# Assessment of Cardiovascular Disease in Autosomal Dominant Polycystic Kidney Disease
###### Abstract
Over the years, many innovations have been reported in renal imaging with computed tomography (CT) or magnetic resonance imaging (MRI) to evaluate, in addition, to renal volume, intermediate volume or renal fibrotic and perfusion volume [6; 7] with important prognostic implications for ADPKD patients. Intracranial aneurysm (ICA) is one of the cardiovascular manifestations of ADPKD, but information about the natural history of ICA in ADPKD patients comes from small, single-center observational studies. These mainly describe an increased prevalence of asymptomatic ICA detected via magnetic resonance angiography (MRA), with 9-12% of patients demonstrating ICA, compared with \(\sim\)2-3% of the general population. The prevalence is higher in ADPKD patients with a positive family history compared to ADPKD patients with a negative family history. Family history is recognized as the main risk factor for ICA rupture as well, which occurs at the average age of 40, almost 10 years earlier than in the general population [8]. Data from pre-symptomatic screening demonstrated that the majority of ICA localize in the anterior circulation of the circle of Willis, with nearly all being of a small size <7 mm, falling in the low-risk category for rupture. Therefore, guidelines suggest to only perform screening for ICA in ADKPK patients with family or personal history of ICA rupture. All patients with ADPKD should receive counseling about the risk of ICA, considering the pros and cons of pre-symptomaticscreening and reserving diagnostic imaging for those individuals who remain anxious about their risk and individuals with high-risk professions [8; 9]. Vascular disorders such as aneurysms and arterial dissections of large arteries, including aorta, coronary, and splenic arteries, are reported in ADPKD patients, representing an important cause of death. However, the prevalence of abdominal aortic aneurysms does not seem to be increased in patients with ADPKD. Polycystin proteins have a role in epithelial cell/matrix interactions, and polycystin mutations are associated with collagen and extracellular matrix abnormalities. The high expression of PKD1 and 2 in the human adult vascular wall, particularly in the dense plaques of the smooth muscle cell, accounts for the risk of vascular wall modification. Characteristic phenotypes of some ADPKD patients showing archnodactylly, high-arched palates, pectorus deformities, joint laxity, flat feet, and positive thumb signs should alert clinicians toward an increased predisposition to vascular involvement [10]. There is no standard assessment suggested in these patients, and the clinical presentation of vascular disorders may be highly variable and mimic many common conditions. Given the high risk of complications in these patients, contrast-enhanced computed tomography should be considered when suggestive symptoms develop for differential diagnosis. The other major extrarrenal complications of ADPKD include hepatic and pancreatic cysts, colonic diverticula, dolichoectasias, abdominal wall hernias, cerebral and ascending thoracic aorta aneurysms, seminal vesicle cysts, and male infertility [2]. ADPKD is also associated with significant pain and discomfort, which may affect the quality of life of these patients. The quality of life (QOL) of patients with ADPKD could be associated with abdominal distention, pain, and anorexia caused by liver and kidney enlargement, even if other associated symptoms could also affect QOL such as sleep disturbance, heartburn, urinary tracts, fever, and hematuria [11]. Current treatment strategies include conservative therapy and reducing cyclic adenosine monophosphate levels, cell proliferation, and fluid secretion with somatostatin analogs and vasopressin V2 receptor antagonists that have been shown to slow the deterioration of renal function and the growth of renal and hepatic cysts [1]. A thorough clinical evaluation is essential to characterize the high cardiovascular risk in these patients through cardiovascular imaging to report important prognostic evaluations.