Hostname: page-component-586b7cd67f-t8hqh Total loading time: 0 Render date: 2024-11-23T04:58:41.308Z Has data issue: false hasContentIssue false
  • English
  • Français

Outcomes for children with cardiomyopathy awaiting transplantation

Published online by Cambridge University Press:  19 August 2008

Lynne E. Nield ,
Brian W. McCrindle ,
Desmond J. Bohn ,
Lori J. West ,
Jhon G. Coles ,
Robert M. Freedom  and
Lee N. Benson
Desmond J. Bohn
Affiliation:
Departments of Paediatrics, Surgery and Critical Care; The Hospital for Sick Children, The University of Toronto School of Medicine, Toronto, Ontario, Canada
Jhon G. Coles
Affiliation:
Division of Cardiology and Cardiovascular Surgery,The Hospital for Sick Children, The University of Toronto School of Medicine, Toronto, Ontario, Canada
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective

To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies.

Background

Childhood cardiomyopathies form a heterogeneous group of diseases, and in many, the prognosis is poor, irrespective of the etiology. When profound heart failure develops, cardiac transplantation can be the only viable option for survival.

Methods

We included all children with cardiomyopathy listed for transplantation between 12/89 and 4/98 in this historical cohort study.

Results

We listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with restrictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted within 30 days of listing. Five patients (16%) died before transplantation. Within the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, having recovered from myocarditis, and two remain waiting transplantation after intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third or fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical ventricular assistance, in 10 patients, was not a predictor of an adverse outcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure.

Conclusions

Children with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.

Keywords

Cardiomyopathytransplantationheart failure
Type
Original Articles
Information
Cardiology in the Young , Volume 10 , Issue 4 , July 2000 , pp. 358 - 366
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

