Extracellular cysteine disulfide bond break at Cys122 disrupts PIP-dependent Kir2.1 channel function and leads to arrhythmias in Andersen-Tawil Syndrome.

Background: Andersen-Tawil Syndrome Type 1 (ATS1) is a rare heritable disease caused by mutations in the strong inwardly rectifying K channel Kir2.1. The extracellular Cys122-to-Cys154 disulfide bond in the Kir2.

Seven Microaneurysms: Description of an Experimental Rodent Model for Neurovascular Training.

Aim: To demonstrate the microsurgical procedures, and to evaluate the feasibility of living models of experimental neurovascular training by developing new complex vascular exercises mimicking the most common intracranial aneurysms.

Material And Methods: The procedures were performed under a Zeiss (OPMI pico f170) microscope using basic microsurgery instruments, 10/0 Nylon and blue Polypropylene micro-sutures. We selected adult albino Wistar rats weighing between 258 and 471g each.

Machine Learning Improves Cardiovascular Risk Definition for Young, Asymptomatic Individuals.

Background: Clinical practice guidelines recommend assessment of subclinical atherosclerosis using imaging techniques in individuals with intermediate atherosclerotic cardiovascular risk according to standard risk prediction tools.

Objectives: The purpose of this study was to develop a machine-learning model based on routine, quantitative, and easily measured variables to predict the presence and extent of subclinical atherosclerosis (SA) in young, asymptomatic individuals. The risk of having SA estimated by this model could be used to refine risk estimation and optimize the use of imaging for risk assessment.

Management of a ruptured intraventricular aneurysm arising from distal anterior choroidal artery (AChA): pediatric case report.

Intracranial aneurysms arising from the distal anterior choroidal artery (AChA) are uncommon entities, with less than 30 cases reported. A 4-year-old boy was admitted to the Emergency Department with a sudden onset of severe headache and vomiting. CT scan of the head showed signs of intraventricular hemorrhage (IVH) and subarachnoid hemorrhage (SAH).

Intelligibility in speech maskers with a binaural cochlear implant sound coding strategy inspired by the contralateral medial olivocochlear reflex.

We have recently proposed a binaural cochlear implant (CI) sound processing strategy inspired by the contralateral medial olivocochlear reflex (the MOC strategy) and shown that it improves intelligibility in steady-state noise (Lopez-Poveda et al., 2016, Ear Hear 37:e138-e148). The aim here was to evaluate possible speech-reception benefits of the MOC strategy for speech maskers, a more natural type of interferer.

[Progress in pediatric cardiology, congenital heart disease in adults, and heart surgery for congenital heart disease].

The field of pediatric cardiology is continually developing and now covers not only congenital and acquired heart disease in children but also congenital heart disease in adults and the prenatal diagnosis and prevention of heart disease. This review highlights new findings in the field of genetics, selected articles on the use of magnetic resonance imaging and multislice CT in diagnosis, and recent publications on electrophysiology and the surgical treatment of children and adults with congenital heart disease. In addition, the increasingly advanced use of mechanical assist devices as a bridge to heart transplantation in children is also discussed.

[Mucocutaneous Leishmaniasis: an imported illness with ENT repercussions].

We report a case of mucocutaneous Leishmaniasis, an uncommon illness in our area. Leishmaniasis covers a group of diseases caused by protozoa of the genus Leishmania with several pathogenic species transmitted by Phlebotomus mosquitoes. Leishmania braziliensis is endemic in parts of South America and is responsible for the mucocutaneous Leishmaniasis reported here.

[Interventional catheterization after the Norwood procedure].

Introduction And Objectives: To carry out a retrospective analysis of the indications for, and the results and complications of interventional catheterization after the Norwood procedure.

Methods: Between February 1993 and December 2006, 25 interventional catheterizations were performed in 14 patients who had undergone the Norwood procedure, prior to the Glenn or Fontan procedure.

Results: Nine angioplasties were carried out for recoarctation in seven of the 14 patients (2 patients developed restenosis after their first angioplasty).

[Balloon angioplasty for native coarctation in children: immediate and medium-term results].

Introduction And Objectives: The use of balloon angioplasty to treat native aortic coarctation in pediatric patients is controversial. Our aims were to report our experience with this technique and to compare retrospectively the immediate and medium-term results obtained during 2 distinct time periods.

Subjects And Method: 53 patients who underwent balloon angioplasty for native coarctation were divided into 2 groups: (A) those treated between 1985-1988 (n=26); and (B) those treated between 1993-2003 (n=27).

Rapid progression of midventricular obstruction in adults with double-chambered right ventricle.

Objective: The purpose of this study was to determine the rate of progression of midventricular obstruction in adolescents and adults with double-chambered right ventricle.

Methods: Clinical and echocardiographic findings in 45 patients (mean age 26 +/- 6 years, range 15-44) diagnosed with double-chambered right ventricle were retrospectively analyzed. Twenty patients underwent surgical repair before the age of 15 years.

[Surgical closure of atrial septal defect before or after the age of 25 years. Comparison with the natural history of unoperated patients].

Introduction: Surgical closure of an atrial septal defect (ASD) before the age of 25 years has been demonstrated to reduce complications during adulthood. However, the outcome for patients operated after the age of 25 is still debated.

Methods: In a retrospective study we examined the outcome of early and late surgical repair of ASD in adults, as compared with the natural evolution of unoperated patients.

[Multiplane transesophageal echocardiography in the preoperative evaluation of the sinus of Valsalva fistula to right chambers].

Introduction And Objectives: Ruptured sinus of Valsalva aneurysm to right cardiac chambers is an uncommon lesion in Western countries. The prognosis is usually serious unless the condition is promptly treated surgically. For this reason an accurate anatomical and functional evaluation is necessary.

Predisposing conditions for atrial fibrillation in atrial septal defect with and without operative closure.

The aims of this study were to determine the prevalence and predisposing conditions for atrial fibrillation (AF) in adults with atrial septal defect (ASD) and to evaluate the influence of age at surgical repair. The study population consisted of 286 adults with ASD (mean age 39.5 +/- 19 years).