Medical management of the failing Fontan

Pediatric Cardiology

Volumn 28, Issue 6, 2007, Pages 465-471

  Ghanayem, N S ^a   Berger, S ^a   Tweddell, J S ^a  

^a CHILDREN S HOSPITAL OF WISCONSIN   (United States)

Author keywords

Fontan; Fontan failure; Late outcomes; Palliation; Protein losing enteropathy; Single ventricle

Indexed keywords

ALBUMIN; ALDOSTERONE ANTAGONIST; AMRINONE; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; BOSENTAN; CALCIUM; CARVEDILOL; CHLOROTHIAZIDE; CORTICOSTEROID; DIGOXIN; DIPEPTIDYL CARBOXYPEPTIDASE INHIBITOR; DIURETIC AGENT; HEPARIN; INOTROPIC AGENT; LOOP DIURETIC AGENT; MILRINONE; NESIRITIDE; PHOSPHODIESTERASE INHIBITOR; PREDNISONE; PROSTACYCLIN; SILDENAFIL; SPIRONOLACTONE; VITAMIN D;

CARDIOVASCULAR RISK; CLINICAL FEATURE; FONTAN PROCEDURE; FORWARD HEART FAILURE; HEART FAILURE; HEART SINGLE VENTRICLE; HEART VENTRICLE FAILURE; HEART VENTRICLE FUNCTION; HIGH RISK PATIENT; HUMAN; HYPOCALCEMIA; HYPOXEMIA; INCIDENCE; LUNG VASCULAR RESISTANCE; MORBIDITY; NONHUMAN; PROTEIN LOSING GASTROENTEROPATHY; REVIEW; RISK ASSESSMENT; RISK FACTOR; TREATMENT FAILURE;

ANOXIA; FONTAN PROCEDURE; HEART DEFECTS, CONGENITAL; HUMANS; INCIDENCE; LUNG DISEASES; PROTEIN-LOSING ENTEROPATHIES; RISK FACTORS; TREATMENT FAILURE; VASCULAR RESISTANCE; VENTRICULAR DYSFUNCTION;

EID: 36349026438     PISSN: 01720643     EISSN: None     Source Type: Journal    
DOI: 10.1007/s00246-007-9007-0     Document Type: Review

References (46)

