Cited 2 times since 2019 (3.4 per year) source: Scopus Interactive cardiovascular and thoracic surgery, Volume 29, Issue 3, 01 September 2019, Pages 461-468 A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome. Rijnberg FM, Blom NA, Sojak V, Bruggemans EF, Kuipers IM, Rammeloo LAJ, Jongbloed MRM, Bouma BJ, Hazekamp MG

Objectives

This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity.

Methods

Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016.

Results

The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1-Q3 7.6-17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18-44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63-96%) at 20 years for lateral tunnel and 99% (95% CI 96-100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III-IV) at 15 years was 65% (95% CI 52-76%) for atriopulmonary connection/Björk, 90% (95% CI 73-97%) for lateral tunnel and 90% (95% CI 82-94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2-5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4-9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4-9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0-11.1; P < 0.001).

Conclusions

With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.

Keywords: Arrhythmia, Protein Losing Enteropathy, Long-term Outcome, Tachyarrhythmia, Single Ventricle, Fontan, Fontan Failure

Interact Cardiovasc Thorac Surg. 2019 Sep;29(3):461-468