Management of Patients with Vascular Ehlers-Danlos Syndrome and Acute Coronary Syndrome: a Case Report

Background: Ehlers-Danlos syndrome (EDS) is a hereditary collagen vascular disorder characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Vascular EDS (vEDS) is a subtype of EDS which is characterized by vascular fragility.Case presentation: This is a case report of a young man with vEDS hospitalized for myocardial infarction. He was presented with a coronary dissection and developed aortic dissection, coronary rupture, and cardiac tamponade until death. Conclusion: This case report highlights how patients with vEDS and acute coronary syndrome show a higher risk of vascular complications compared with other patients, and their admission to the institution with a cardiac surgery room could be helpful and safe for better management of the complications. Non-invasive methods could be useful to exclude other vascular diseases, before the emergency coronary intervention

Outcomes of Redo Transcatheter Aortic Valve Replacement for the Treatment of Postprocedural and Late Occurrence of Paravalvular Regurgitation and Transcatheter Valve Failure

Background-Transcatheter aortic valves can degenerate in a manner similar to surgical bioprostheses. Methods and Results-Clinical and echocardiographic outcomes of patients who underwent redo transcatheter aortic valve replacement (TAVR) procedures >2 weeks post procedure were collected from 14 centers. Among 13 876 patients, 50 (0.4%) underwent redo TAVR procedure at participating centers. Indications for redo TAVR were moderate-severe prosthetic aortic valve stenosis (n=10, 21.7%), moderate-severe central prosthetic aortic valve regurgitation (n=13, 28.3%), and moderate-severe paraprosthetic aortic valve regurgitation (n=25, 50.0%). The index TAVR was most commonly a Medtronic CoreValve (N=38, 76.0%), followed by Edwards SAPIEN-type valves (n=12, 24.0%) and Portico (n=1, 2.0%). The redo TAVR device was most commonly a CoreValve/Evolut R (n=29, 58.0%), followed by a SAPIEN-type valve (n=20,40.0%) or a Boston Lotus valve (n=1, 2.0%). In 40 patients (80.0%), redo TAVR was performed using the identical device type or that of the succeeding generation. Valve performance was uniformly good after redo TAVR (mean transvalvular gradient post redo TAVR: 12.5±6.1 mm Hg). At hospital discharge, all patients remained alive, with 1 nondisabling stroke (2.0%) and 1 life-threatening bleed (2.0%). Permanent pacemaker implantation was required in 3 out of 35 patients without a prior pacemaker (8.6%). Late survival was 85.1% at a median follow-up of 1589 days (range: 31-3775) after index TAVR and 635 days (range: 8-2460) after redo TAVR. Conclusions-Redo TAVR for the treatment of postprocedural and late occurrence of paravalvular regurgitation and transcatheter aortic valve prosthesis failure seems to be safe, and it is associated with favorable acute and midterm clinical and echocardiographic outcomes