Resolving the Fontan paradox: Addressing socioeconomic and racial disparities in patients with a single ventricle

Abstract

for the members of the Southwest Congenital Cardiac Consortium Feature Editor's Note-The Fontan operation, in many respects, is the signature operation for congenital heart surgeons. At our Heart Center, palliative operations leading up to the Fontan procedure, the Fontan procedure itself, and post-Fontan surgical interventions account for 20% of our congenital cardiac surgery case volume. Over the past 45 years, the Fontan operation has evolved steadily with numerous technical modifications as surgeons have sought to improve patient outcomes. The resources and attention devoted by heart centers to the totality of care of Fontan patients is enormous. In a recent issue of The Journal of the American College of Cardiology, Atz and colleagues from the Pediatric Heart Network Investigators reported on nearly 500 patients undergoing a thorough health assessment 9 years after their previous landmark Fontan crosssectional study. The authors noted a concerning finding in these patients: Exercise performance decreased over the years and this was associated with worse functional health status. We are fortunate to present an invited expert opinion summarizing and augmenting this article by Yves d'Udekem. d'Udekem is a passionate advocate for Fontan patients. He is the founder and chair of the Australian and New Zealand Fontan Registry. He estimates that there are currently 70,000 patients with a Fontan circulation. d'Udekem has reviewed the findings of Atz and colleagues and added to this his own extensive experience with the very robust Australian and New Zealand Fontan Registry. That comprehensive database goes back 35 years, and they have found that a high level of physical activity and exercise training optimizes outcomes after the Fontan procedure. This analysis will further advance steps we can take to improve the lives of this complex cohort of patients.

Non-invasive biomarkers of Fontan-associated liver disease

“…Relationships between obesity, race, and ethnicity and poor long-term outcomes in the Fontan population have been reported (41,42). Prior studies have not investigated hepatic steatosis in the context of FALD, though one reported it on histology (5).…”

Biventricular Conversion in Unseptatable Hearts: “Ventricular Switch”

“…Although contemporary reports of Fontan palliation have demonstrated re-assuring long-term survival in selected groups of patients, 10 it is clear that specific subgroups fare worse with this approach. Atz et al 11 detailed the sobering long-term results among Fontan patients in Fontan 3 À the most recent installment of the PHN cross-sectional study. Despite overall good survival (90% at 9.4 years), there was a steady attrition in transplant-free survival.…”

Access or excess? Examining the argument for regionalized cardiac care

“…Comparably worse outcomes among African Americans and those in lower income quartiles are consistently reported following both congenital and acquired cardiac surgery. 29,30 Benavidez and colleagues 31 investigated race/ethnic disparities in outcomes following congenital heart surgery using the Kids Inpatient Database 2000. In this study, in-patient mortality was significantly increased in the black population compared with white ethnicity and regional geographic differences in racial/ethnic outcomes were apparent.…”

“…In this study, in-patient mortality was significantly increased in the black population compared with white ethnicity and regional geographic differences in racial/ethnic outcomes were apparent. In the Benavidez study, socioeconomic status was not associated with increased risk of death, but as Karamlou and colleagues 29 discussed, studies of sociodemographic influence are often superficial (usually as a result of nongranular variable capture in most available datasets) and incompletely assess interactions among highly collinear or modifying factors. Building on this contention, we recently used the Pediatric Health Information Systems database to explore whether inflection points could be determined whereby race/ethnicity could be mitigated by positive modifiers, including discrete income level or more evolved programmatic/process factors on the one hand, or exacerbated by negative modifiers, including genetic abnormalities or center experience.…”