The Ross–Yacoub procedure for aneurysmal autograft roots: A strategy to preserve autologous pulmonary valves

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VAD in failing Fontan: simulation of ventricular, cavo-pulmonary and biventricular assistance in systolic/diastolic ventricular dysfunction and in pulmonary vascular resistance increase.

Aim: Due to the lack of donors, VADs could be an alternative to heart transplantation for Failing Fontan patients (PTs). Considering the complex physiopathology and the type of VAD connection, a numerical model (NM) could be useful to support clinical decisions. The aim of this work is to test a NM simulating the VADs effects on failing Fontan for systolic dysfunction (SD), diastolic dysfunction (DD) and pulmonary vascular resistance increase (PRI). Methods: Data of 10 Fontan PTs were used to simulate the PTs baseline using a dedicated NM. Then, for each PTs a SD, a DD and a PRI were simulated. Finally, for each PT and for each pathology, the VADs implantation was simulated. Results: NM can well reproduce PTs baseline. In the case of SD, LVAD increases the cardiac output (CO) (35%) and the arterial systemic pressure (ASP) (25%). With cavo-pulmonary assistance (RVAD) a decrease of inferior vena cava pressure (IVCP) (39%) was observed with 34% increase of CO. With the BIVAD an increase of ASP (29%) and CO (37%) was observed. In the case of DD, the LVAD increases CO (42%), the RVAD decreases the IVCP. In the case of PRI, the highest CO (50%) and ASP (28%) increase is obtained with an RVAD together with the highest decrease of IVCP (53%). Conclusions: The use of NM could be helpful in this innovative field to evaluate the VADs implantation effects on specific PT to support PT and VAD selection

Arterial tortuosity syndrome in two Italian paediatric patients

<p>Abstract</p> <p>Background</p> <p>Arterial tortuosity syndrome (ATS) (OMIM #208050) is a rare autosomal recessive connective tissue disorder characterized by tortuosity and elongation of the large and medium-sized arteries, propensity to aneurysms formation, vascular dissection, and pulmonary arteries stenosis. ATS is caused by mutations in <it>SLC2A10 </it>gene, encoding for the facilitative glucose transporter 10 (GLUT10). So far, 17 <it>SLC2A10 </it>mutations have been reported in 32 families, two of which were Italian with a total of five patients. Here we present the clinical and molecular characterization of two novel Italian paediatric ATS patients.</p> <p>Methods</p> <p>The exons and intronic flanking regions of <it>SLC2A10 </it>gene were amplified and direct sequencing was performed.</p> <p>Results</p> <p>In both patients, the involvement of major- and medium-sized arteries was characteristic; the nonvascular connective tissue manifestations were mild and not pathognomic of the disorder. Both patients, born from non-consanguineous parents, were heterozygous for two different <it>SLC2A10 </it>mutations, three of which were recurrent and one was novel (p.Arg231Trp). This mutation is localized at the endofacial loop between the transmembrane domains 6 and 7 of GLUT10.</p> <p>Conclusion</p> <p>Two novel ATS patients were characterized at clinical and molecular level. Overall, four ATS unrelated families are known in Italy so far. Though ATS clinical delineation improved in the last years, further works in the comprehension of disease presentation and complications onset, particularly in paediatric age, and on ATS molecular basis are needed to add new insights for diagnosis and prevention strategies for related complications.</p

Endomyocardial Biopsy in Pediatric Myocarditis and Dilated Cardiomyopathy: A Tool in Search for a Role

Endomyocardial biopsy (EMB) is a well-known diagnostic tool for the investigation and treatment of myocardial diseases and remains the gold standard for the diagnosis of myocarditis. Due to its invasiveness, with a complication rate ranging from 1 to 15%, its role in the diagnostic work-up of pediatric heart failure is not well established. The aim of this review is to define the role of EMB as diagnostic technique in the work up of children presenting with severe left ventricular dysfunction with the support of our center experience

Bicuspid aortic valve disease from infancy to older age: A 25-year experience from an Italian referral center

Aim: Bicuspid aortic valve (BAV) is the most common congenital heart defect, with considerable risk of morbidity and mortality. The purpose of the study was to analyze clinical and echocardiographic presentation of BAV in a large-volume tertiary Italian center and to test their interaction with full age span, sex, and first diagnosis versus second referral. Methods: Consecutive patients of all ages diagnosed with BAV at our center from January 1988 to December 2012 were retrospectively included. Exclusion criteria were as follows: associated complex congenital cardiac disease, systemic syndrome, and previous cardiac surgery. Results: Eligible patients were 790, divided by age quartiles. Seventy-two percent of patients had any grade BAV dysfunction. Aortic valve stenosis was more frequent in the first (24%) and fourth (24%) quartiles. This corresponds to a double-peak stenosis severity curve, being more severe at a very young age and in the elderly. Aortic valve regurgitation was more prevalent in each quartile than stenosis, with a prevalence of 72% in the second quartile and 77% in the third quartile. This corresponds to a single-peak regurgitation severity curve, being more severe in the fourth and fifth decades of life. Patients with previously diagnosed BAV had more significant valve dysfunction in comparison to patients with first diagnosis of BAV, either stenosis (15% vs. 21%, P = 0.024) or regurgitation (58% vs. 68%, P = 0.006). Conclusion: The dominant BAV dysfunction in this large Northern Italian community is regurgitation, with higher severity of disease in the fourth and fifth decades of life

Is late luminal loss an accurate predictor of the clinical effectiveness of drug-eluting stents in the coronary arteries?

Late luminal loss after percutaneous coronary intervention, although not normally distributed, has been shown to be monotonically correlated with the probability of binary angiographic restenosis after drug-eluting stent implantation. A recently proposed method has been shown to predict the restenosis rate after sirolimus-eluting stent and paclitaxel-eluting stent implantation using a power transformation of the value of late loss obtained after implantation of the 2 stent types. We used the same method to compute and compare restenosis rates from late loss values observed in the "head-to-head" randomized sirolimus-eluting stent versus paclitaxel-eluting stent comparisons available thus far. Our results showed that the model proposed has a poor overall ability to predict the real incidence and relative risk of restenosis because the use of late loss as an end point tended to overestimate the difference in restenosis, and the derived effect estimate of 1 stent compared with that of the other seemed to be overemphasized with respect to the real risk. We thus believe that further investigations are needed to appraise the real clinical effect of late loss and its reliability as an end point in direct comparative drug-eluting stent trials before its use can be recommended as a clinical surrogate. (C) 2006 Elsevier Inc. All rights reserved

Complete surgical resection of giant fibroma of the interventricular septum and left ventricle in an infant

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