Hostname: page-component-cd9895bd7-gxg78 Total loading time: 0 Render date: 2024-12-27T19:09:01.408Z Has data issue: false hasContentIssue false
  • English
  • Français

Growth of children with a functionally single ventricle following palliation at moderately increased altitude

Published online by Cambridge University Press:  19 August 2008

Ronald W. Day ,
David M. Denton  and
W. Daniel Jackson
Ronald W. Day*
Affiliation:
Department of Pediatrics, University of Utah and Primary Children's Medical Center, Salt Lake City, UTUSA
David M. Denton
Affiliation:
Department of Pediatrics, University of Utah and Primary Children's Medical Center, Salt Lake City, UTUSA
W. Daniel Jackson
Affiliation:
Department of Pediatrics, University of Utah and Primary Children's Medical Center, Salt Lake City, UTUSA
*
Ronald W Day, MD, Division of Pediatric Cardiology, Primary Children's Medical Center, 100 North Medical Drive, Salt Lake City, UT 84113, USA. Tel: (801) 588–2600; Fax: (801) 588–2612
Get access Rights & Permissions [Opens in a new window]

Abstract

The bidirectional Glenn and Fontan procedures are empirically performed as interim and definitive procedures in children with a functionally single ventricle. The optimal stage of palliation, nonetheless, remains unknown. During childhood, growth is a fundamental measure of response to therapy. Growth may be influenced by the degree of cyanosis, the volume load on the ventricle, and cardiac performance. Thus, the weight and stature of children with a functionally single ventricle who underwent a bidirectional Glenn procedure or a Fontan procedure were studied to determine the effect of each intervention on growth. Z scores for weight and stature were retrospectively determined prior to palliation, at yearly intervals for 4 years, and from long-term measurements until 18 years of age in all patients with at least 2 years of observation following palliation. Growth was evaluated in 54 patients with a bidirectional Glenn procedure, and 65 patients with a Fontan procedure. The Z scores for weight were improved after each method of surgical palliation. Stature, however, was improved only following the bidirectional Glenn procedure. Growth was impaired in patients who developed protein losing enteropathy. Weight improved only during the initial 2 years after the Fontan procedure in patients who had a surgical fenestration. Over the long-term, patients who underwent a Fontan procedure were more likely to have a Z score less than −2.0 for weight and stature than patients who underwent only a bidirectional Glenn procedure. Late mortality and the incidence of heart transplantation were increased in patients who experienced a decrease in their rate of growth, defined as a negative change of more than one Z score in weight or stature, following the Fontan procedure. In conclusion, at moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure. A decrease in the rate of growth is associated with a poor prognosis following the Fontan procedure.

Keywords

Bidirectional Glenn procedurecyanosisFontan proceduregrowthheart defectscongenitalprotein losing enteroparhy
Type
Original Articles
Information
Cardiology in the Young , Volume 10 , Issue 3 , May 2000 , pp. 193 - 200
Copyright
Copyright © Cambridge University Press 2000

