XXXX late after Fontan operation are related to increase in mortality and hospitalization.

@entity1 @entity4710 @entity943 @entity204
BACKGROUND: @entity4710 are a known complication late after Fontan operation. Limited information is available on their prognostic value. METHODS: All @entity1 with previous Fontan operation followed at our institution since 1999 were identified from the electronic database and included in this study. Demographic and clinical characteristics including history of @entity4710 were recorded at the earliest full clinical assessment and @entity1 were followed thereafter for all-cause mortality and hospitalization. RESULTS: A total of 98 @entity1 , mean age 31.5    8.9 years, 43.8% male, 31.6% with a total cavopulmonary connection (TCPC) were identified. A history of @entity4710 was present at baseline in 60.2% of @entity1 who were older (33.0    8.3 vs 29.1    9.4 years, p=0.002), less likely to have a TCPC (13.5% vs 58.9%, p<0.001), and more symptomatic in terms of NYHA class (51.9% vs 26.7%, p=0.007) compared to @entity943 -free @entity1 . During a median follow-up of 6.7 years 18 @entity1 died and 64 @entity1 were hospitalized. Even after adjustment for baseline clinical characteristics, @entity4710 was an independent predictor of @entity204 (propensity score adjusted HR 9.35, 95% CI: 1.10-79.18, p=0.04, c-statistic 0.88) and composite of @entity204 or hospitalization (propensity score adjusted HR 5.00, 95% CI: 2.47-10.09, p<0.0001). CONCLUSIONS: In adult @entity1 with a Fontan-type operation, the presence of @entity4710 is associated with higher morbidity and mortality at mid-term follow-up. Whether early @entity943 targeting intervention may improve clinical outcome needs to be studied in a prospective manner.
