Left Cardiac Sympathetic Denervation for Long QT Syndrome: 50 Years' Experience Provides Guidance for Management.

Objectives: This study sought to report our single-center experience with left cardiac sympathetic denervation (LCSD) for long QT syndrome (LQTS) since 1973.

Background: LCSD is still underutilized because clinicians are often uncertain whether to use it versus an implantable cardioverter-defibrillator (ICD).

Methods: We performed LCSD in 125 patients with LQTS (58% women, mean QT interval corrected for frequency [QTc] 527 ± 60 ms, 90% on beta blockers) with a follow-up of 12.

Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia: The Role of Left Cardiac Sympathetic Denervation.

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Βlockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging.

Allogeneic lethally irradiated cord blood mononuclear cells in no-option critical limb ischemia: a "box of rain".

Critical limb ischemia (CLI) is burdened by a 40% major amputation rate, and a 5-year life expectancy <50%. We report the first in-human injection of lethally γ-irradiated non-human leukocyte antigen (HLA)-matched cord blood (CB)-derived mononuclear cells in a no-option CLI patient, to induce therapeutic neo-angiogenesis, with evidence of successful outcome supported by clinical findings (ulcer healing and pain relief), instrumental assessment (transcutaneous O2 pressure, ankle/brachial index, and contrast-enhanced ultrasonography), and histological demonstration of muscular tissue repair and capillary network expansion. If our approach will be confirmed, the huge number of CB units currently discarded might be redirected toward regenerative medicine purposes, leading to cutting-edge solutions for important unmet clinical needs, such as ischemic diseases, which remain the main cause of disability and mortality in western countries.

Successful endovascular treatment of a bronchial artery aneurysm refractory to transcatheter embolization.

Bronchial artery aneurysm (BAA) is a rare entity, detected in <1% of all patients who undergo selective bronchial arteriography. BAAs are potentially life-threatening when untreated. We describe the first BAA case, in the English language, treated only by thoracic aorta endografting, with an uneventful postoperative course with exclusion and thrombosis of the BAA.

Massive vertebral destruction associated with chronic rupture of infrarenal aortic aneurysm: case report and systematic review of the literature in the English language.

Study Design: Case report and review of literature.

Objective: To highlight the specific features of a rare, life-threatening, clinical picture.

Summary Of Background Data: Vertebral erosion (VE) is rarely associated with contained rupture of an abdominal aortic aneurysm.

Acute first onset of Ehlers-Danlos syndrome type 4 with spontaneous rupture of posterior tibial artery pseudoaneurysm.

Ehlers-Danlos syndrome type 4, the vascular type, is a rare, life-threatening inherited disorder of the connective tissue. Affected patients are at risk of arterial, bowel and uterine rupture during pregnancy. Generally, this syndrome remains undiagnosed until a sudden, acute presentation with organ rupture, and results in premature death, even if the patients survive the first and second major complications.

Impact of carotid stent cell design on vessel scaffolding: a case study comparing experimental investigation and numerical simulations.

Purpose: To quantitatively evaluate the impact of carotid stent cell design on vessel scaffolding by using patient-specific finite element analysis of carotid artery stenting (CAS).

Methods: The study was organized in 2 parts: (1) validation of a patient-specific finite element analysis of CAS and (2) evaluation of vessel scaffolding. Micro-computed tomography (CT) images of an open-cell stent deployed in a patient-specific silicone mock artery were compared with the corresponding finite element analysis results.

Risk of dissection in thoracic aneurysms associated with mutations of smooth muscle alpha-actin 2 (ACTA2).

Objective: To evaluate the prevalence and phenotype of smooth muscle alpha-actin (ACTA2) mutations in non-syndromic thoracic aortic aneurysms and dissections (TAAD).

Design: Observational study of ACTA2 mutations in TAAD.

Setting: Centre for Inherited Cardiovascular Diseases.

Rationale and design of a trial evaluating the effects of losartan vs. nebivolol vs. the association of both on the progression of aortic root dilation in Marfan syndrome with FBN1 gene mutations.

Background: The major clinical problem of Marfan syndrome (MFS) is the aortic root aneurysm, with risk of dissection when the root diameter approximates 5 cm. In MFS, a key molecule, transforming growth factor-beta (TGF-beta), normally bound to the extracellular matrix, is free and activated. In an experimental setting, TGF-beta blockade prevents the aortic root structural damage and dilatation.

Hypoplasia and fibromuscular dysplasia of infrarenal abdominal aorta with downstream aneurysm: case report and review of the literature.

Fibromuscular dysplasia represents one of the more common types of arterial fibrodysplasia, a heterogeneous group of nonatherosclerotic vascular occlusive and aneurysmal diseases. This disorder mainly affects renal and cerebral arteries, and less frequently, arm, leg, and visceral arteries. Exceptionally, it has been described in the abdominal aorta.