Clinical characteristics and outcomes of patients with adult congenital heart disease listed for heart and heart‒lung transplantation in the Eurotransplant region.

Becher, Peter Moritz;Schrage, Benedikt;Weimann, Jessica;Smits, Jacqueline;Sinning, Christoph;et.al.
(2020) The Journal of Heart and Lung Transplantation — Vol. 39, n° 11, p. 1238-1249 (2020)

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Authors
  • Becher, Peter Moritz
    Author
  • Schrage, Benedikt
    Author
  • Weimann, Jessica
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  • Smits, Jacqueline
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  • Van Caenegem, OlivierUCLouvain
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  • Sinning, Christoph
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Abstract
BACKGROUND: The therapeutic success in patients with congenital heart disease (CHD) leads to a growing number of adults with CHD (adult CHD [ACHD]) who develop end-stage heart failure. We aimed to determine patient characteristics and outcomes of ACHD listed for heart transplantation. METHODS: Using data from all the patients with ACHD in 20 transplant centers in the Eurotransplant region from 1999 to 2015, we analyzed patient characteristics, waiting list, and post-transplantation outcomes. RESULTS: A total of 204 patients with ACHD were listed during the study period. The median age was 38 years, and 62.3% of the patients were listed in high urgency (HU), and 37.7% of the patients were in transplantable (T)-listing status. A total of 23.5% of the patients died or were delisted owing to clinical worsening, and 75% of the patients underwent transplantation. Median waiting time for patients with HU-listing status was 4.18 months and with T-listing status 9.07 months. There was no difference in crude mortality or delisting between patients who were HU status listed and T status listed (p = 0.65). In multivariable regression analysis, markers for respiratory failure (mechanical ventilation, hazard ratio [HR]: 1.41, 95% CI: 1.11-1.81, p = 0.006) and arrhythmias (anti-arrhythmic medication, HR: 1.42, 95% CI: 1.01-2.01, p = 0.044) were associated with a higher risk of death or delisting. In the overall cohort, post-transplantation mortality was 26.8% after 1 year and 33.4% after 5 years. CONCLUSIONS: Listed patients are at high risk of death without differences in the urgency of listing. Respiratory failure requiring invasive ventilation and possibly arrhythmias requiring anti-arrhythmic medication indicate worse outcomes on waiting list.
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Becher, P. M., Schrage, B., Weimann, J., Smits, J., Magnussen, C., Reichenspurner, H., Goßling, A., Rodrigus, I., Dumfarth, J., de Pauw, M., François, K., Van Caenegem, O., Ancion, A., Van Cleemput, J., Milicic, D., Moza, A., Schenker, P., Röhrich, L., Schönrath, F., et al. (2020). Clinical characteristics and outcomes of patients with adult congenital heart disease listed for heart and heart‒lung transplantation in the Eurotransplant region. The Journal of Heart and Lung Transplantation, 39(11), 1238-1249. https://doi.org/10.1016/j.healun.2020.07.012 (Original work published 2020)