# Assessment of Cardiovascular Disease in Autosomal Dominant Polycystic Kidney Disease
###### Abstract  
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited kidney disease which leads to progressive kidney failure. About 5-10% of patients requiring renal replacement therapy are affected by ADPKD. Cardiovascular diseases are the main causes of morbidity and mortality in these patients with ADPKD; arterial hypertension (AH) is the first symptom with a very early onset. Anyway, some other cardiovascular abnormalities have been reported in ADPKD regardless of the presence of AH. With this background, we conducted a systematic review, collecting all randomized controlled trials (RCTs) and quasi-RCTs found on the main databases; we evaluated the evidence about different imaging techniques to grade the cardiovascular risk in a very early stage of disease. This review aims to describe all cardiovascular assessments in ADPKD patients to improve clinicians' ability to discover cardiovascular involvement early, allowing appropriate therapies promptly. autosomal dominant polycystic kidney disease; left ventricular hypertrophy; heart rate variability; cardiovascular risk; flow-mediated dilation; carotid intima-media thickness; pulse wave velocity +
Footnote †: journal: Medical  
compared to the general population, with 33% of deaths mainly due to ischemic heart disease and congestive heart failure [2]. Beyond the classic cardiovascular complications characterized by heart failure and coronary artery disease, well known in chronic kidney disease (CKD), ADPKD itself results in a genetically defined increased risk of cardiovascular complications. Arterial hypertension (AH), a common finding in these patients, often occurs before the onset of renal failure and is associated with the most rapid progression to ESRD, with an increased cardiovascular risk. The frequency of AH is increased at young age and is more frequent in patients with mutations in _PKD1_ than in _PKD2_ and in polycystic patients with hypertensive parents. Early diagnosis is facilitated by the self-measurement of blood pressure or ambulatory BP monitoring (ABPM), particularly in those patients with masked hypertension who do not show a normal BP decrease at night-time (non-dippers) [3]. The pathogenesis of AH in ADPKD is not fully elucidated, but specific mechanisms such as the activation of the renin-angiotensin-aldosterone system (RAAS), impaired nitric oxide (NO)-related vasorelaxation, increased sympathetic nerve activity, increased plasma endothelin-1 concentrations, and insulin resistance have been revealed. Sodium overload is also characteristic of ADPKD patients, and sodium-sensitive hypertension is described, associated with increased total kidney volume [3]. Left ventricular hypertrophy, a powerful, independent risk factor for cardiovascular morbidity and mortality, frequently occurs in patients with ADPKD. Both AH and left ventricular hypertrophy have important roles in cardiovascular complications in these individuals. The effect of cyst enlargement on renal vessels with parenchyma ischemia explains the activation of RAAS, which plays an important role in the development of hypertension in ADPKD [3]. Altered intrarenal hemodynamics cause endothelial dysfunction, NO production, and the hyperactivation of the sympathetic nervous system (SNS). The RAAS is stimulated at an early stage of the disease, even before the appearance of hypertension and other clinical findings. Similarly, increased left ventricular mass indices and diastolic dysfunction are reported in ADPKD patients with well-preserved renal function before the development of AH. Endothelial dysfunction, inflammation, and accelerated atherosclerosis are early-stage changes in ADPKD patients [4]. Biventricular diastolic dysfunction, endothelial dysfunction, increased carotid intima-media thickness (IMT), and increased arterial stiffness are present even in young ADPKD patients with normal blood pressure and well-preserved renal function [5].