Hostname: page-component-586b7cd67f-r5fsc Total loading time: 0 Render date: 2024-11-22T21:03:28.000Z Has data issue: false hasContentIssue false

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: [email protected]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Rychik, J, Atz, AM, Celermajer, DS, et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the american heart association. Circulation 2019; 140: E234E284. DOI 10.1161/CIR.0000000000000696.10.1161/CIR.0000000000000696CrossRefGoogle ScholarPubMed
Toncu, A, Radulescu, CR, Dorobantu, D, Stoica, S. Does routine fenestration improve early and late postoperative outcomes in patients undergoing fontan palliation? Interact Cardiovasc Thorac Surg 2020; 30: 773779. DOI 10.1093/icvts/ivaa002.10.1093/icvts/ivaa002CrossRefGoogle ScholarPubMed
Fisher, DJ, Geva, T, Feltes, TF, et al. Lifelong management of patients with a single functional ventricle: a protocol. Texas Hear Inst J 1995; 22: 284295.Google ScholarPubMed
Máček, M, Vávra, J, Radvanský, J. Spotřeba kyslíku při zátěži na bicyklovém ergometru. In: Fyziologie a patofyziologie tělesné zátěže. Avicenum, Prague, 1988, p. 280.Google Scholar
Kotani, Y, Chetan, D, Saedi, A, et al. Persistent fenestration may be a marker for physiologic intolerance after fontan completion. J Thorac Cardiovasc Surg 2014; 148: 25322538. DOI 10.1016/j.jtcvs.2014.06.062.10.1016/j.jtcvs.2014.06.062CrossRefGoogle ScholarPubMed
Nakano, T, Kado, H, Tatewaki, H, et al. Results of extracardiac conduit total cavopulmonary connection in 500 patients. Eur J Cardio-Thoracic Surg 2015; 48: 825832. DOI 10.1093/ejcts/ezv072.10.1093/ejcts/ezv072CrossRefGoogle ScholarPubMed
Atz, AM, Travison, TG, McCrindle, BW, et al. Late status of fontan patients with persistent surgical fenestration. J Am Coll Cardiol 2011; 57: 24372443. DOI 10.1016/j.jacc.2011.01.031.10.1016/j.jacc.2011.01.031CrossRefGoogle ScholarPubMed
Gorla, SR, Jhingoeri, NK, Chakraborty, A, et al. Incidence and factors influencing the spontaneous closure of fontan fenestration. Congenit Heart Dis 2018; 13: 776781. DOI 10.1111/chd.12652.10.1111/chd.12652CrossRefGoogle ScholarPubMed
Kansy, A, Brzezinska-Rajszys, G, Zubrzycka, M, et al. Pulmonary artery growth in univentricular physiology patients. Kardiol Pol 2013; 71: 581587. DOI 10.5603/KP.2013.0121.10.5603/KP.2013.0121CrossRefGoogle ScholarPubMed
Adachi, I, Yagihara, T, Kagisaki, K, et al. Preoperative small pulmonary artery did not affect the midterm results of fontan operation. Eur J Cardio-thoracic Surg 2007; 32: 156162. DOI 10.1016/j.ejcts.2007.03.024.10.1016/j.ejcts.2007.03.024CrossRefGoogle Scholar
Sung, HK, Kang, IS, Huh, J, Heung, JL, Yang, JH, Jun, TG. Transcatheter closure of fenestration with detachable coils after the fontan operation. J Korean Med Sci 2006; 21: 859864. DOI 10.3346/jkms.2006.21.5.859.Google Scholar
Jeong, SI, Huh, J, Lee, HJ, Yang, JH, Jun, TG, Kang, IS. Closure of conduit fenestration after extracardiac fontan procedure using amplatzer vascular plug: comparison with detachable coil. Pediatr Cardiol 2010; 31: 4449. DOI 10.1007/s00246-009-9543-x.10.1007/s00246-009-9543-xCrossRefGoogle ScholarPubMed
Chaloupecký, V, Svobodová, I, Hadačova, I, et al. Coagulation profile and liver function in 102 patients after total cavopulmonary connection at mid term follow up. Heart 2005; 91: 7379. DOI 10.1136/hrt.2003.026419.10.1136/hrt.2003.026419CrossRefGoogle ScholarPubMed
Khuong, JN, Wilson, TG, Grigg, LE, et al. Fontan-associated nephropathy: predictors and outcomes. Int J Cardiol 2020; 306: 7377. DOI 10.1016/j.ijcard.2020.01.014.10.1016/j.ijcard.2020.01.014CrossRefGoogle ScholarPubMed
Lee, D, Levin, A, Kiess, M, et al. Chronic kidney damage in the adult fontan population. Int J Cardiol 2018; 257: 6266. DOI 10.1016/j.ijcard.2017.11.118.10.1016/j.ijcard.2017.11.118CrossRefGoogle ScholarPubMed