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Incidence, pathophysiology, and treatment of failing Fontan after the total cavopulmonary connection

Published online by Cambridge University Press:  03 October 2024

Paula Gaebert Open the ORCID record for Paula Gaebert [Opens in a new window] ,
Thibault Schaeffer ,
Jonas Palm Open the ORCID record for Jonas Palm [Opens in a new window] ,
Chiara Di Padua Open the ORCID record for Chiara Di Padua [Opens in a new window] ,
Carolin Niedermaier ,
Nicole Piber ,
Alfred Hager ,
Peter Ewert ,
Jürgen Hörer Open the ORCID record for Jürgen Hörer [Opens in a new window]  and
Masamichi Ono Open the ORCID record for Masamichi Ono [Opens in a new window]
Paula Gaebert
Affiliation:
Department of Congenital and Pediatric Heart Surgery, Technische Universität München, German Heart Center Munich, Munich, Germany Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany Europäisches Kinderherzzentrum München, Munich, Germany
Thibault Schaeffer
Affiliation:
Department of Congenital and Pediatric Heart Surgery, Technische Universität München, German Heart Center Munich, Munich, Germany Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany Europäisches Kinderherzzentrum München, Munich, Germany
Jonas Palm
Affiliation:
Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich at the Technical University of Munich, Munich, Germany
Chiara Di Padua
Affiliation:
Department of Congenital and Pediatric Heart Surgery, Technische Universität München, German Heart Center Munich, Munich, Germany Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany Europäisches Kinderherzzentrum München, Munich, Germany
Carolin Niedermaier
Affiliation:
Department of Congenital and Pediatric Heart Surgery, Technische Universität München, German Heart Center Munich, Munich, Germany Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany Europäisches Kinderherzzentrum München, Munich, Germany
Nicole Piber
Affiliation:
Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany
Alfred Hager
Affiliation:
Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich at the Technical University of Munich, Munich, Germany
Peter Ewert
Affiliation:
Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich at the Technical University of Munich, Munich, Germany
Jürgen Hörer
Affiliation:
Department of Congenital and Pediatric Heart Surgery, Technische Universität München, German Heart Center Munich, Munich, Germany Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany Europäisches Kinderherzzentrum München, Munich, Germany
Masamichi Ono*
Affiliation:
Department of Congenital and Pediatric Heart Surgery, Technische Universität München, German Heart Center Munich, Munich, Germany
*
Corresponding author: Masamichi Ono; Email: [email protected]
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Abstract

Background:

Failing Fontan poses a significant clinical challenge. This study aims to improve patients’ outcomes by comprehensively understanding the incidence, pathophysiology, risk factors, and treatment of failing Fontan after total cavopulmonary connection.

Methods:

We performed a retrospective analysis of patients who underwent total cavopulmonary connection at the German Heart Center Munich between 1994 and 2022. The onset of failing Fontan was defined as: protein-losing enteropathy, plastic bronchitis, NYHA class IV, NYHA class III for > one year, unscheduled hospital admissions for heart failure symptoms, and evaluation for heart transplantation.

Results:

Among 634 patients, 76 patients presented with failing Fontan, and the incidence was 1.48 per 100 patient-years. Manifestations included protein-losing enteropathy (n = 34), hospital readmission (n = 28), NYHA III (n = 18), plastic bronchitis (n = 16), evaluation for heart transplantation (n = 14), and NYHA IV (n = 4). Risk factors for the onset of failing Fontan were dominant right ventricle (p = 0.010) and higher pulmonary artery pressure before total cavopulmonary connection (p = 0.004). A total of 72 interventions were performed in 59 patients, including balloon dilatation/stent implantation in the total cavopulmonary connection pathway (n = 49) and embolization of collaterals (n = 24). Heart transplantation was performed in four patients. The survival after the onset of Fontan failure was 77% at 10 years. Patients with failing Fontan revealed significantly higher zlog-NT-proBNP levels after onset compared to those without (p = 0.021)

Conclusions:

The incidence of Fontan failure was 1.5 per 100 patient years. Dominant right ventricle and higher pulmonary artery pressure before total cavopulmonary connection were significant risks for the onset of failing Fontan. Zlog-NT-proBNP is only a late marker of Fontan failure.

Keywords

Failing fontantotal cavopulmonary connectionventricular assist deviceheart transplantation
Type
Original Article
Information
Cardiology in the Young , Volume 34 , Issue 11 , November 2024 , pp. 2406 - 2413
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Copyright
© The Author(s), 2024. Published by Cambridge University Press

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Footnotes

Paula Gaebert and Thibault Schaeffer contributed equally.

Meeting presentation: Presented at the 60th Annual Meeting of the Society of Thoracic Surgeons, San Antonio, Texas, January 27-29, 2024.

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