Congenital coronary arteriovenous fistulae in a transplanted heart

4nononeWe report a very rare case of a heart transplanted man with angiographic documentation of multiple congenital fistulae arising from both coronary arteries and draining into main pulmonary artery. A transthoracic echocardiogram and right heart catheterization were also performed, to assess the functional importance of the shunt. We also carried out a brief review about this rare condition and we reported considerations about its prognosis and treatment.noneCannarile, Pierpaolo; Iadanza, Alessandro; Mondillo, Sergio; Pierli, CarloCannarile, Pierpaolo; Iadanza, Alessandro; Mondillo, Sergio; Pierli, Carl

Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett Syndrome

International audienceRett syndrome (RTT) is a devastating neurodevelopmental disorder with a 300-fold increased risk rate for sudden cardiac death. A subclinical myocardial biventricular dysfunction has been recently reported in RTT by our group and found to be associated with an enhanced oxidative stress (OS) status. Here, we tested the effects of the naturally occurring antioxidants ω-3 polyunsaturated fatty acids (ω-3 PUFAs) on echocardiographic parameters and systemic OS markers in a population of RTT patients with the typical clinical form. A total of 66 RTT girls were evaluated, half of whom being treated for 12 months with a dietary supplementation of ω-3 PUFAs at high dosage (docosahexaenoic acid ~71.9 ± 13.9 mg/kg b.w./day plus eicosapentaenoic acid ~115.5 ± 22.4 mg/kg b.w./day) versus the remaining half untreated population. Echocardiographic systolic longitudinal parameters of both ventricles, but not biventricular diastolic measures, improved following ω-3 PUFAs supplementation, with a parallel decrease in the OS markers levels. No significant changes in the examined echocardiographic parameters nor in the OS markers were detectable in the untreated RTT population. Our data indicate that ω-3 PUFAs are able to improve the biventricular myocardial systolic function in RTT and that this functional gain is partially mediated through a regulation of the redox balance