Early cardiovascular manifestations in children and adolescents with autosomal dominant polycystic kidney disease: a single center study

Abstract

Background: This study aims to describe the cardiovascular manifestations in children with autosomal dominant polycystic kidney disease (ADPKD) and detect their relation with kidney disease and type of gene mutation.

Methods: Twenty-one patients (7 to 19 years old) were included. Cardiovascular evaluation involved blood pressure (BP), indexed left ventricular mass (LVMI), pulse wave velocity (PWV), and carotid intima media thickness (cIMT) measurement. Patients were classified according to percentile reference values of these parameters in healthy children. The 95th percentile was the highest level of normal values. Glomerular filtration rate (GFR) and microalbuminuria were also measured.

Results: Antihypertensive treatment, large LVMI, high PWV, and increased cIMT were observed in 6 (28.6%), 2 (9.5%), 4 (19%), and 8 (38.1%) patients respectively. Antihypertensive treatment was not associated with either high PWV or increased cIMT. Linear correlation was noticed between LVMI and PWV (r² = 0.243, p = 0.023) and also between LVMI and cIMT (r² = 0.203, p = 0.041). The median age of patients with high PWV, increased cIMT, and large LVMI was 9.5, 13, and 18 years old. GFR was normal in all patients. Patients with increased cIMT presented higher levels of urine microalbumin to creatinine ratio (p = 0.025). Genetic mutation was available in 18 patients. Antihypertensive treatment was more frequent in patients without PKD1 missense mutation (p = 0.044).

Conclusions: High PWV and increased cIMT indicating arterial stiffness and hypertrophic vasculopathy may be present in children with ADPKD regardless BP status, and prior to GFR decline, suggesting that vascular disease precedes chronic kidney disease in ADPKD.

Keywords: ADPKD; Arterial stiffness; Cardiovascular; Gene mutation; Pediatrics; Renal function.