3073-3083.
54. McCrindle BW, Williams RV, Mital S, etal. Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health. Arch Dis Child. 2007;92:509-514.
55. Anderson PA, Sleeper LA, Mahony L 3rd, etal. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol. 2008;52:85-98.
56. Khairy P, Fernandes SM, Mayer JE, etal. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117:85-92.
57. Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998;115:1063-1073.
58. Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis. 2007;2:288-300.
59. Brancaccio G, Carotti A, D'Argenio P, Michielon G, Parisi F. Protein-losing enteropathy after Fontan surgery: resolution after cardiac transplantation. J Heart Lung Transplant. 2003;22:484-486.
60. Shah H, Kuehl K, Sherker AH. Liver disease after the Fontan procedure: what the hepatologist needs to know. J Clin Gastroenterol. 2010;44:428-431.
61. Baek JS, Bae EJ, Ko JS, etal. Late hepatic complications after Fontan operation; non-invasive markers of hepatic fibrosis and risk factors. Heart. 2010;96:1750-1755.
62. Saliba T, Dorkhom S, O'Reilly EM, Ludwig E, Gansukh B, Abou-Alfa GK. Hepatocellular carcinoma in two patients with cardiac cirrhosis. Eur J Gastroenterol Hepatol. 2010;22:889-891.
63. Tomita H, Yamada O, Ohuchi H, etal. Coagulation profile, hepatic function, and hemodynamics following Fontan-type operations. Cardiol Young. 2001;11:62-66.
64. 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;86:177-182.
65. Yeh T Jr, Williams WG, McCrindle BW, etal. Equivalent survival following cavopulmonary shunt: with or without the Fontan procedure. Eur J Cardiothorac Surg. 1999;16:111-116.
66. Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, etal. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol. 2004;44:2065-2072.
67. Kanter KR, Mahle WT, Vincent RN, Berg AM, Kogon BE, Kirshbom PM. Heart transplantation in children with a Fontan procedure. Ann Thorac Surg. 2011;91:823-829. discussion 29-30.
68. Gamba A, Merlo M, Fiocchi R, etal. Heart transplantation in patients with previous Fontan operations. J Thorac Cardiovasc Surg. 2004;127:555-562.
69. Nurnberg JH, Ovroutski S, Alexi-Meskishvili V, Ewert P, Hetzer R, Lange PE. 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. discussion 88.
70. Stephenson EA, Lu M, Berul CI, etal. Arrhythmias in a contemporary fontan cohort: prevalence and clinical associations in a multicenter cross-sectional study. J Am Coll Cardiol. 2010;56:890-896.
71. Cohen MI, Rhodes LA. Sinus node dysfunction and atrial tachycardia after the Fontan procedure: the scope of the problem. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 1998;1:41-52.
72. Cohen MI, Bridges ND, Gaynor JW, etal. Modifications to the cavopulmonary anastomosis do not eliminate early sinus node dysfunction. J Thorac Cardiovasc Surg. 2000;120:891-900.
73. Cohen MI, Wernovsky G, Vetter VL, etal. Sinus node function after a systematically staged Fontan procedure. Circulation. 1998;98:II352-II358. discussion II58-9.
74. Dilawar M, Bradley SM, Saul JP, Stroud MR, Balaji S. Sinus node dysfunction after intraatrial lateral tunnel and extracardiac conduit fontan procedures. Pediatr Cardiol. 2003;24:284-288.