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Somatic growth failure after the Fontan operation

Published online by Cambridge University Press:  19 August 2008

Mitchell I. Cohen
Divisions of Cardiology, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
David M. Bush
Divisions of Cardiology, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Robert J. Ferry Jr
Endocrinology, and The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Thomas L. Spray
Cardiothoracic Surgery and the Departments of Pediatrics and Surgery, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Thomas Moshang Jr
Endocrinology, and The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Gil Wernovsky
Divisions of Cardiology, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Victoria L. Vetter
Divisions of Cardiology, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
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Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was-1·5± 1·2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (−0·91 ±0·99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were −0·67 ±1·1 and −0·89±1·2 respectively. At most recent follow-up, with a mean age of 6·1 ± 1·3 years, and a mean time from the Fontan operation of 4·4±1·4 years, the mean Z score for height was −1·15 ±1·2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings

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1.Guyer, B, Martin, JA, MacDorman, MF, Anderson, RN, Strobino, DM. Annual summary of vital statistics. Pediatrics 1997;100(6): 905918.CrossRefGoogle ScholarPubMed
2.Fyler, DC.Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65: 376460.Google Scholar
3.Ferencz, C.Congenital heart disease: Prevalence at live birth; The Baltimore-Washington Infant Study. Am J Epidemiol 1984;121: 131.Google Scholar
4.Wechsler, SB, Wernovsky, G. Cardiac Disorders. In: Manual of Neonatal Care 4th Ed. Lippincott Raven Publishers, New York (NY): 1998. P 393451.Google Scholar
5.Fyler, DC. Single Ventricle. In:Nadas' Pediatric Cardiology. Hanley Belfus, Inc. 1992. p. 649657.Google Scholar
6.Gentles, TL, Mayer, JE Jr, Gauvreau, K, Newburger, JW, Lock, JE, Kupferschmid, JP, Burnett, JA, Jonas, RA, Castaneda, AC, Wernovsky, G. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg 1997;114: 376391.CrossRefGoogle ScholarPubMed
7.Koutlas, TC, Gaynor, JW, Nicolson, SC, Steven, JM, Wernovsky, G, Spray, TL. Modified ultrafiltration reduces postoperative morbidity after cavopulmonary connection. Ann Thorac Surg 1997;64: 3743.CrossRefGoogle ScholarPubMed
8.Bayer, LM, Robinson, SJ. Growth history of children with congenital heart defects. Am J Dis Child 1969;117: 564572.CrossRefGoogle ScholarPubMed
9.Ehlers, KH. Growth failure in association with congenital heart disease. Pediatr Ann 1978;7: 750759.Google ScholarPubMed
10.Levy, RJ, Rosenthal, A, Castenada, AR. Growth after surgical repair of simple D-transposition of the great arteries. Ann Thorac Surg 1978;25: 225230.CrossRefGoogle ScholarPubMed
11.Mehrizi, A, Drash, A. Growth disturbance in congenital heart disease. J Pediatr 1962;61: 418429.CrossRefGoogle ScholarPubMed
12.Krieger, I. Growth failure and congenital heart disease. Am J Dis Child 1970;120: 497502.CrossRefGoogle ScholarPubMed
13.Sholler, GF, Celermajer, JM. Cardiac surgery in the first year of life: the effect on weight gains of infants with congenital heart disease. Aust Paediatr J 1986;22: 305308.Google ScholarPubMed
