Author + information
- Sheetal Patela,b,
- David Kwiatkowskia,b,
- Adin-Cristian Andreia,b,
- Ankita Devareddya,b,
- Catherine Krawczeskia,b,
- Barbara J. Deala,b,
- Craig Langmana,b and
- Bradley Marinoa,b
Background: Single ventricle congenital heart disease patients s/p Fontan operation are at risk for chronic kidney disease (CKD). We sought to estimate the prevalence of and risk factors for CKD in Fontan survivors.
Methods: This was a two-center cross sectional study of Fontan survivors between 01/08 and 07/16. The primary outcome was presence of CKD defined by estimated Glomerular Filtration Rate (eGFR) of <90ml/min/1.73m2 derived using serum creatinine based age appropriate eGFR equations. Group comparisons used Chi-square and t-tests. Multivariable models to predict eGFR were created using stepwise variable selection and aggregation across bootstrap samples.
Results: 404 Fontan survivors were included: male 61%; age 13.3 yrs (2.3 – 49.9); time from Fontan 10 yrs (0.02- 36.8); dominant ventricle 44% left; Fontan type [71% external conduit (EC), 14% lateral tunnel (LT), 15% atrio-pulmonary; 8% of EC and 68% of LT had fenestration]. CKD was present in 44% (176/404) of patients (pts). Older age correlated with lower eGFR in both pediatric (n=280, r=-0.23; p<0.001) and adult (n=124, r=-0.35; p<0.001) pts. Patients with CKD had higher single ventricular end diastolic pressure (7±3 vs. 6+2 mmHg; p=0.007), higher SVC mean pressure (11+3 vs. 9+3 mmHg; p=0.002), lower SVC oxygen saturation (70±8 vs. 72+6; p=0.05) and higher indexed pulmonary vascular resistance (1.5+0.7 vs. 1.3+0.5 Woods units; p=0.048) at time of pre-Fontan cath. Pts with CKD were more likely to have EC Fontan (p=0.005), less likely to have fenestration (p=0.012), more likely to have ascites (p=0.014) and require Sildenafil therapy (p=0.041). Multivariate regression revealed that lower eGFR is associated with older age (-1.2 per yr; p<0.001) and sildenafil use (-9; p=0.004) in pediatric pts; and with longer duration since Fontan (-1.9 per yr; p<0.001) and EC Fontan type (-11.3; p<0.001) in adult pts.
Conclusions: By 10 yrs after the Fontan; almost half of the survivors develop CKD. Increasing age is associated with progressively lower eGFR. Worse pre-Fontan hemodynamic factors, Fontan type and absence of fenestration are associated with the development of CKD. Our data suggest a need for more intense screening for CKD in Fontan survivors.
Moderated Poster Contributions
Congenital Heart Disease and Pulmonary Hypertension Moderated Poster Theater, Poster Hall, Hall C
Sunday, March 19, 2017, 1:30 p.m.-1:40 p.m.
Session Title: Finding Out What Works in Pediatric and Congenital Heart Disease
Abstract Category: 11. Congenital Heart Disease: Therapy
Presentation Number: 1312M-11
- 2017 American College of Cardiology Foundation