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Progression in Fontan conduit stenosis and hemodynamic impact during childhood and adolescence

Published:October 28, 2020DOI:https://doi.org/10.1016/j.jtcvs.2020.09.140

      Abstract

      Objective

      To characterize changes in Fontan conduit size over time and determine if cross-sectional area (CSA) affects cardiac output, pulmonary artery growth, and exercise capacity.

      Methods

      We conducted a retrospective cross-sectional study of patients with Fontan physiology who underwent cardiac magnetic resonance imaging or cardiac catheterization between January 2013 and October 2019. We collected Fontan and pulmonary artery measurements, hemodynamic data, and cardiopulmonary exercise test data. We identified 158 patients with an extracardiac Fontan. We measured minimum and mean Fontan conduit CSA and assessed whether these correlated with Nakata index, cardiac index, or exercise capacity.

      Results

      Minimum Fontan CSA decreased by a median of 33% (24%, 40%) during a mean follow-up of 9.6 years. Median percentage decrease in Fontan CSA did not differ among 16-, 18-, and 20-mm conduits (P = .29). There was a significant decrease in the minimum Fontan CSA (33% [25%, 41%]) starting less than 1-year post-Fontan. Median Nakata index was 177.6 mm2/m2 (149.1, 210.8) and was not associated with Fontan CSA/BSA (ρ = 0.09, P = .29). Fontan CSA/BSA was not associated with cardiac index (ρ = –0.003, P = .97). A larger Fontan CSA/BSA had a modest correlation with % predicted oxygen consumption (ρ = 0.31, P = .013).

      Conclusions

      Fontan conduit CSA decreases as early as 6 months post-Fontan. The minimum Fontan CSA/BSA was not associated with cardiac index or pulmonary artery size but did correlate with % predicted peak oxygen consumption.

      Graphical abstract

      Key Words

      Abbreviations:

      BSA (body surface area), CHLA (Children's Hospital Los Angeles), CPET (cardiopulmonary exercise test), CSA (cross-sectional area), IVC (inferior vena cava), LPA (left pulmonary artery), MRI (magnetic resonance imaging), Qp:Qs (ratio of pulmonary blood flow to systemic blood flow), RPA (right pulmonary artery), SVC (superior vena cava), VO2 (oxygen consumption)
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      Linked Article

      • Commentary: You should occasionally look at the results!!
        The Journal of Thoracic and Cardiovascular SurgeryVol. 162Issue 2
        • Preview
          How often are we delusional, even in our most simple beliefs? We have for long implanted 18-mm or 20-mm Gore-Tex conduits (W. L. Gore & Associates, Inc, Flagstaff, Ariz) as extracardiac Fontan conduits because we believed that this was the size of an adult inferior vena cava and we wanted to have the most streamlined venous. Patel and colleagues1 report in this issue of the Journal a detailed analysis of Fontan circuit sizes by both magnetic resonance imaging and catheterization in 156 patients over a mean follow-up period of 10 years.
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      • Commentary: As we learn more, we know little
        The Journal of Thoracic and Cardiovascular SurgeryVol. 162Issue 2
        • Preview
          The Fontan operation is the goal for patients with a single ventricle, and the procedure has undergone various modifications over the years. Originally introduced in 1990,1 extracardiac conduit Fontan possesses some advantages such as improved flow dynamics, lower arrhythmias, and technically less challenging. Nonetheless, lack of growth potential and thrombogenecity are important drawbacks and lead to reinterventions. Patel and colleagues2 have provided us with an important study looking at the intermediate outcome of the extracardiac Fontan.
        • Full-Text
        • PDF
      • Commentary: The heart of the matter: Close clinical follow-up and exercise capacity in Fontan circulation
        The Journal of Thoracic and Cardiovascular SurgeryVol. 162Issue 2
        • Preview
          In this issue of the Journal, Patel and colleagues1 evaluate the cross-sectional area (CSA) of extracardiac Fontan conduits and associated hemodynamic impact at various times during follow-up. The authors are to be commended for their compilation of a large number of patients with Fontan circulation (FC) with magnetic resonance imaging, catheter, and cardiopulmonary exercise testing (CPET) data. Their analysis revealed a median CSA decline of 68% compared with the original CSA of the conduit implanted.
        • Full-Text
        • PDF