DO CHILDREN WITH ALPORT SYNDROME HAVE AN INCREASED RISK OF AORTIC DILATATION?

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https://storage.unitedwebnetwork.com/files/1099/fe0334708588acf7cbb95567f257d977.pdf

Abstract Title

DO CHILDREN WITH ALPORT SYNDROME HAVE AN INCREASED RISK OF AORTIC DILATATION?

First Name *

MARINA

Last Name *

AKSENOVA

Co-author 1

Konstantin Tutelman ktutelman@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Department of functional diagnostics Moscow

Co-author 2

Lilia Anikalchuk lanikalchuk@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Department of functional diagnostics Moscow

Co-author 3

Tatyana Lepaeva tlepaeva@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Nephrology department Moscow

Co-author 4

Tatyana Nikishina tnikishina@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Nephrology department Moscow

Co-author 5

Natalia Zaikova nzaikova@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Nephrology department Moscow

Co-author 6

Oxana Piruzeeva opiruzeeva@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Nephrology department Moscow

Co-author 7

Varvara Obukhova vobukhova@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Nephrology department Moscow

Co-author 8

Natalia Konkova nkonkova@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Nephrology department Moscow

Co-author 9

Vladimir Dlin vdlin@pedklin.ru Y.Veltischev Research and Clinical Institute for Pediatrics at N.Pirogov Russian National Research Nephrology department Moscow

Co-author 10

Co-author 11

Co-author 12

Co-author 13

Co-author 14

Co-author 15

Introduction

Aortic diseases have been described in patients with Alport syndrome (AS). It is believed that this is due to loss of a1a2a5a6 networks of type IV collagen in basement membrane of aortic smooth muscle cells. Aortic morphology has not been evaluated in children with AS. The aim was to define prevalence and risk factors for aortic dilatation in children with AS.

Methods

Retrospective cross-section single center study included children with AS (1st group) and CACUT (2nd, comparison group). Echocardiograms were evaluated for the presence of aorta dilatation (Z score ≥ 1,65 (≥ 99th %) from the mean) at the Sinuses of Valsalva (SoV). Clinical (body mass index (BMI), Z-score of mean systolic blood pressure (zSBP), mean diastolic blood pressure (zDBP), mean blood pressure (zMBP)), laboratory (proteinuria (Pr, mg/m2/day, eGFR (ml/min/1.73m2), echocardiograms (left ventricular mass index (LVMI, z-score), left ventricular end diastolic diameter (LVEDD, z-score), relative wall thickness of the left ventricle (RWT, N<0,42) data were obtained.

Results

The study included 138 pts with confirmed AS (age 10.2±4.3 yrs, 96M) and 27 children with CACUT (age 11.8±3.9 yrs, 12M). SoV dilatation was revealed in 16 children with AS (including 1 pts with heterozygous COL4A3 variant, 3 pts with homozygous/compound heterozygous COL4A3 variants and 12 pts with X-linked AS; 0.17 vs 0.38 vs 0.1, respectively in AS subgroups, p>0.05) and in 4 pts with CACUT (q1=0,12 vs q2=0.15, p>0.05). In multivariable regression analysis SoV dilatation was associated with male gender (β1=0.31, p=0.02; β2=0.34, p=0.02) and LVMI (β1=0.26, p=0.02; β2=0.24, p=0.02) in both groups, and with BMI (β1= -0.27, p=0.03), Pr (β1=0.23, p=0.04) and LVEDD (β1=0.21, p = 0.04) in children with AS.

Conclusions

SoV dilatation was revealed in about 10% of children with CKD (regardless of its causes) and was associated with male gender and left ventricular remodeling. Children with proteinuric stage of AS have a risk for SoV dilatation. More detailed epidemiological studies are needed to establish associations between AS and aortic diseases.

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