Guido Michielon1, Joost P van Melle2, Djoeke Wolff3, Duccio Di Carlo4, Jeffrey P Jacobs5, Ilkka P Mattila6, Hakan Berggren7, Harald Lindberg8, Massimo A Padalino9, Bart Meyns10, René Prêtre11, Morten Helvind12, Thierry Carrel13, Tjark Ebels3. 1. Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, Netherlands g.michielon@umcg.nl. 2. Department of Cardiology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands. 3. Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, Netherlands. 4. DMCCP, Ospedale Pediatrico Bambino Gesù, Rome, Italy. 5. Congenital Heart Institute of Florida, St Petersburg, FL, USA. 6. Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland. 7. Children's Heart Center, The Queen Silvia Children's Hospital, Goteborg, Sweden. 8. Rikshospitalet, University Hospital Oslo, Oslo, Norway. 9. Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy. 10. Department of Cardiac Surgery, University Hospital UZ Leuven, Leuven, Belgium. 11. Pediatric and Congenital Cardiac Surgery, University Children's Hospital, Zurich, Switzerland. 12. Department of Cardiothoracic Surgery, Rigs Hospitalet, Copenhagen, Denmark. 13. Clinic for Cardiovascular Surgery, Swiss Cardiovascular Center University Hospital, Bern, Switzerland.
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.
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.
Authors: Jacob R Miller; Kathleen E Simpson; Deirdre J Epstein; Timothy S Lancaster; Matthew C Henn; Richard B Schuessler; David T Balzer; Shabana Shahanavaz; Joshua J Murphy; Charles E Canter; Pirooz Eghtesady; Umar S Boston Journal: J Heart Lung Transplant Date: 2016-03-10 Impact factor: 10.247
Authors: Sophie L Meyer; Djoeke Wolff; Floris-Jan S Ridderbos; Graziella Eshuis; Hans Hillege; Tineke P Willems; Tjark Ebels; Joost P van Melle; Rolf M F Berger Journal: J Am Heart Assoc Date: 2020-05-08 Impact factor: 5.501
Authors: Gregor Gierlinger; Eva Sames-Dolzer; Michaela Kreuzer; Roland Mair; Mohammad-Paimann Nawrozi; Andreas Tulzer; Christoph Bauer; Gerald Tulzer; Rudolf Mair Journal: Interact Cardiovasc Thorac Surg Date: 2022-08-03