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.