14.Levy, RJ, Rosenthal, A, Miettinen, OS, Nadas, AS. Determinants of growth in patients with ventricular septal defect. Circulacion 1978;57: 793797.CrossRefGoogle ScholarPubMed
15.Feldt, RH, Strickler, GB, Weidman, WH. Growth of children with congenital heart disease. Am J Dis Child 1969; 117573.Google ScholarPubMed
16.Fontan, F, Baudet, E. Surgical repair of tricuspid atresia. Thorax 1971;26: 240248.CrossRefGoogle ScholarPubMed
17.Rhee, EK, Evangelista, JK, Nigrin, DJ, Erickson, LC. The impact of isolated secundum atrial septal defect closure on growth in undersized children. J Am Coll Cardiol 1999;33: 524A [abstract].Google Scholar
18.Weintraub, RG, Menahem, S. Early surgical closure of a large ventricular septal defect: influence on long-term growth. J Am Coll Cardiol 1991;18: 552558.CrossRefGoogle ScholarPubMed
19.Page, RE, Deverall, PB, Watson, DA, Scott, O. Height and weight after total correction of Fallot's tetralogy. Br Heart J 1989;40: 416420.CrossRefGoogle Scholar
20.Razzouk, AJ, Chinnock, RE, Gundry, SR, Bailey, LL. Cardiac transplantation for infants with hypoplastic left heart syndrome. Prog Ped Cardiol 1996;5: 3747.CrossRefGoogle Scholar
21.Baum, M, Chinnock, R, Ashwal, S, Peverini, R, Trimm, F, Bailey, L. Growth and neurodevelopmental outcome of infants undergoing heart transplantation. J Heart Lung Transplant 1993;12: S211–17.Google ScholarPubMed
22.Baum, D, Bernstein, D, Starnes, V, Oyer, P, Pittlick, P, Stinson, E, Shumway, N. Pediatric heart transplantation at Stanford: Results of a 15-year experience. Pediatrics 1991;88: 203214.Google ScholarPubMed
23.Norwood, WI, Jacobs, ML. Fontan's procedure in two stages. Am J Surg 1993;166: 548551.CrossRefGoogle ScholarPubMed
24.Seliem, MA, Baffa, JM, Vetter, JM, Chen, SL, Chin, AJ, Norwood, WI. Changes in right ventricular geometry and heart rate after hemi-Fontan procedure. Ann Thorac Surg 1993;55: 15081512.CrossRefGoogle ScholarPubMed
25.Rychik, J, Jacobs, ML, Norwood, WI. Acute changes in left ventricular geometry after volume reduction Ann Thorac Surg 1995;60: 12671274.CrossRefGoogle ScholarPubMed
26.Choussat, A, Fontan, F, Besse, P, Vallot, F, Chauve, A, Bricaud, H. Selection criteria for Fontan's procedure. In: Anderson, RH, Shinebourne, EA eds. Paediatric Cardiology. Edinburgh: Churchill Livingstone, 1978: p. 559566.Google Scholar
27.Cetta, F, Feldt, RH, O'Leary, PW, Mair, DD, Warner, CA, Driscoll, DJ, Hagler, DJ, Porter, C, Offord, KP, Schaff, HV, Puga, FJ, Danielson, GK. Improved early morbidity and mortality after Fontan operation: The Mayo Clinic experience, 1987 to 1992. J Am Coll Cardiol 1996;28: 480486.CrossRefGoogle Scholar
28.Jacobs, ML, Norwood, WI Jr. Fontan operation: influence of modifications on morbidity and mortality. Ann Thorac Surg 1994;58: 945952.CrossRefGoogle ScholarPubMed
29.Pearl, JM, Laks, H, Drinkwater, DC, Caouya, ER, George, BL, Williams, RG. Modified Fontan procedure in patients less than 4 years of age. Circulation 1992;86(suppl II): II-100-II-105Google ScholarPubMed
30.Rychik, J, Rome, JJ, Jacobs, ML. Late surgical fenestration for complications after the Fontan operation. Circulation 1997;96: 3336.CrossRefGoogle ScholarPubMed
31.Hamill, PV, Drizd, TA, Johnson, CL, Reed, RB, Roche, AF, Moore, WM. Physical growth: National Center for Health Statistics Percentiles. Am J Clin Nutr 1979;32: 607.CrossRefGoogle ScholarPubMed
