Nephrology 2: CAKUT/Genetic Clinical and Basic Science
264 - Determinants of left ventricular mass (LVM) in children with autosomal recessive polycystic kidney disease (ARPKD)
Saturday, April 29, 2023
3:30 PM – 6:00 PM ET
Poster Number: 264 Publication Number: 264.251
Mathew Lin, Children's Hospital of Philadelphia, Ithaca, NY, United States; Erum A. Hartung, Erum Hartung, PHILADELPHIA, PA, United States; Jarcy Zee, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States; Jeremy S. Rubin, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States
Undergraduate Researcher Children's Hospital of Philadelphia Ithaca, New York, United States
Background: Hypertension can be severe in children with ARPKD and may precede decline in glomerular filtration rate (GFR). LV hypertrophy (LVH)is relatively common in children with ARPKD,but itsclinical determinants are under-studied. Objective: To examine associations between LVM, blood pressure (BP), and GFRin children with ARPKD. We hypothesized BP would be the primary determinant of LVM. Design/Methods: Single-center retrospective study. Eligibility: clinical ARPKD diagnosis, no history of dialysis/kidney transplant, echocardiogram (echo) data available. Data collected within 6 months of echo: mean systolic and diastolic casual BP %iles (SBPP, DBPP) and number of BP medications (#BPmeds) calculated from up to 3 clinic visits; ambulatory BP monitor (ABPM) SBP and DBP load; estimated GFR (eGFR, U25 creatinine equation). M-mode LVM was normalized to LVM-for-height Z-scores (LVM-Z) and indexed to height2.7 (LVMI, g/m2.7). LVH was defined as LVMI >95th %ile and/or LVH noted in report. We used Spearman correlation to examine relationships between BP, #BPmeds, age, eGFR, and LVM-Z; the Wilcoxon rank sum test to assess differences between patients with vs. without LVH; and linear regression to assess relationships between LVMI and LVM-Z, BP, and eGFR, with and without adjusting for age and sex. Results: We identified n=30 eligible patients with ARPKD; n=11 had ABPM data (Table 1). In the full cohort, median age was 7.2 (range 0.2-19.4) years, median eGFR was 47 mL/min/1.73m2, 93% were on BP meds, and 23% had LVH. SBPP, DBPP, and #BPmeds negatively correlated with age (ρ -0.66, P=0.0001; ρ -0.81, P< 0.0001; ρ -0.46, P=0.01 respectively). eGFR had little correlation with age (ρ -0.02, P=0.9), SBPP (ρ 0.18, P=0.4), or DBPP (ρ 0.01, P=0.9). LVM-Z also had little correlation with age (ρ -0.05, P=0.8), but children with LVH were younger than those without LVH (median age 1.8 vs. 9.1 years, P=0.02). SBPP and DBPP did not differ between children without vs. with LVH [median SBPP 78 vs. 74 (P=0.9); DBPP 67 vs. 87 (P=0.2)](Figure 1). LVMI and LVM-Z were not significantly associated with BP parameters in unadjusted regression. After adjusting for age, sex, and eGFR, there remained no significant associations between LVMI or LVM-Z and BP, except for a negative association between LVM-Z and DBPP. LVMI and LVM-Z were negatively associated with eGFR in unadjusted and adjusted analyses (Table 2).
Conclusion(s): Unexpectedly, LVM was not significantly associated with BP in this cohort of children with ARPKD. Instead, LVM was independently associated with eGFR, suggesting a primary relationship between ARPKD severity and LVH.
