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

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/m^2.7 and g/(m^2.16 + 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/m^2.7 > 95th percentile, and in 50% based on LVMI > 45 g/(m^2.16 + 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/m^2.7 and g/(m^2.16 + 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.