32.Roche, AF, Wainer, H, Thissen, D. The RWT method for the prediction of adult stature. Pediatrics 1975;56: 10271033.Google ScholarPubMed
33.Driscoll, DJ, Offord, KP, Feldt, RH, Schaff, HV, Puga, FJ, Danielson, GK. Five- to fifteen-year follow-up after Fontan operation. Circulation 1992;85: 469496.CrossRefGoogle ScholarPubMed
34.Cohen, MI, Wernovsky, G, Vetter, VL, Wieand, TS, Gaynor, JW, Jacobs, ML, Spray, TL, Rhodes, LA. Sinus node function after a systematically staged Fontan procedure. Circulation 1998;98: II-352-II-359.Google ScholarPubMed
35.Gewillig, M, Wyse, RK, de Leval, MR, Deanfield, JE. Early and late arrhythmias after the Fontan operation: predisposing factors and clinical consequences. Br Heart J 1992;67: 72–79CrossRefGoogle ScholarPubMed
36.Fishberger, SB, Wernovsky, G, Gentles, TL, Gavreau, K, Burnett, J, Mayer, JE Jr, Walsh, EP. Factors that influence the development of atrial flutter after the Fontan operation. J Thorac Cardiovasc Surg 1997;113: 8086.CrossRefGoogle ScholarPubMed
37.Gentles, TL, Gauvreau, K, Mayer, JE Jr, Fishberger, SB, Burnett, J, Colan, SD, Newburger, JW, Wernovsky, G. Functional outcome after the Fontan operation: factors influencing late morbidity.J Thorac Cardiovasc Surg 1997;114: 392403CrossRefGoogle ScholarPubMed
38.Cromme-Dijkihuis, AH, Hess, J, Hahien, K, Henkens, CMA, Bink-Boelkens, MthE, Eygelaar, AA, Bos, E.Specific sequelae after the Fontan operation at mid- and long-term follow-up. J Thorac Cardiovsc Surg 1993;106: 11261132.Google Scholar
39.Hill, D, Feldt, R, Porter, C. Protein-losing enteropathy after Fontan operation: A preliminary report. Circulation 1989;80: 409.Google Scholar
40.Rychik, J, Piccoli, D, Barber, G. Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan operation. Am J Cardiol 1991;68: 819821.CrossRefGoogle Scholar
41.Kern, JH, Hinton, VJ, Nereo, NE, Hayes, CJ, Gersony, WM. Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome. Pediatrics 1998;102: 11481152.CrossRefGoogle ScholarPubMed
42.Du Plessis, AJ, Chang, AC, Wessel, DL, Lock, JE, Wernovsky, G, Newburger, JL, Mayer, JE Jr. Cerebrovascular accidents following the Fontan operation. Pediatr Neurol 1995;12: 230236.CrossRefGoogle ScholarPubMed
43.Rogers, BT, Msall, ME, Buck, GM, Lyons, NR, Norris, MK, Roland, JM, Gingell, RL, Cleveland, DC, Pleroni, DRNeurodevelopmental outcome of infants with hypoplastic left heart syndrome. J Pediatr 1995;126(3): 496498CrossRefGoogle ScholarPubMed
44.Rosenthal, D, Friedman, A, Kleinman, C, Kopf, G, Rosenfeld, L, Hellenbrand, W. Thromboembolic complications after Fontan operations. Circulation 1995;92 (Suppl): 11287–93.CrossRefGoogle ScholarPubMed
45.Umansky, R, Hauck, AAJ. Factors in the growth of children with ventricular septal defects. Pediatrics 1962;30: 540545.Google Scholar
46.Pittman, JG, Cohen, P. The pathogenesis of cardiac cachexia. N Engl J Med 1964;271: 403409.CrossRefGoogle ScholarPubMed
47.Menon, G, Poskitt, EM. Why does congenital heart disease cause failure to thrive? Arch Dis Child 1985;60: 11341139.CrossRefGoogle ScholarPubMed
48.Stocker, FP, Wilkoff, W, Miettinen, OS. Oxygen consumption in children with heart disease. J Pediatr 1972;80: 4351.CrossRefGoogle Scholar
49.Schwarz, SM., Gewitz, MH, See, CC, Berezin, S, Glassman, MS, Medow, CM, Fish, BC, Newman, LJ. Enteral nutrition in infants with congenital heart disease and growth failure. Pediatrics 1990;86(3): 368373.Google ScholarPubMed