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Glenn, WWL. Circulatory bypass of the right side of the heart.IV. Shunt between the superior vena cava and the right pulmonary artery-report of clinical application. New Engl J Med 1958; 259:117–120.CrossRefGoogle Scholar
2.Azzoiina, G, Eufrate, S, Pensa, P Tricuspid atresia. Experience in surgical management with a modified cavopulmonary anastomosis. Thorax 1972; 27: 111–115.CrossRefGoogle Scholar
3.Salmon, APSethia, B, Silove, D, Goh, D, Alton, MH, deGiovanni, JV, Wright, JGC, Abrams, LD. Cavopulmonary anastornosis as long-term palliation for patients with tricuspid atresia. Eur J Cardiothorac Surg 1989; 3: 494–498.CrossRefGoogle ScholarPubMed
4.Trusler, GA, Williams, WG, Cohen, AJ, Rabinovitch, M, Moes, CAF, Smallhorn, JF, Coles, JG, Lightfoot, NE, Freedom, PThe cavopuimonary shunt: evolution of a concept. Circulation 1990; 82: IV–131–IV–138.Google ScholarPubMed
5.Bridges, ND, Jonas, RA, Mayer, JE, Flanagan, MF, Keane, JF, Casteneda, AR. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates: early results. Circulation 1990; 82: IV–170–IV–176.Google ScholarPubMed
6.Fontan, F, Mounicot, FB, Baudet, E, Simmoneau, J, Gordo, J, Gouffrant, JM. “Correction” de l'atresie tricuspidienne. Rapport de deux cas “corriges” par lutilisation dune technique chirur gicale nouvelle. Ann Chir Thorac Cardiovasc 1971; 10: 39–47.Google Scholar
7.Kreutzer, G, GalIndez, E, Bono, H, dePalms, C, Laura, JPAn operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 1973; 66: 613–621.CrossRefGoogle ScholarPubMed
8.De Leval, MR, Kilner, P, Gewillig, M, Bull, C. Total cavopulmonary connection: a logical alternative to atriopuimonary connection for complex Fontan operations. J Thorac Cardiovasc Surg 1988; 96: 682–695.CrossRefGoogle ScholarPubMed
9.Bridges, ND, Lock, JE, Casteneda, AR. Baffle fenestration with subsequent transcatheter closure: modification of the Fontan operation for patients at increased risk. Circulation 1990; 82: 1681–1689.CrossRefGoogle ScholarPubMed
10.Laks, H, Pearl, JM, Haas, GS, Drinkwater, DC, Milgaiter, E, Jarmakani, JM, lsabel-Jones, J, George, BL, Williams, KG. Partial Fontan: advantages of an adjustable interatrial communi cation. Ann Thorac Surg 1991; 52:1084–1095.CrossRefGoogle Scholar
11.Day, RW, Baker, CM, Caton, JR, Hawkins, JA, McGough, EC. Bidirectional cavopulmonary shunt with an additional source of pulmonary flow: an interim or final stage of palliation. Cardiol Young 1997; 7: 63–70.CrossRefGoogle Scholar
12.Rosenthal, A, Casteneda, AR. Growth and development after cardiovascular surgery in infants and children. Prog Cardiovasc Dis 1975; 18: 27–37.CrossRefGoogle ScholarPubMed
13.Weintraub, KG, Menahem, S. Eady surgical closure uf a large ventricular septal defect: influence on long-term growth. J Am Coll Cardiol 1991; 18: 552–558.Google Scholar
14.Day, RW, Orsmond, GS, Sturtevant, JE, Hawkins, JA, Duty, DB, McGough, EC. Early and intermediate results of the Fontan procedure at moderately high altitude. Ann Thorac Surg 1994; 57: 170–176.CrossRefGoogle ScholarPubMed
15.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: 602–629.CrossRefGoogle ScholarPubMed
16.Hijazi, ZM, Fahey, JT, Kleinman, CS, Kopf, GS, Hellenbrand, WE. Hemodynamic evaluation before and after closure of fenestrated Fontan: an acute study of changes in oxygen delivery. Circulation 1992; 86: 196–202.CrossRefGoogle Scholar
17.Mavroudis, C, Zales, VR, Backer, CL, Muster, AJ, Latson, LA. Fenestrated Fontan with delayed catheter closure: effects of volume loading and baffle fenestration on cardiac index and oxygen delivery. Circulation 1992; 86: 11–85–11–92.Google ScholarPubMed
18.Harake, B, Kuhn, MA, Jarmakani, JM, Laks, H, Al-Khatib, Y, Elami, A, Williams, KG. The acute hemodynamic effects of adjustable atrial septal defect closure in the lateral tunnel Fontan. J Am Coil Cardiol 1994; 23: 1671–1676.CrossRefGoogle ScholarPubMed