Aortic Dilatation in Children and Young People With ADPKD

Abstract

Introduction: Aortic root dilatation is a reported cardiovascular sequela seen in children and young people (CYP) with chronic kidney disease (CKD) but has yet to be described in those with autosomal dominant polycystic kidney disease (ADPKD).

Methods: Single center, cross-sectional study in a dedicated ADPKD clinic. Echocardiograms were evaluated for the presence of dilatation (defined by a z-score ≥2 [≥99th percentile] SDs from the mean) at 4 standardized locations, namely the aortic valve annulus, sinuses of Valsalva (SoV), sinotubular junction (STJ), and the ascending aorta. Measurements were compared with a control group to assess prevalence, severity, and determinants of aortic dilatation.

Results: Ninety-seven children, median age (interquartile range) of 9.3 (6.1, 13.6) years were compared with 19 controls without ADPKD or other CKD. The prevalence of dilatation ranged from 5.2% to 17% in ADPKD, depending on anatomical location with no aortic dilatation identified in the control group. In multivariable regression, aortic root dilatation was significantly associated with cyst burden at the aortic valve annulus and SoV (β = 0.42 and β = 0.39, both P < 0.001), with age at SoV (β = -0.26, P = 0.02), systolic blood pressure (SBP) z-score at SoV (β = -0.20, P = 0.04) and left ventricular mass index (LVMI) at SoV and STJ (β = 0.24, P = 0.02 and β = 0.25, P = 0.03, respectively) following adjustment for age, sex (male or female), body mass index (BMI) z-score, estimated glomerular filtration rate (eGFR), SBP z-score, and LVMI.

Conclusion: Our data suggests increased prevalence of aortic root and ascending aortic dilatation in CYP with ADPKD compared with controls. Further studies are needed to understand the pathogenesis and its contribution to the high cardiovascular morbidity in ADPKD.

Keywords: ADPKD; aortic root; children; dilatation; echocardiography.

Graphical abstract

Figure 1

Parasternal long-axis echocardiographic image showing the aortic measurements. Ao, aorta; LA, left atrium; LV, left ventricle; MV, mitral valve; RV, right ventricle; SoV, sinuses of Valsalva; STJ, sinotubular junction The aortic root and ascending is measured at 4 standardized places, inner-edge to inner-edge technique in end-systole as indicated by the arrows: the aortic valve annulus (1); SoV (2), STJ (3) and the ascending aorta at its widest diameter, at least 2cm above the STJ (4).

Figure 2

Distribution of aortic z-scores in 97 children and young people with ADPKD and healthy controls without ADPKD. ADPKD, autosomal dominant polycystic kidney disease. Aortic dilatation was defined as z-score ≥2 (≥99^th percentile) using the Lopez Pediatric Heart Network regression equations.

Figure 3

Aortic diameters in mm and as z-scores in 97 children and young people with ADPKD stratified by cyst burden and compared with healthy controls. ADPKD, autosomal dominant polycystic kidney disease; AoV, aortic valve annulus; AsAo, ascending aorta; SBP, systolic blood pressure; SoV, sinuses of Valsalva; STJ, sinotubular junction Data shown as marginal means after adjustment for age, sex (male or female), and SBP z-score. Error bars represent 95% confidence interval. Panel A: aortic diameters in mm; Panel B aortic diameter z-scores. P-values refer to difference between groups assessed by analysis of variance.