50.Hintz, RL, Suskind, R, Amatayakal, KTanangkul, O, Olson, R. Plasma somatomedin and growth hormone levels in children with protein-calorie malnutrition. J Pediatr 1978;92(1): 153156.CrossRefGoogle ScholarPubMed
51.Bernstein, D, Jasper, JR, Rosenfeld, RGHintz, RL. Decreased serum insulin-like growth factor-I associated with growthfailure in newborn lambs with experimental cyanotic heart disease. J Clin Invest 1992;89: 11281132.CrossRefGoogle ScholarPubMed
52.Lange, PESievers, HH, Onnasch, DGW, Yacoub, MH, Bernhard, A, Heinrzen, PH. Up to 7 years of follow-up after two-stage anatomic correction of simple transposition of the great arteries. Circulation 1986;74: 4752.Google ScholarPubMed
53.Schuurmans, FM, Heintzberger-Pulles, CFM, Gerver, WJM, Kester, ADM, Forget, P-Ph. Long-term growth of children with congenital heart disease: a retrospective study. Acta Pediatr 1998;87: 12501255.CrossRefGoogle ScholarPubMed
54.Casey, FA, Craig, BG, Mulholland, HC. Quality of life in surgically palliated complex congenital heart disease. Arch Dis Child 1994;70: 382386.CrossRefGoogle ScholarPubMed
55.Farrell, AG, Schamberger, MS, Olson, IL, Ensing, GJ, Leitch, CA. Large left to right shunts and congestive heart failure increase total energy expenditure in infants with ventricular septal defects [abstract]. Pediatrics 1998;102(3): 678.Google Scholar
56.Gewillig, M. The Fontan circulation: Late functional results. Seminars in Thorac and Cardiovasc Surg 1994;6(1): 5663Google ScholarPubMed
57.National cooperative growth study: Ten years of guidance in growth. J Pediatr 1997;131: 281–82.Google Scholar
58.Lee, PDK, Hintz, RL, Sperry, JB, Baxter, TLC, Powell, DR. Insulin-like growth factor binding proteins in growth retarded children with chronic renal failure. Pediatr Res 1989;26:308315.CrossRefGoogle ScholarPubMed
59.Oberfield, SE, Chin, D, Uli, N, Dand, R, Sklar, C. Endocrine late effects of childhood cancer. J Pediatr 1997;131: S37–41.CrossRefGoogle Scholar
60.Hubbard, US, Davis, TB, Di Sant'agnese, PA, Gorden, PSchwartz, RH. Isolated growth hormone deficiency and cystic fibrosis: a report of two cases. Am J Dis Child 1980;134: 317319.Google ScholarPubMed
61.Hardin, DS. Effects of growth hormone treatment in children with cystic fibrosis: The National Cooperative Growth Study experience. J Pediatr 1997;13: S65–69.CrossRefGoogle Scholar
62.Sasaki, H, Baba, K, Nishida, Y. Treatment of children with congenital heart disease and growth retardation with recombinanr growth hormone. Acta Pediatr 1996;85: 251253.CrossRefGoogle ScholarPubMed
63.Eidee, E. A follow-up study of physical growth following failure to thrive with special reference to a critical period in the first year of life. Acta Pediatr Scand 1971;60: 39–52.Google Scholar
64.Humphrey, AWebber, S, McCaughey, ES. Cardiac effects of growth hormone in short normal children: Results of longterm treatment. [abstract] J Am Coll Cardiol 1999;33: 536A.Google Scholar
65.Cittadini, A, Cuocolo, AMerola, B, Fazio, S, Sabatini, A, Nicolai, E, Colao, A, Longobandi, S, Lombard, G, Sacca, L. Impaired cardiac performance in GH-deficient adults and its improvement after GH replacement. Am J Physiol. 1994;267: E219–225.Google ScholarPubMed
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Somatic growth failure after the Fontan operation
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