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Atrioventricular septal defect in Fontan circulation: Right ventricular dominance, not valve surgery, adversely affects survival

      Abstract

      Objective

      The effect of ventricular dominance and previous atrioventricular valve (AVV) surgery on patient outcomes after Fontan operation remains unclear. We sought to determine the effect of ventricular dominance and previous AVV surgery on transplantation-free survival and long-term AVV competency in patients with atrioventricular septal defect (AVSD) and Fontan circulation.

      Methods

      We conducted a retrospective study of 1703 patients in the Australia and New Zealand Fontan Registry, who survived Fontan operation between 1987 and 2021.

      Results

      Of 174 patients with AVSD, 60% (105/174) had right ventricular (RV) dominance and 40% (69/174) had left ventricular (LV) dominance. The cumulative incidence of moderate or greater AVV regurgitation at 25 years after Fontan operation in patients with LV dominance was 56% (95% CI, 35%-72%), compared with 54% (95% CI, 40%-67%) in patients with RV dominance (P = .6). Nonetheless, transplantation-free survival at 25 years in patients with LV dominance was 94% (95% CI, 86%-100%), compared with 67% (95% CI, 52%-87%) in patients with RV dominance (hazard ratio, 5.9; 95% CI, 1.4-25.4; P < .01). Of note, transplantation-free survival was not different in patients who underwent AVV surgery before or at Fontan completion compared with those who did not (15 years: 81% [95% CI, 62%-100%] vs 88% [95% CI, 81%-95%]; P = .3).

      Conclusions

      In patients with AVSD and Fontan circulation the rate of moderate or greater common AVV regurgitation is similar in those with LV and RV dominance. RV dominance, rather than previous AVV surgery, is a risk factor for death or transplantation.

      Graphical abstract

      Key Words

      Abbreviations and Acronyms:

      AVSD (atrioventricular septal defect), AVV (atrioventricular valve), HR (hazard ratio), LV (left ventricular), RV (right ventricular)
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      Linked Article

      • Commentary: The hit parade
        The Journal of Thoracic and Cardiovascular SurgeryVol. 165Issue 2
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          In the Venn diagram overlap of characteristics for single-ventricle candidacy, those with both unbalanced atrioventricular (AV) septal defects and significant AV valve regurgitation are among the greatest risk for palliation failure. In this issue of the Journal, King and colleagues1 present an analysis of the Australia and New Zealand Fontan Registry that attempts to tease apart the contributions of ventricular dominance (right ventricle [RV] vs left) and AV-valve surgery in this population to better profile those at greatest risk for death or transplantation.
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