Abstract 18607: Prevalence, Outcome, and Predictors of Cardio-Renal Syndrome in Children with Dilated Cardiomyopathy

Abstract

Background: Cardio-renal syndrome (CRS) describes variety of acute or chronic conditions where the primary failing organ can be either the heart or kidney. The prevalence, predictors and outcome of CRS has not been reported in children with dilated cardiomyopathy (DCM). Methodology: Data from patients > 1 year of age with DCM enrolled in the Pediatric Cardiomyopathy Registry (PCMR) was assessed. CRS was defined as glomerular filtration rate <90 ml/min/1.73m 2 estimated by modified Schwartz formula (eGFR). Patients with eGFR>150 ml/min/1.73m 2 were excluded. CRS prevalence was determined by cross-sectional design in patients newly diagnosed with DCM using the baseline PCMR data. Survival analysis compared patients with vs. without CRS using annual PCMR data. Regression models were applied on both baseline and annual data to study the interactions between eGFR and different echocardiographic measures. Results: The baseline data had 484 children with DCM. Serum Cr data to calculate eGFR were available for 92 patients. Fifty six (62.0%) patients had eGFR < 90 ml/min/1.73m 2 , which is greater than the 48% prevalence published in 3256 healthy historical controls (P<0.001). CRS patients had a mean eGFR of 61 (±22.6) vs. 108 (±14) (P<0.001). Death occurred in 8 (14%) of CRS patients compared to no deaths in patients without CRS (P=0.052) after 7 years follow-up. Univariate regression models showed a significant positive correlation between fractional shortening (FS) and eGFR (P= 0.0013, r 2 = 0.09). No correlation was observed between eGFR and ejection fraction (EF), left ventricular end-diastolic dimension (LVEDD) and LVEDD Z-scores. Conclusion: This is the first study to show that CRS is common in children newly diagnosed with DCM and is associated with higher risk of subsequent death. Worsening FS was associated with decreasing eGFR which suggests that patients with poor FS might have significant CRS. We suggest kidney functions should be evaluated systematically in children with DCM to assess for chronic kidney disease and direct potential management.