Management of children with dilated cardiomyopathy in The Netherlands: Implications of a low early transplantation rate

Susanna L. den Boer, M. (Lennie) van Osch-Gevers, Gijs van Ingen, Gideon J. du Marchie Sarvaas, Gabrielle G. van Iperen, Ronald B. Tanke, Ad P. C. M. Backx, Arend D. J. ten Harkel, Willem A. Helbing, Tammo Delhaas, Ad J. J. C. Bogers, Lukas A. J. Rammeloo, Michiel Dalinghaus*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

27 Citations (Web of Science)

Abstract

BACKGROUND: The policy for listing and transplant for children with dilated cardiomyopathy (DCM) in The Netherlands has been conservative because of low donor availability. The effects of this policy on outcome are reported. METHODS: This was a multicenter, nationwide study performed in 148 children with DCM. The primary outcome was death or heart transplant. RESULTS: Overall, 43 patients (29%) died or were transplanted. Within 1 year of diagnosis, 21 patients died, and only 4 underwent transplantation (3 on mechanical circulatory support). The I-year survival was 85% (95% confidence interval [CI] = 79-91), and 5-year survival was 84% (95% CI = 78-90). Transplantation-free survival at 1 year was 82% (95% CI = 75-88) and at 5 years was 72% (95% CI = 64-80). Within 1 year of diagnosis, with death as the main end-point (21 of 25, 84%), intensive care unit admission (hazard ratio = 2.6, p = 0.05) and mechanical circulatory support (hazard ratio = 3.2, p = 0.03) were risk factors (multivariable Cox analysis); inotropic support was longer in patients reaching an end-point. At >1 year after diagnosis, with transplantation as the main end-point (15 of 18, 83%), age >6 years (hazard ratio = 6.1, p = 0.02) was a risk factor. There were 56 (38%) children who recovered, 50% within 1 year of diagnosis. Recovery was associated with younger age; was similar in patients with myocarditis (43%) and idiopathic disease (41%); and was similar in patients initially admitted to the intensive care unit, admitted to the ward, or treated as outpatients. CONCLUSIONS: The transplantation rate in our cohort in the first year was low, with 1-year and 5-year survival rates similar to other cohorts. Our results suggest that a conservative approach to list children for transplantation early after presentation may be justifiable except for patients with prolonged intensive care unit or mechanical circulatory support.
Original languageEnglish
Pages (from-to)963-969
JournalJournal of Heart and Lung Transplantation
Volume34
Issue number7
DOIs
Publication statusPublished - Jul 2015

Keywords

  • cardiomyopathy
  • heart failure
  • pediatric cardiology
  • heart transplantation

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