Elsevier

The American Journal of Cardiology

Volume 137, 15 December 2020, Pages 103-110
The American Journal of Cardiology

Effect of Earlier Atrioventricular Valve Intervention on Survival After the Fontan Operation

https://doi.org/10.1016/j.amjcard.2020.09.028Get rights and content

Whereas the prevalence and impact of atrioventricular valve (AVV) regurgitation in patients with single ventricle physiology has become increasingly apparent, the optimal timing for valve intervention is unclear. To investigate this, we performed a retrospective review of all 1,167 patients from the Mayo Clinic Fontan database. Thirteen percent (153 patients) had AVV repair or replacement during their staged single ventricle palliation. We found that patients with right ventricular morphology and common AVV were at increased risk for AVV intervention. Patients who underwent AVV intervention had increased risk of death/transplant compared with those who did not (hazards ratio [HR] = 1.75, 95% CI 1.37 to 2.23, p <0.001). With respect to valve intervention timing, whereas AVV intervention before Fontan presented similar risk for death/transplant compared with no AVV intervention (HR = 0.85, 95% CI 0.32 to 2.27, p = 0.74), intervention at time of Fontan had a significantly higher risk (HR = 1.46, 95% CI 1.09 to 1.97, p = 0.01), and intervention after Fontan had a much more substantial risk (HR = 3.83, 95% CI 2.54 to 5.79, p <0.001). AVV repair failure occurred in 11% of patients. In terms of relative risk of valve repair versus replacement, in post-Fontan AVV intervention patients, AVV replacement carried a 2.9 fold risk of death/transplant compared with AVV repair. In conclusion, AVV disease remains a considerable challenge for durable Fontan physiology. This data demonstrates that earlier intervention on valve pathology improves survival with the Fontan circulation. Continued surveillance of single ventricle patients and prompt referral of those with valve pathology can improve outcomes in this challenging population.

Section snippets

Methods

A retrospective review was performed of all patients in the Mayo Clinic Fontan database from January 1973 to February 2019. Permission was granted by the Institutional Review Board to perform this study. Data gathered included standard demographics, underlying anatomy, operative details, timing of first AVV intervention relative to Fontan, as well as long-term follow-up. Fontan connection type was based on the first Fontan operation in cases when patients underwent multiple Fontan revisions.

Results

Of the 1,167 patients in the database, 153 (13%) required AVV intervention throughout the stages of single ventricle palliation. Compared with patients who did not receive AVV intervention, those having intervention were more likely to have heterotaxy and unbalanced atrioventricular septal defect, but less likely to have double inlet left ventricle (Table 1). Those requiring AVV intervention were more likely to have a dominant right ventricle, a common AVV (CAVV), and less likely to have a

Discussion

The present study expands our knowledge of AVV disease in Fontan patients by examining the impact of timing of AVV intervention on outcomes. Similar to previous studies, we demonstrated a substantially increased risk of transplant and death among patients who undergo AVV intervention,2,5 with patients having a dominant right ventricle and CAVV at greatest risk for requiring AVV intervention. Importantly, whereas patients who had their first AVV intervention before Fontan were not at increased

Credit Author Statement

Stephens: Conceptualization, Methodology, Investigation, Formal Analysis, Writing (original draft, Review/editing).

Dearani: Conceptualization, Writing (Review/Editing).

Niaz: Conceptualization, Investigation.

Arghami: Methodology, Formal analysis.

Phillips: Investigation, Writing (Review/Editing).

Cetta: Conceptualization, Methodology, Investigation, Writing (original draft, Review/editing).

Declaration of Interests

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

References (18)

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    Although the current study suggests that earlier Fontan completion is associated with improved neurodevelopmental outcomes, further studies are required to confirm these findings and elucidate underlying mechanistic pathways. Previous work has demonstrated that AVV intervention at the time of Fontan completion is associated with increased morbidity and mortality in the post-Fontan period.31-33 To this end, there has been a shift in practice by some to preferentially address the AVV before completion of Fontan.31-34

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    As such, maintaining competency of the AVV is crucial, particularly in patients with RV dominance.17 AVV operation before or at Fontan completion has previously been identified as a risk factor for death or transplantation after Fontan operation.5-8 However, it is important to note that a disproportionate number of patients with RV dominance undergo AVV operation compared with patients with LV dominance.

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Funding: None.

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