Favourable mid-term outcome after heart transplantation for late Fontan failure

Guido Michielon, Joost P. van Melle, Djoeke Wolff, Duccio D. Di Carlo, Jeffrey P. Jacobs, Ilkka P. Mattila, Hakan Berggren, Harald Lindberg, Massimo A. Padalino, Bart Meyns, René Prêtre, Morten Helvind, Thierry Carrel, Tjark Ebels

Research output: Contribution to journalArticle

Abstract

OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.

Original languageEnglish
Article numberezu280
Pages (from-to)665-671
Number of pages7
JournalEuropean Journal of Cardio-thoracic Surgery
Volume47
Issue number4
DOIs
Publication statusPublished - Apr 1 2015

Fingerprint

Heart Transplantation
Protein-Losing Enteropathies
Ventricular Function
Survival
Kaplan-Meier Estimate
Mortality
Hospital Mortality
Coinfection
Survivors
Cardiac Arrhythmias
Pediatrics
Transplants

Keywords

  • Congenital heart disease
  • Failing fontan
  • Fontan operation
  • Heart failure
  • Heart transplantation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine
  • Medicine(all)

Cite this

Michielon, G., van Melle, J. P., Wolff, D., Di Carlo, D. D., Jacobs, J. P., Mattila, I. P., ... Ebels, T. (2015). Favourable mid-term outcome after heart transplantation for late Fontan failure. European Journal of Cardio-thoracic Surgery, 47(4), 665-671. [ezu280]. https://doi.org/10.1093/ejcts/ezu280

Favourable mid-term outcome after heart transplantation for late Fontan failure. / Michielon, Guido; van Melle, Joost P.; Wolff, Djoeke; Di Carlo, Duccio D.; Jacobs, Jeffrey P.; Mattila, Ilkka P.; Berggren, Hakan; Lindberg, Harald; Padalino, Massimo A.; Meyns, Bart; Prêtre, René; Helvind, Morten; Carrel, Thierry; Ebels, Tjark.

In: European Journal of Cardio-thoracic Surgery, Vol. 47, No. 4, ezu280, 01.04.2015, p. 665-671.

Research output: Contribution to journalArticle

Michielon, G, van Melle, JP, Wolff, D, Di Carlo, DD, Jacobs, JP, Mattila, IP, Berggren, H, Lindberg, H, Padalino, MA, Meyns, B, Prêtre, R, Helvind, M, Carrel, T & Ebels, T 2015, 'Favourable mid-term outcome after heart transplantation for late Fontan failure', European Journal of Cardio-thoracic Surgery, vol. 47, no. 4, ezu280, pp. 665-671. https://doi.org/10.1093/ejcts/ezu280
Michielon, Guido ; van Melle, Joost P. ; Wolff, Djoeke ; Di Carlo, Duccio D. ; Jacobs, Jeffrey P. ; Mattila, Ilkka P. ; Berggren, Hakan ; Lindberg, Harald ; Padalino, Massimo A. ; Meyns, Bart ; Prêtre, René ; Helvind, Morten ; Carrel, Thierry ; Ebels, Tjark. / Favourable mid-term outcome after heart transplantation for late Fontan failure. In: European Journal of Cardio-thoracic Surgery. 2015 ; Vol. 47, No. 4. pp. 665-671.
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title = "Favourable mid-term outcome after heart transplantation for late Fontan failure",
abstract = "OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8{\%}) or total cavopulmonary connection (44 patients = 72.2{\%}). Modality of FF included arrhythmia (14.8{\%}), complex obstructions in the Fontan circuit (16.4{\%}), protein-losing enteropathy (PLE) (22.9{\%}), impaired ventricular function (31.1{\%}) or a combination of the above (14.8{\%}). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18{\%}, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3{\%}, mainly secondary to infection (36.4{\%}) and graft failure (27.3{\%}). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8{\%} at 1 year, 73 ± 2.7{\%} at 5 years and 56.8 ± 4.3{\%} at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9{\%} in late FF and 32.7 ± 15.0{\%} in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8{\%} 5-year OHT survival. PLE was cured in 77.7{\%} of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5{\%} in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.",
keywords = "Congenital heart disease, Failing fontan, Fontan operation, Heart failure, Heart transplantation",
author = "Guido Michielon and {van Melle}, {Joost P.} and Djoeke Wolff and {Di Carlo}, {Duccio D.} and Jacobs, {Jeffrey P.} and Mattila, {Ilkka P.} and Hakan Berggren and Harald Lindberg and Padalino, {Massimo A.} and Bart Meyns and Ren{\'e} Pr{\^e}tre and Morten Helvind and Thierry Carrel and Tjark Ebels",
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T1 - Favourable mid-term outcome after heart transplantation for late Fontan failure

AU - Michielon, Guido

AU - van Melle, Joost P.

AU - Wolff, Djoeke

AU - Di Carlo, Duccio D.

AU - Jacobs, Jeffrey P.

AU - Mattila, Ilkka P.

AU - Berggren, Hakan

AU - Lindberg, Harald

AU - Padalino, Massimo A.

AU - Meyns, Bart

AU - Prêtre, René

AU - Helvind, Morten

AU - Carrel, Thierry

AU - Ebels, Tjark

PY - 2015/4/1

Y1 - 2015/4/1

N2 - OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.

AB - OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.

KW - Congenital heart disease

KW - Failing fontan

KW - Fontan operation

KW - Heart failure

KW - Heart transplantation

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