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 z-score (HAZ) −0.66, weight-for-age z-score (WAZ) −0.81, and weight-for-height z-score (WHZ) −0.50. Systemic left ventricle (LV) patients had higher WAZ vs those with right (−0.66 vs −0.93, P < .01). Over 21.2 years of median follow-up through 2022, 78 in-hospital and 184 postdischarge 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 = .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 = .02). High HAZ and middle WAZ tertiles were associated with lower mortality (adjusted hazard ratio vs low tertiles: 0.42; 95% CI: 0.20-0.87, P = .02 and .52; 95% CI: 0.28-0.97, P = .04). Overweight (WHZ > 2) was associated with increased mortality (adjusted hazard ratio vs normal WHZ: 2.43; 95% CI: 1.15-5.14, P = .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.