Does leaving native antegrade pulmonary blood flow at the time of the superior cavopulmonary connection impact long-term outcomes after the Fontan?

Hannah Davidson; Diana Zannino; Yves d'Udekem; Rachael Cordina; Yishay Orr; Igor E Konstantinov; Robert Weintraub; Gavin Wheaton; Jelena Saundankar; Gananjay Salve; Ajay Iyengar; Nelson Alphonso; Julian Ayer

doi:10.1016/j.xjon.2023.09.009

Does leaving native antegrade pulmonary blood flow at the time of the superior cavopulmonary connection impact long-term outcomes after the Fontan?

JTCVS Open. 2023 Sep 19:16:825-835. doi: 10.1016/j.xjon.2023.09.009. eCollection 2023 Dec.

Authors

Hannah Davidson^{1

2}, Diana Zannino³, Yves d'Udekem⁴, Rachael Cordina^{5

6}, Yishay Orr^{1

2}, Igor E Konstantinov^{3

7}, Robert Weintraub^{3

8

9}, Gavin Wheaton¹⁰, Jelena Saundankar¹¹, Gananjay Salve¹, Ajay Iyengar¹², Nelson Alphonso¹³, Julian Ayer^{1

2}

Affiliations

¹ The Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia.
² Discipline of Paediatrics and Child Health, The University of Sydney, Sydney, Australia.
³ Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia.
⁴ Division of Cardiac Surgery, Children's National Hospital, Washington, DC.
⁵ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
⁶ Sydney Medical School, The University of Sydney, Sydney, Australia.
⁷ Department of Cardiac Surgery, Royal Children's Hospital Melbourne, Parkville, Australia.
⁸ Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Australia.
⁹ Department of Paediatric Cardiology, Royal Children's Hospital Melbourne, Parkville, Australia.
¹⁰ Women's and Children's Hospital, Adelaide, Australia.
¹¹ Department of Paediatric Cardiology, Perth Children's Hospital, Perth, Australia.
¹² Greenlane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand.
¹³ Department of Cardiac Surgery, Queensland Children's Hospital, Brisbane, Australia.

Abstract

Objectives: Antegrade pulmonary blood flow (APBF) may be left or eliminated at the time of the superior cavopulmonary connection (SCPC). Our aim was to assess the impact of leaving native APBF at the SCPC on long-term Fontan outcomes.

Methods: In the Australia and New Zealand Fontan Registry (1985-2021), 587 patients had pre-existing native APBF at the SCPC. At the SCPC, 302 patients had APBF eliminated (APBF^-) and 285 patients had APBF maintained (APBF⁺). The incidence of Fontan failure (composite end point of Fontan takedown, transplant, plastic bronchitis, protein losing enteropathy and death) and atrioventricular (AV) valve repair/replacement post SCPC was compared between the 2 groups.

Results: Sex, predominant-ventricle morphology, isomerism, primary diagnosis, and age/type of Fontan were similar between groups. APBF^- versus APBF⁺ had a higher incidence of arch obstruction/coarctation (17% vs 7%) and previous pulmonary artery band (54% vs 45%) and a lower rate of Fontan fenestration (27% vs 41%). The risk of Fontan failure was similar between the 2 groups (hazard ratio [HR], 1.01; 95% confidence interval [CI], 0.58-1.78; P = .96). The risk of AV-valve repair/replacement was greater in APBF⁺ versus APBF- (HR, 2.32; CI, 1.13-4.75; P = .022). The risk of AV-valve repair/replacement remained after adjustment for arch obstruction/coarctation, previous pulmonary artery band and Fontan fenestration (HR, 2.27; CI, 1.07-4.81; P = .033).

Conclusions: Maintaining APBF at the time of the SCPC does not impact the risk of Fontan failure but may increase the incidence of AV-valve repair and/or replacement post-SCPC.

Keywords: Fontan; antegrade pulmonary blood flow; atrioventricular valve regurgitation; superior cavopulmonary connection.