Arola, A, Tuominen, J, Ruuskanen, O, Jokinen, E. Idiopathic dilated cardiomyopathy in children: prognostic indicators and outcome. Pediatrics 1998; 101: 369–376.CrossRefGoogle Scholar
Akagi, T, Benson, LN, Lightfoot, NE, Chin, K, Wilson, G, Freedom, RM. Natural history of dilated cardiomyopathy in children. Am Heart J 1991; 121: 1502–1506.CrossRefGoogle ScholarPubMed
Gewillig, M, Mertens, L, Moerman, P, Dumoulin, M. Idiopathic restrictive cardiomyopathy in childhood. Eur Heart J 1996; 17: 1413–1420.CrossRefGoogle ScholarPubMed
Denfield, SW, Rosenthal, G, Gajarski, RJ, Bricker, JT,Schowengerdt, KO, Price, JK, Towbin, JA. Restrictive cardiomyopathies in childhood. Tex Heart Inst J 1997; 24: 38–44.Google ScholarPubMed
Lewis, AB. Clinical profile and outcome of restrictive cardiomyopathy in children. Am Heart J 1992; 123: 1589–1593.CrossRefGoogle ScholarPubMed
Cetta, F, O'Leary, PW, Seward, JB, Driscoll, DJ. Idiopathic restrictive cardiomyopathy in childhood: diagnostic features and clinical course. Mayo Clin Proc 1995; 70: 634–640.CrossRefGoogle ScholarPubMed
Boucek, MM, Novick, RJ, Bennett, LE, Fiol, B, Keck, BM, Hosenpud, JD. The Registry of the International Socitey of Heart and Lung Transplantation: First official pediatric report— 1997. The Journal of Heart and Lung Transplantation 1-12-1997; 16: 1189–1206.Google Scholar
Shaddy, R, Naftel, DC, Kirklin, JK, Boyle, G, McGiffin, DC, Towbin, JA, Ring, WS, Pearce, B, Addonizio, L, and Morrow, WR. Outcome of cardiac transplantation in children. Survival in a contemporary multi-institutional experience. Pediatric Heart Transplant Study. Circulation 94[9 Suppl], 1169–1173. 1996.Google Scholar
Canter, C, Naftel, D, Caldwell, R, Chinnock, R, Pahl, E, Frazier, E, Kirklin, JK, Boucek, MM, and Morrow, WR. Survival and risk factors for death after cardiac transplantation in infants. Amulti-institutional study. The Pediatric Heart Transplant Study. Circulation 96[1], 227–231. 1997.CrossRefGoogle Scholar
Lewis, AB. Prognostic value of echocardiography in children with idiopathic dilated cardiomyopathy. Am Heart J 1994; 128: 133–136.CrossRefGoogle ScholarPubMed
Pelliccia, F, d'Amati, G, Cianfrocca, C, Bernucci, P, Nigri, AMarino, B, Gallo, p. Histomorphometric features predict l-year outcome of patient with idiopathic dilated cardiomyopathy considerd to be at be at low priorty for cardiac transplantation Am Heart J 1994; 128: 316–325.CrossRefGoogle Scholar
Carvalho, JS, Silva, CMC, Shinebourne, EA, Redington, AN. Prognostic value of posterior wall thickness in childhood dilated cardiomyopathy and myocarditis. Eur Heart J 1996; 17: 1233–1238.CrossRefGoogle Scholar
Obrador, D, Ballester, M, Carrio, I, Moya, C, Bosch, I,Marti, V, Berna, L, Estorch, M, Udina, C, Marrugat, J, Auge, JM, Carreras, F, Pons-Llado, G, Caralps, JM. Presence,evolving changes and prognostic implications of myocardialdamage detected in idio-pathic and alcoholic dilated cardiomyopathy by 111 In mono-clonal antimyosin antibodies. Circulation 1994;89: 2054–2061.CrossRefGoogle Scholar
Juilliere, Y, Barbier, G, Feldmann, L, Grentzinger, A, Danchin, N, Cherrier, F. Additional predictive value of both left and right ventricular ejection fractions on long-tem survival in diopathic dilated cardiomyopathy Eur Heart J.1997; 18: 276–280.CrossRefGoogle Scholar
Stelken, AM, Younis, LT, Jennison, SH, Miller, DD, Miller, LW, Shaw, LJ, Kargl, D, Chaitman, BR. Prognostic value of cardiopul-opathy. J Am Coll Cardiol 1996; 27: 345–352.CrossRefGoogle Scholar
Grzybowski, J, Bilinska, ZT, Ruzyllo, W, Kupsc, W, Michalak, E, Szczesniewska, D, Poplawska, W, Rydlewska-Sadowska, w. Determinants of progosis in nonischemic dilared cardimy-opathy. J Cardiac Failure 1996;2 77–85.CrossRefGoogle Scholar
Mancini, DM, Eisen, H, Kussmaul, W, Mull, R, Edmunds, LH Jr, Wilson, JR. Value of peak exercise Oxygen consumption for optimal timing of cardiac transplantation in ambulatory patiens with heart failure. Circulation 1991; 83: 778–786.CrossRefGoogle Scholar
