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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: [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

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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