A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome

Friso M. Rijnberg, Nico A. Blom, Vladimir Sojak, Eline F. Bruggemans, Irene M. Kuipers, Lukas A. J. Rammeloo, Monique R. M. Jongbloed, Berto J. Bouma, Mark G. Hazekamp

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

OBJECTIVES: This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity. METHODS: Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016. RESULTS: The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1-Q3 7.6-17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18-44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63-96%) at 20 years for lateral tunnel and 99% (95% CI 96-100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III-IV) at 15 years was 65% (95% CI 52-76%) for atriopulmonary connection/Björk, 90% (95% CI 73-97%) for lateral tunnel and 90% (95% CI 82-94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2-5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4-9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4-9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0-11.1; P < 0.001). CONCLUSIONS: With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.
Original languageEnglish
Pages (from-to)461-468
JournalInteractive Cardiovascular and Thoracic Surgery
Volume29
Issue number3
DOIs
Publication statusPublished - 2019

Cite this

Rijnberg, Friso M. ; Blom, Nico A. ; Sojak, Vladimir ; Bruggemans, Eline F. ; Kuipers, Irene M. ; Rammeloo, Lukas A. J. ; Jongbloed, Monique R. M. ; Bouma, Berto J. ; Hazekamp, Mark G. / A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome. In: Interactive Cardiovascular and Thoracic Surgery. 2019 ; Vol. 29, No. 3. pp. 461-468.
@article{cee6de057523482b9fc39058d7af231a,
title = "A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome",
abstract = "OBJECTIVES: This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity. METHODS: Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016. RESULTS: The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Bj{\"o}rk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1{\%}). Median follow-up of the study cohort was 11.9 years (Q1-Q3 7.6-17.5). Longest survival estimates were 31{\%} [95{\%} confidence intervals (CI) 18-44{\%}] at 35 years for atriopulmonary connection/Bj{\"o}rk, 87{\%} (95{\%} CI 63-96{\%}) at 20 years for lateral tunnel and 99{\%} (95{\%} CI 96-100{\%}) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III-IV) at 15 years was 65{\%} (95{\%} CI 52-76{\%}) for atriopulmonary connection/Bj{\"o}rk, 90{\%} (95{\%} CI 73-97{\%}) for lateral tunnel and 90{\%} (95{\%} CI 82-94{\%}) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95{\%} CI 1.2-5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95{\%} CI 1.4-9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95{\%} CI 1.4-9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95{\%} CI 2.0-11.1; P < 0.001). CONCLUSIONS: With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.",
author = "Rijnberg, {Friso M.} and Blom, {Nico A.} and Vladimir Sojak and Bruggemans, {Eline F.} and Kuipers, {Irene M.} and Rammeloo, {Lukas A. J.} and Jongbloed, {Monique R. M.} and Bouma, {Berto J.} and Hazekamp, {Mark G.}",
year = "2019",
doi = "10.1093/icvts/ivz111",
language = "English",
volume = "29",
pages = "461--468",
journal = "Interactive Cardiovascular and Thoracic Surgery",
issn = "1569-9293",
publisher = "European Association for Cardio-Thoracic Surgery",
number = "3",

}

A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome. / Rijnberg, Friso M.; Blom, Nico A.; Sojak, Vladimir; Bruggemans, Eline F.; Kuipers, Irene M.; Rammeloo, Lukas A. J.; Jongbloed, Monique R. M.; Bouma, Berto J.; Hazekamp, Mark G.

In: Interactive Cardiovascular and Thoracic Surgery, Vol. 29, No. 3, 2019, p. 461-468.

Research output: Contribution to journalArticleAcademicpeer-review

TY - JOUR

T1 - A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome

AU - Rijnberg, Friso M.

AU - Blom, Nico A.

AU - Sojak, Vladimir

AU - Bruggemans, Eline F.

AU - Kuipers, Irene M.

AU - Rammeloo, Lukas A. J.

AU - Jongbloed, Monique R. M.

AU - Bouma, Berto J.

AU - Hazekamp, Mark G.

PY - 2019

Y1 - 2019

N2 - OBJECTIVES: This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity. METHODS: Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016. RESULTS: The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1-Q3 7.6-17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18-44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63-96%) at 20 years for lateral tunnel and 99% (95% CI 96-100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III-IV) at 15 years was 65% (95% CI 52-76%) for atriopulmonary connection/Björk, 90% (95% CI 73-97%) for lateral tunnel and 90% (95% CI 82-94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2-5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4-9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4-9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0-11.1; P < 0.001). CONCLUSIONS: With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.

AB - OBJECTIVES: This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity. METHODS: Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016. RESULTS: The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1-Q3 7.6-17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18-44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63-96%) at 20 years for lateral tunnel and 99% (95% CI 96-100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III-IV) at 15 years was 65% (95% CI 52-76%) for atriopulmonary connection/Björk, 90% (95% CI 73-97%) for lateral tunnel and 90% (95% CI 82-94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2-5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4-9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4-9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0-11.1; P < 0.001). CONCLUSIONS: With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.

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UR - https://www.ncbi.nlm.nih.gov/pubmed/31038168

U2 - 10.1093/icvts/ivz111

DO - 10.1093/icvts/ivz111

M3 - Article

VL - 29

SP - 461

EP - 468

JO - Interactive Cardiovascular and Thoracic Surgery

JF - Interactive Cardiovascular and Thoracic Surgery

SN - 1569-9293

IS - 3

ER -