Marfan Syndrome (MFS) is a systemic disorder caused by mutations in fibrillin-1. The most common cause of mortality in MFS is dissection and rupture of the aorta. Due to a highly variable and age-dependent clinical spectrum, the diagnosis of MFS still remains sophisticated. The aim of the study was to determine if there exist phenotypic features that can play the role of “red flags” in cases of MFS suspicion. The study population included 306 patients (199 children and 107 adults) who were referred to the Department of Pediatric Cardiology due to suspicion of MFS. All patients underwent complete clinical evaluation in order to confirm the diagnosis of MFS according to the modified Ghent criteria. MFS was diagnosed in 109 patients and marfanoid habitus in 168 patients. The study excluded 29 patients with other hereditary thoracic aneurysm syndromes. Comparative analysis between patients with Marfan syndrome and marfanoid habitus was performed. Symptoms with high prevalence and high positive likelihood ratio were identified (pectus carinatum, reduced elbow extension, hindfoot deformity, gothic palate, downslanting palpebral fissures, lens subluxation, myopia ≥ 3 dioptres remarkably high stature). The differentiation between patients with MFS and marfanoid body habitus is not possible by only assessing external body features; however, “red flags” could be helpful in the screening phase.
Background: Evidence concerning the efficacy of the embolic protection devices (EPDs) in saphenous vein graft (SVG) percutaneous coronary intervention (PCI) is sparse. The study was designed to compare major cardiovascular events of all-comer population of SVG PCI with and without EPDs at one year of follow-up. Methods and results: A multi-center registry comparing PCI with and without EPDs in consecutive patients undergoing PCI of SVG. The group comprised 792 patients, among which 266 (33.6%) had myocardial infarction (MI). The primary composite endpoint was major adverse cardiac and cerebrovascular event (MACCE) defined as death, MI, target vessel revascularization (TVR), and stroke assessed at one year. After propensity score analysis, there were no differences in MACCE (21.9% vs. 23.9%; HR 0.91, 95% CI 0.57–1.45, p = 0.681, respectively) nor in secondary endpoints of death, MI, TVR, target lesion revascularization (TLR) and stroke at one year in EPDs PCI group vs. no-EPDs PCI group. Similarly, there were no differences between groups in the study endpoints at 30 days follow-up. Conclusions: There were no clinical benefit for routine use of EPDs during SVG PCI in short and long-term follow-up. Further studies are warranted to explore the effect of individual types of EPDs on clinical outcomes.
30 June 2017 in Archives of Medical Science – Atherosclerotic Diseases
Background: The balance between thromboembolic complications and bleeding risk in anticoagulated patients with atrial fibrillation remains challenging, with left atrial appendage occlusion (LAAO) representing a potential alternative. Our prospective multicenter study aimed to evaluate the feasibility, safety, technical, and procedural outcomes of the contemporary practice of stand‐alone LAAO. Methods: The SALAMANDER (Stand‐Alone Left Atrial Appendage Occlusion for Thromboembolism Prevention in Nonvalvular Atrial Fibrillation Disease) registry is a real‐world, multicenter, observational cohort study conducted at 16 cardiac centers in Europe between 2010 and 2024, evaluating the safety and efficacy of LAAO using contemporary devices. Results: A total of 1660 patients were enrolled, with a median age of 76 (interquartile range, 70–81) years and 38% being women. The median CHA 2 DS 2 ‐VASc score was 4 (interquartile range, 3–5). The most common indication for LAAO was significant bleeding (83.7% of patients), most frequently during treatment with direct oral anticoagulants (68.8%) more than vitamin K antagonists (24.9%). The predominant bleeding sites were the lower (27.9%) and upper (21.7%) gastrointestinal tracts, with 17.4% having a history of hemorrhagic stroke. The most common antithrombotic regimen before the procedure was direct oral anticoagulants (48.9%), while postprocedural therapy most often included dual antiplatelet therapy (50%). The technical success rate was 95.5%, with residual leak (2.2%) and tamponade (1.4%) as the main causes of failure. Procedural success was 90.5%, most often limited by vascular complications (3.7%), periprocedural death (1.1%), and major bleeding (0.7%). Technical and procedural success rates did not differ significantly between the devices used. Conclusions: In this large prospective cohort, technical and procedural success rates were similar across all LAAO devices, suggesting comparable safety and efficacy. Postprocedural therapy typically involves dual antiplatelet therapy, with all patients requiring some pharmacological treatment. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT05144958.
We present a 68-year-old men with paroxysmal atrial fibrillation, in whom the aorta was inadvertently punctured while obtaining transseptal access for pulmonary vein isolation. After transseptal sheath had been retracted from the aorta the patient was stable with no pericardial effusion, therefore, the leakage was left to heal. Transoesophageal echocardiography taken 2 months later revealed connection between the non-coronary sinus of Valsalva and the right atrium (RA), which measured 3 mm in diameter (PanelA). Percutaneous closure of the fistula was attempted. The fistula was intubated from the aortic side with a guiding Amplatz Right 2 5-Fr catheter. Two intracoronary wires were passed through the fistula into the RA. With a support of a balloon catheter positioned in the RA, the catheter was able to cross the...
1 October 2013 in International Journal of Cardiology