Cystic burden meets blood pressure: cardiac remodelling in paediatric autosomal dominant polycystic kidney disease

Abstract Background We evaluated the prevalence and phenotype of hypertension in untreated children with autosomal dominant polycystic kidney disease (ADPKD) and examined associations with blood pressure (BP), cyst burden, and left ventricular (LV) structure and function. Methods Single-centre, cross-sectional study included children and young people (< 18 years) with ADPKD. Data regarding demographics, office BP, 24-h ambulatory BP monitoring if > 5-years, biochemistry and echocardiography were collated. Cyst burden was assessed by kidney ultrasound and classified as ‘low burden’ or ‘high burden’. Participants with hypertension (BP ≥ 95th percentile) or ‘high-normal’ BP (BP ≥ 90th and < 95th percentile) were grouped as ‘High BP’ (BP ≥ 90th percentile), and the remaining as normotensive (BP < 90th percentile). Results Amongst 116 children (mean age 9.99 ± 5.2 years; 57.8% female), hypertension prevalence was 12% ( n = 14), 8 with masked hypertension. Those with High BP (26% ( n = 30)) had a higher cyst burden (56% vs. 34%, P = 0.036), LV hypertrophy (LVH) (16.6% vs. 4.6%, P = 0.034) and relative LV wall thickness (0.34 ± 0.08 vs. 0.29 ± 0.05, P < 0.001) but similar indexed LV mass (LVMI) and LV function when compared with normotensives. In multivariable analysis, cyst burden independently predicted LVMI (standardised β = 0.23, P = 0.02), whereas systolic BP did not (standardised β = 0.19, P = 0.14). Conclusions Hypertension affected 12% of untreated children with ADPKD and preserved kidney function. High BP was associated with increased prevalence of LVH and relative wall thickness when compared to those who were normotensive. Cyst burden correlated with adverse cardiac remodelling, suggesting a BP-independent mechanism. Graphical Abstract