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. 2025 Oct 7.
doi: 10.1007/s40620-025-02426-y. Online ahead of print.

Determinants of left ventricular mass in children with autosomal recessive polycystic kidney disease

Mathew Lin  1 , Jeremy Rubin  2 , Robert A Palermo  3   4 , Jarcy Zee  2   5 , Erum A Hartung  6   7
Affiliations

Determinants of left ventricular mass in children with autosomal recessive polycystic kidney disease

Mathew Lin et al. J Nephrol. .

Abstract

Background: Hypertension and left ventricular (LV) hypertrophy (LVH) are common in autosomal recessive polycystic kidney disease (ARPKD). We examined clinical determinants of LV mass in children with ARPKD.

Methods: Retrospective study of patients with ARPKD with available echocardiogram data. Casual blood pressure (BP) percentiles, 24-h ambulatory BP monitor (ABPM) parameters, antihypertensive medications, and estimated glomerular filtration rate (eGFR) within 6 months of echocardiogram were collected. Outcomes included LV mass Z-score, LV mass index [LVMI in g/m2.7 and g/(m2.16 + 0.09)], and LVH.

Results: Thirty patients with ARPKD (median age 7.2 years [IQR 3.4, 12.8]) had echocardiograms, 28 had casual BPs, 11 had ABPMs, and 93% were on antihypertensives. LVH occurred in 23% based on LVMI in g/m2.7 > 95th percentile, and in 50% based on LVMI > 45 g/(m2.16 + 0.09). Younger age correlated with higher number of antihypertensives (ρ = - 0.46, P = 0.014) and higher casual systolic and diastolic BP percentiles (r = - 0.74, P < 0.001; r = - 0.81, P < 0.001). After adjusting for age, sex, and eGFR, LV mass was not significantly associated with casual BP or ABPM, except for a negative association between LV mass Z-score and casual diastolic BP percentile (β coefficient - 0.31, P = 0.04). After adjusting for age, sex, and casual BP, both LVMI [in g/m2.7 and g/(m2.16 + 0.09)] and LV mass Z-score were significantly negatively associated with eGFR (β -1.08, P = 0.003; β -0.79, P = 0.007; and β -0.07, P = 0.01, respectively).

Conclusions: Young children with ARPKD have a higher burden of hypertension. LV mass was unexpectedly not significantly associated with BP but was negatively associated with eGFR.

Keywords: Ambulatory blood pressure monitoring; Autosomal recessive polycystic kidney disease; Hypertension; Left ventricular hypertrophy; Left ventricular mass.

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Conflict of interest statement

Declarations. Conflict of interests: The authors have no relevant financial or non-financial interests to disclose. Ethical approval: The study was determined by the CHOP Institutional Review Board (IRB 21–018989) to meet exemption criteria per Title 45 of the U.S. Code of Federal Regulations §46.104(d) 4(iii). A waiver of HIPAA authorization/consent was granted by the CHOP Institutional Review Board (IRB 21–018989). Consent for publication: A waiver of HIPAA authorization/consent was granted by the CHOP Institutional Review Board (IRB 21–018989).

References

    1. Alzarka B, Morizono H, Bollman JW et al (2017) Design and implementation of the Hepatorenal Fibrocystic Disease Core Center clinical database: a centralized resource for characterizing autosomal recessive polycystic kidney disease and other hepatorenal fibrocystic diseases. Front Pediatr 5:80. https://doi.org/10.3389/fped.2017.00080 - DOI - PubMed - PMC
    1. Nagasawa Y, Matthiesen S, Onuchic LF et al (2002) Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD gene. J Am Soc Nephrol 13:2246–2258. https://doi.org/10.1097/01.ASN.0000030392.19694.9D - DOI - PubMed
    1. Menezes LFC, Cai Y, Nagasawa Y et al (2004) Polyductin, the PKHD1 gene product, comprises isoforms expressed in plasma membrane, primary cilium, and cytoplasm. Kidney Int 66:1345–1355. https://doi.org/10.1111/j.1523-1755.2004.00844.x - DOI - PubMed
    1. Adeva M, El-Youssef M, Rossetti S et al (2006) Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD). Medicine 85:1–21. https://doi.org/10.1097/01.md.0000200165.90373.9a - DOI - PubMed
    1. Guay-Woodford LM, Desmond RA (2003) Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics 111:1072–1080 - DOI - PubMed

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