1. Bendayan I, Casaldaliga J, Castello F, et al. (2000) Heparin therapy and reversal of protein-losing enteropathy in a case with congenital heart disease. Pediatr Cardiol 21:267-268
2. Border WL, Syed AU, Michelfelder EC, et al. (2003) Impaired systemic ventricular relaxation affects postoperative short-term outcome in Fontan patients. J Thorac Cardiovasc Surg 126:1760-1764
3. Bruns LA, Chrisant MK, Lamour JM, et al. (2001) Carvedilol as therapy in pediatric heart failure: an initial multicenter experience. J Pediatr 138:505-511
4. Burkhart HM, Dearani JA, Mair DD, et al. (2003) The modified Fontan procedure: early and late results in 132 adults. J Thorac Cardiovasc Surg 125:1252-1259
5. Cesario D, Clark J, Maisel A (1998) Beneficial effects of intermittent home administration of the inotrope/vasodilator milrinone in patients with end-stage congestive heart failure: a preliminary study. Am Heart J 135:121-129
6. Chakrabarti S, Keeton BR, Salmon AP, et al. (2003) Acquired combined immunodeficiency associated with protein losing enteropathy complicating Fontan operation. Heart 89:1130-1131
7. Cheung YF, Penny DJ, Redington AN (2000) Serial assessment of left ventricular diastolic function after Fontan procedure. Heart 83:420-424
8. Donnelly JP, Rosenthal A, Castle VP, et al. (1997) Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation. J Pediatr 130:474-478
9. Driscoll DJ, Offord KP, Feldt RH, et al. (1992) Five- to fifteen-year follow-up after Fontan operations. Circulation 85:469-496
10. Feldt RH, Driscoll DJ, Offord KP, et al. (1996) Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 112:672-680
11. Fonarow G (2003) B-type natriuretic peptide: spectrum of application. Nesiritide (recombinant BNP) for heart failure. Heart Failure Rev 8:321-325
12. Gentles TL, Gauvreau K, Mayer JE, et al. (1997) Functional outcome after the Fontan operation: factors influencing late morbidity. J Thorac Cardiovasc Surg 114:392-403
13. Gewillig M (1994) The Fontan circulation: late functional results. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 6:56-63
14. Hennein HA (2002) The Fontan operation for hypoplastic left heart syndrome. In: Hennein HA, Bove EL (eds) Hypoplastic Left Heart Syndrome 2002. Futura, New York, pp 155-178
15. Heragu N, Mahony L (1999) Is captopril useful in decreasing pleural drainage in children after modified Fontan operation. Am J Cardiol 84:1109-1112
16. Hjortdal VE, Stenberg EV, Ravn HB, et al. (2000) Neurohumoral activation late after cavopulmonary connection. Heart 83:439-443
17. Hoffman TM, Wernovsky G, Atz AM, et al. (2003) Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 107:996-1002
18. Jacobs ML, Rychik J, Byrum CJ, et al. (1996) Protein-losing enteropathy after Fontan operation: resolution after baffle fenestration. Ann Thorac Surg 61:206-208
19. Khambadkone S, Li J, de Leval MR, et al. (2003) Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation 107:3204-3208
20. Kim S, In-Seung P, Song J, et al. (2004) Reversal of protein-losing enteropathy with calcium replacement in a patient after Fontan operation. Ann Thorac Surg 77:1456-1457
21. Knott-Craig CJ, Danielson GK, Schaff HV, et al. (1995) The modified Fontan operation: an analysis of risk factors for early postoperative death or takedown in 702 consecutive patients from one institution. J Thorac Cardiovasc Surg 109:1237-1243
22. Kukin ML (2002) β-Blockers in chronic heart failure: considerations for selecting an agent. Mayo Clinic Proc 77:1199-1206
23. Lenz D, Hambsch J, Schneider P, et al. (2003) Protein-losing enteropathy in patients with Fontan circulation: is it triggered by infection? Crit Care 7:185-190
24. Levy M, Danel C, Laval A, et al. (2003) Nitric oxide synthase expression by pulmonary arteries: a predictive marker of Fontan procedure outcome? J Thorac Cardiovasc Surg 125:1083-1090
25. Lopez-Candales A, Vora T, Gibbons W, et al. (2002) Symptomatic improvement in patients treated with intermittent infusion of inotropes: a double-blind placebo controlled pilot study. J Med 33:129-146
26. Mahle WT, Coon PD, Wernovsky G, et al. (2001) Quantitative echocardiographic assessment of the performance of the functionally single right ventricle after the Fontan operation. Cardiol Young 11:399-406
27. Mainwaring RD, Lamberti JJ, Carter TL, et al. (1995) Renin, angiotensin II, and the development of effusions following bidirectional Glenn and Fontan procedures. J Card Surg 10:111-118
28. Mair DD, Hagler DJ, Puga FJ, et al. (1992) Fontan operation in 176 patients with tricuspid atresia. Results and a proposed new index for patient selection. Circulation 82(Suppl IV):IV-164-IV-169
29. Mair DD, Puga FJ, Danielson GK (2001) The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients. J Am Coll Cardiol 37:933-939
30. Marino BS (2002) Outcomes after the Fontan procedure. Curr Opin Pediatr 14:620-626
31. Marshall J, Berkenbosch JW, Russo P (2004) Preliminary experience with nesiritide in the pediatric population. J Intens Care Med 19:164-170
32. Merten L, Hagler DJ, Sauer U, et al. (1998) Protein losing enteropathy after the Fontan operation: an international multicenter study. J Thorac Cardiovasc Surg 115:1063-1073
33. Murch SH, Winyard PJD, Koletzko S, et al. (1996) Congenital enterocyte heparan sulfate deficiency with massive albumin loss, secretory diarrhea, and malnutrition. Lancet 347:1299-1301
34. Ohuchi H, Hasegawa S, Yasuda K, et al. (2001) Severely impaired cardiac autonomic nervous activity after the Fontan operation. Circulation 104:1513-1518
35. Pfeffer MA, Braunwald E, Moye LA, et al. (1992) Effect of captopril on mortality and morbidity in patients with left ventricular dysfunction after myocardial infarction. Results of the Survival and Ventricular Enlargement trial. The SAVE Investigators. N Engl J Med 327:669-667
36. Powell AJ, Gauvreau K, Jenkins KJ, et al. (2001) Perioperative risk factors for development of protein-losing enteropathy following a Fontan procedure. Am J Cardiol 88:1206-1209
37. Ringel RE, Peddy SB (2003) Effect of high-dose spironolactone on protein-losing enteropathy in patients with Fontan palliation of complex congenital heart disease. Am J Cardiol 91:1031-1032
38. Rothman A, Mayer JE, Freed MD (1987) Treatment of chronic pleural effusions after the Fontan procedure with prednisone. Am J Cardiol 60:408-409
39. Rychik J, Spray TL (2002) Strategies to treat protein-losing enteropathy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 5:3-11
40. Sensaki H, Masutani S, Kobayashi J, et al. (2002) Ventricular afterload and ventricular work in Fontan circulation: comparison with normal two-ventricle circulation and single-ventricle circulation with Blalock-Taussig shunts. Circulation 105:2885-2892
41. Silverman A, Roy CC (1983) Protein-losing enteropathy. In: Silverman A, Roy CC, (eds) Pediatric Clinical Gastroenterology, 3rd edn. Mosby, St. Louis, pp 304-323
42. Sorenson GK, Ramamoorthy C, Lynn AM, et al. (1996) Hemodynamic effects of amrinone in children after Fontan surgery. Anesth Analg 82:241-246
43. Stewart JM, Gewitz MH, Clark BJ, et al. (1991) The role of vasopressin and atrial natriuretic factor in postoperative fluid retention after the Fontan procedure. J Thorac Cardiovasc Surg 102:821-829
44. Stier CT Jr, Koenig S, Lee DY, et al. (2003) Aldosterone and aldosterone antagonism in cardiovascular disease: focus on eplerenone (inspra). Heart Dis 5:102-118
45. Therrien J, Webb GD, Gatzoulis MA (1999) Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation? Heart 82:241-243
46. Zellers TM, Brown K (1996) Protein-losing enteropathy after the modified Fontan operation: oral prednisone treatment with biopsy and laboratory proved improvement. Pediatr Cardiol 17:115-117