Failures of the Fontan System in Univentricular Hearts and Mortality Risk in Heart Transplantation: A Systematic Review and Meta-Analysis

The Fontan procedure (FP) is the standard surgical treatment for Univentricular heart diseases. Over time, the Fontan system fails, leading to pathologies such as protein-losing enteropathy (PLE), plastic bronchitis (PB), and heart failure (HF). FP should be considered as a transitional step to the final treatment: heart transplantation (HT). This systematic review and meta-analysis aims to establish the risk of death following HT according to the presence of FP complications. There was a total of 691 transplanted patients in the 18 articles, immediate survival 88% (n = 448), survival from 1 to 5 years of 78% (n = 427) and survival from 5.1 to 10 years of 69% (n = 208), >10 years 61% (n = 109). The relative risk (RR) was 1.12 for PLE (95% confidence interval [CI] = 0.89-1.40, p = 0.34), 1.03 for HF (0.7-1.51, p = 0.88), 0.70 for Arrhythmias (0.39-1.24, p = 0.22), 0.46 for PB (0.08-2.72, p = 0.39), and 5.81 for CKD (1.70-19.88, p = 0.005). In patients with two or more failures, the RR was 1.94 (0.99-3.81, p = 0.05). After FP, the risk of death after HT is associated with CKD and with the presence of two or more failures.

Automated summary

The study shows that the major complications of FP have minimal impact on the risk of death after HT, but the presence of chronic kidney disease (CKD) and two or more failures significantly increase the risk of mortality.