Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle.

25. PMID: 34706905


Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle.


Anigwe C, Yogeswaran V, Moon-Grady A, McAllister S, Aggarwal A, Blissett S, Harris IS, Kouretas PC, Mahadevan VS, Sabanayagam A, Agarwal A.Heart. 2022 Jul 13;108(15):1209-1215. doi: 10.1136/heartjnl-2021-319760.PMID: 34706905


Take Home points:

  • Adverse outcomes seen in 54.4% of TCPC patients with up to 34 year follow-up post palliation
  • The most frequent adverse events were arrhythmias, pacemaker/ICD implantation, and thromboembolic events
  • Higher METS and peak heart rate at exercise was associated with fewer adverse events.
  • Adverse outcome-free survival rates were 89% at 1o year, 60% at 20 years and 24% at 25 years post-Fontan palliation.
  • ECC Fontan was an independent predictor of adverse events after adjusting for other risk factors.


Pediatric Cardiac Professionals

Commentary from Dr. Blanche Cupido (Cape Town, South Africa), chief section editor of ACHD Journal Watch:


Total cavopulmonary circulation (TCPC) has become the preferred surgery for single ventricle palliation. Robust data on the prevalence and predictors of long terms outcomes is lacking. The existing data is limited by predominantly looking at the paediatric population, focusing on peri-operative and short term outcomes, with little info in the adult population. Furthermore, most studies are heterogenous, in that the TCPC population is not studied in isolation, not is morbidity assessed well (older studies focused predominantly on mortality outcomes).


This single center retrospective study aimed to assess the prevalence and factors associated with long term outcomes in adults who had undergone the TCPC procedure between 1 January 2000 and 1 July 2019. Adverse outcomes assessed included: arrhythmia, pacemaker or ICD placement, liver cirrhosis, protein losing enteropathy (PLE), heart failure hospitalization or thromboembolic complications or death.


The analysis included 160 patients, of which 73.1% had an extra-cardiac conduit (ECC). The underlying pathologies included: tricuspid atresia (27.5%), hypoplastic left heart syndrome (25%), double-inlet LV (19.4%), double-outlet RV (10%), PA-IVS (5.62%), Ebstein’s anomaly (3.12%), hypoplastic right heart syndrome (1.88%), TGA (1.25%), pulmonary atresia (0.62%), heterotaxy (5.62%).


The TCPC’s were done between July 1985 and December 2011. Median age at TCPC was 4.7 years and the median duration to last follow-up was 17.5 years. Apart from  lower METs and peak heart rate, there were no significant differences in the cardiac diagnostic data between the group that experienced adverse events and those who did not.


Adverse outcomes were observed in 87 patients. Patients with adverse events were older, had a longer time since the TCPC operation and had a fenestration (Table 1).



The most frequently observed complications were arrhythmias (37.5%), pacemaker/ICD implantation (21.3%), and thrombo-embolic events (14.4%) (Figure 2)



The median adverse outcome-free survival was 22.9 years after TCPC procedure.


Adverse outcome-free survival was 89% at 10 years, 60% at 20 years and 24% at 25 years post TCPC palliation surgery. Unadjusted analysis showed non-white race and extra-cardiac conduit (ECC) to have worse outcome-free survival. Type of ventricle or presence of a fenestration was not a predictor of poor outcome. (Figure 3)



When adjusted for age, race, type of Fontan, systemic ventricle type and fenestration, ECC Fontan was independently associated with a greater risk for adverse outcome. This held true for the composite of all adverse outcomes, arrhythmias, cirrhosis, pacemaker/ICD implantation, and other. (Figure 4)