Growth Characteristics at Time of Fontan Procedure and Their Association with Long-Term Outcomes.

Abstract

Objective: To assess whether somatic growth may predict long-term success after a Fontan procedure.

Study design: The National Death Index was used to track outcomes of Fontan procedures from 1982 through 2011, using data from the Pediatric Cardiac Care Consortium, a US-based registry. Kaplan-Meier plots and Cox models were used to compare outcomes by growth characteristics, adjusting for sex, dominant ventricle, and era.

Results: Among 1461 patients (median age 3.12 years at Fontan evaluation), median z-scores indicated delayed growth: height-for-age (HAZ) -0.66, weight-for-age (WAZ) -0.81, weight-for-height (WHZ) -0.50. Systemic left ventricle (LV) patients had higher WAZ vs those with right (-0.66 vs -0.93, p<0.01). Over 21.2 years of median follow-up through 2022, 78 in-hospital and 184 post-discharge deaths occurred. High WAZ tertile was associated with decreased in-hospital mortality or takedown (aOR vs low tertile: 0.54; 95%CI: 0.31-0.95, p=0.03). Long-term survival was not universally linked to growth, but systemic LV patients with higher HAZ tertile had better 25-year survival (high 93.8% vs middle 92.2% and low 82.2%, p=0.02). High HAZ and middle WAZ tertiles were associated with lower mortality (aHR vs low tertiles: 0.42; 95%CI: 0.20-0.87, p=0.02 and 0.52; 95%CI: 0.28-0.97, p=0.04). Overweight (WHZ>2) was associated with increased mortality (aHR vs normal WHZ: 2.43; 95%CI: 1.15-5.14, p=0.02).

Conclusions: Suboptimal growth is prevalent after Fontan procedure. Higher weight correlates with improved perioperative outcomes, and taller height and balanced growth are associated with improved long-term survival, particularly in patients with a systemic LV.