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Influence of fenestration on long-term Fontan survival

Published online by Cambridge University Press:  31 August 2021

Ondřej Materna*
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Vojtěch Illinger
Affiliation:
Department of Rehabilitation and Sports Medicine, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Denisa Jičínská
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Karel Koubský
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Jan Kovanda
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Miroslav Ložek
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Petr Tax
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Oleg Reich
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Václav Chaloupecký
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
Jan Janoušek
Affiliation:
Children’s Heart Centre, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
*
Author for correspondence: O. Materna, MD, Children’s Heart Centre, Motol University Hospital, V Úvalu 84, 150 06, Prague 5, Czech Republic. Tell: +420224432991; Fax: +420224432920 E-mail: ondrej.materna@fnmotol.cz

Abstract

Introduction:

Fenestration in the total cavopulmonary connection system may improve the outcome of patients with significant risk factors for Fontan haemodynamics. Our study aims to analyse the difference in long-term survival between non-fenestrated and fenestrated patients.

Methods:

All consecutive patients (n = 351) who underwent total cavopulmonary connection between 1992 and 2016 were identified. Six early deaths were excluded resulting in a group of 345 patients. Median (interquartile range,) length of follow-up was 14.4 (7.1–19.7) years. Freedom from the composite endpoint of death, total cavopulmonary connection take-down or indication for a heart transplant was analysed.

Results:

Fenestration was absent in 237 patients (68.7%, Group 1), was created and closed later in 79 patients (22.9%, Group 2), and remained open in 29 patients (8.4%, Group 3). Mean survival probability until composite endpoint was 97.1 and 92.9% at 10 and 20 years, respectively. Patients with patent fenestration had worse survival (p < 0.001) as compared to both the non-fenestrated and fenestration closure groups. Despite a similar outcome, exercise capacity was lower in Group 2 than 1 (p = 0.013). In 58 patients with interventional fenestration closure, Nakata index was lower at the time of closure than pre-operatively, and both the pressure in the circuit and oxygen saturation in the aorta increased significantly (p < 0.001).

Conclusions:

Patients with persisting risk factors preventing fenestration closure are at higher risk of reaching the composite endpoint. Patients after fenestration closure have the worse functional outcome; their survival is, however, not different from the non-fenestrated group.

Type
Original Article
Copyright
© The Author(s), 2021. Published by Cambridge University Press

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