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Proximal aortic enlargement is associated with an increased risk of heart failure and all-cause mortality. Recently, aortic root dilatation (ARD) was reported in postoperative patients with ventricular septal defects (VSDs). However, the impact of ARD on left ventricular (LV) function in postoperative VSD patients remains unclear. Thus, the aim of this study was to investigate the effect of ARD on LV function in patients with postoperative VSD. One hundred and thirty-five patients (> 15 years of age) with surgically repaired isolated ventricular defects and who underwent transthoracic echocardiography in our institution between 2009 and 2013 were identified. ARD was defined as an observed aortic root diameter/body surface area > 2.1 cm/m2. The propensity score estimating the probability of having ARD adjusted for anatomical and clinical characteristics was calculated. Forty-four patients (32.6%) had ARD. In unadjusted analyses, right ventricular systolic pressure, Tei index, and E/e’ were significantly (p

Mucopolysaccharidoses (MPS) are disorders characterized by impaired glycosaminoglycan (GAG) catabolism as a consequence of a deficiency or the absence of lysosomal enzymes directly involved in their degradation. Multiple organ systems are involved in MPS, including the cardiovascular system. Recently, aortic root dilatation (ARD) has been described in these patients. Thus, we reviewed aortic root diameter measurements in 69 MPS patients from a single center from 2000 to 2016. Aortic root diameter z scores were calculated based on data published by Colan et al. according to the body surface area (BSA) determined using the Haycock formula. The overall incidence of ARD in MPS patients was 39.1%. Higher mean z scores were present in patients with MPS IVA and VI when compared to MPS I and II. Aortic root z scores were higher in older MPS IVA patients, which may suggest a progressive ARD change in this MPS type. No significant differences were found before and after enzyme replacement therapy (ERT) in 11 patients with available data (2 with MPS I; 4 with MPS II; 2 with MPS IVA, and 3 with MPS VI). This work provides further evidence that ARD is common in different types of MPS, being especially evident in MPS IVA, but with a significant occurrence also in MPS VI.

Accumulating data suggest that activity of matrix metalloproteinases (MMPs) is increased in aortic dissection, and in thoracic and abdominal aneurysms. In the present study we sought to determine circulating stromelysin (MMP-3) concentration and its relationship with hypertension-induced aortic root dilatation. The study population included 42 patients with essential hypertension. The subjects were divided into two groups according to echocardiographically measured aortic diameter as those with aortic dilatation (n = 22) and without aortic root dilatation (n = 20). Plasma concentration of MMP-3 was determined by one-step sandwich enzyme immunoassay (EIA) method and compared in both groups. Baseline demographic properties were similar in both groups. Plasma stromelysin (MMP-3) level was significantly higher in patients with aortic dilatation than those without aortic dilatation (5.2 +/- 2.3 vs 3.3 +/- 1.9 ng/ml; P = 0.007). In conclusion, we found that circulating stromelysin (MMP-3) concentration was elevated in hypertension-induced aortic root dilatation.