Friedman, RA, Moak, JP, Garson, A. Clinical course of idiopathic dilated cardiomyopathy in children.J Am Coll Cardiol 1991;18: 152–156.CrossRefGoogle ScholarPubMed
Wiles, HB, McArthur, PD, Taylor, AB, Gillette, Pc, Fyfe, DA, Matthews, JP, Simpson, JW. Prognostic features of children with idiopathic dilated cardiomyopathy. Am J Cardiol 1991; 68: 1372–1376.CrossRefGoogle ScholarPubMed
McGiffin, DC, Naftel, DC, Kirklin, JK, Morrow, WR, Towbin, J, Shaddy, R, Alejos, J, Rossi, A and pediatric Heart Transplant Study Gorup. Prediction outcome after listing for heart transplantation in childern: comparison of kaplan-Meier and paramtric competing risk analysis. J Heart Lung Transplant 1997; 16: 713–722.Google Scholar
Morrow, WR, Nafrel, DC, Chinoock, R, Canter, C, Boucek, MM, Zales, V, McGiffin, DC, Kirklin, JK. Outcome of listing for heart transplantation in infants yonger than six months: predictors of death and intercal to transplantation. The Pediatric Heart Transplantation Study Group. Heart Lung transplant 1997;16:1255–1266.Google Scholar
Benson, LN, Pearlman, L, Gildner, J, Coles, JG, and West, LJ.Paedistric Cardiac Transplantation Manual: A guide for health care delivery for the patient and family requiring cardiac transplantation. 1998.Google Scholar
Griffin, ML, Hernandez, A, Martin, TC, Goldring, D, Bolman, RM, Spray, TL and strauss, AW. Delated cardiomyopathy in infants and children J Am Coll Cardiol 11 [1],139–144 1988.CrossRefGoogle Scholar
Morley, D, Brozena, SC. Assessing risk by hemodynamic profile in patients awaiting cardiac transplantation. Am J Cardiol 1994; 73: 379–383.CrossRefGoogle ScholarPubMed
Gavazzi, A, Berzuini, C, Campana, C, Inserra, C, Ponzetta, M, Sebastiani, R, Ghio, S, Recusani, F. Value of right ventricular ejection fraction in predicting short-term prognosis of patients with severe chronic heart failure. J Heart Lung Transplant 1997; 16:774–785.Google ScholarPubMed
Campana, C, Gavazzi, A, Berzuini, C, Larizza, C, Marioni, R, D'Armim, A, Pederzolli, N, Martinelli, L, Vigano, M. Predictors of prognosis in patients awaiting heart transplantation. J Heart Lung Transplant 1993; 12: 756–765.Google ScholarPubMed
Di, Lenarda A, Secoli, G, Perkan, A, Gregori, D, Lardieri, G, Pinamonti, B, Sinagra, G, Zecchin, M, Camerini, F, and the Heart Muscle Disease Study Group. Changing mortality in dilated cardiomyopathy. Br Heart J 1994; 72 (Supplement): S46–S51.Google Scholar
Regitz-Zagrosek, V, Leuchs, B, Krulls-Munch, J, Fleck, E. Angiotensin-converting enzyme inhibitors and beta-blockers in long-term treatment of dilated cardiomyopathy. Am Heart J 1995; 129:754–761.CrossRefGoogle ScholarPubMed
del, Nido PJ, Armitage, JM, Fricker, FJ, Shaver, M, Cipriani, L, Dayal, G, Park, SC, Siewers, RD. Extracorporeal membrane oxygenation support as a bridge to pediatric heart transplantation. Circulation 1994; 90 (part 2): II–66.Google Scholar
Meliones, JN, Custer, JR, Snedecor, S, Moler, FW, O'Rourke, PP, Delius, RE. Extracorporeal life support for cardiac assist in pediatric patients. Review of ELSO Registry data. Circulation 1991; 84:III168–III172.Google ScholarPubMed
Delius, RE. Prolonged extracorporeal life support of pediatric and adolescent cardiac transplant patients. Ann Thorac Surg 1998; 65. 877–878.Google ScholarPubMed
del, Nido PJ. Extracorporeal membrane oxygenation for cardiac support in children. Ann Thorac Surg 1996; 61: 336–339.Google Scholar
Ishino, K, Weng, Y, Alexi-Meskishvili, VLoebe, M, Uhlemann, F, Lange, PE, Hetzer, RExtracorporeal membrane oxygenation as a bridge to cardiac transplantation in children. Art Org 1996, 20: 728–732.CrossRefGoogle ScholarPubMed
Ishino, K, Loebe, MUhlemann, F, Weng, Y, Hennig, E, Hetzer, R.Circulatory support with paracorporeal pneumatic ventricular assist device (VAD) in infants and children. Eur J Cardio Thorac Surg 1997; 11: 965–972CrossRefGoogle ScholarPubMed
Batista, RJ, Santos, JVL, Cunha, MA et al. , Ventriculectomia, parcial: um novo conceito no tratamento cirurgico de cardiopatias em fase final. Anais do XXII Congresso Nacional de Cirurgiia Cardiaca, Brasilia. Soc Brasileira de Cirurgia Cardiaca 1995; 140–141.Google Scholar