Direct Implantation of Transcatheter Valve in Mitral Annular Calcification: A Multicenter Study.

Background: Mitral valve replacement in the setting of severe annular calcification (MAC) is associated with high morbidity and mortality. Direct surgical implantation of a transcatheter heart valve (THV) through a transatrial approach is a strategy to mitigate surgical risk. This study reports the perioperative and 1-year outcomes of mitral valve replacement using a THV in patients with severe circumferential MAC at 3 Canadian centers.

DICER1-sarcomas of GYN tract: Expanding on an emerging entity.

Tumors with pathogenic DICER1 mutation are rare and encompass sporadic or hereditary benign, intermediate and malignant tumors. DICER1-associated sarcomas are heterogeneous; however, the prototypical ones in the GYN-tract include embryonal rhabdomyosarcoma, adenosarcoma and moderately to poorly differentiated Sertoli-Leydig tumor. In this report, we present three unique uterine sarcomas with DICER1 mutation and remarkable diffuse round/spindle cell morphology.

Aberrant activation of AXL may drive progression of squamous cell carcinoma in CLL patients: a mechanistic study with clinical implications.

Background: Occurrence of squamous cell carcinoma (SCC) even in early-stage, untreated chronic lymphocytic leukemia (CLL) patients can be a significant morbidity issue with occasional transformation into metastatic skin lesions.

Methods: CLL cells and extracellular vesicles (EVs) from CLL patients' blood/plasma were purified and used. Expression/activation of AXL and its functions in normal keratinocytes (HEKa) were assessed in vitro co-culture system and in SCC tissues.

Adamantinoma-Like Ewing Sarcoma of the Mandible Evaluated on 18F-FDG PET/CT.

Ewing sarcoma is the second most common primary bone tumor in children. Typical Ewing sarcoma most frequently occurs in long bones and within the pelvis. ALES (adamantinoma-like Ewing sarcoma) is a rare subtype of Ewing sarcoma that is characterized by epithelial differentiation in addition to small round blue cells.

Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in Disguise.

Pulmonary artery intimal sarcoma (PAIS) is a very rare tumour. The prevalence of PAIS is estimated to be between 0.001% and 0.

Consensus Approaches to Identify Incident Dementia in Cohort Studies: Systematic Review and Approach in the Successful Aging after Elective Surgery Study.

Objectives: To survey the current methods used to ascertain dementia and mild cognitive impairment (MCI) in longitudinal cohort studies, to categorize differences in approaches and to identify key components of expert panel methodology in current use.

Methods: We searched PubMed for the past 10 years, from March 6, 2007 to March 6, 2017 using a combination of controlled vocabulary and keyword terms to identify expert panel consensus methods used to diagnose MCI or dementia in large cohort studies written in English. From these results, we identified a framework for reporting standards and describe as an exemplar the clinical consensus procedure used in an ongoing study of elective surgery patients (the Successful Aging after Elective Surgery study).

Postoperative atrial fibrillation is not pulmonary vein dependent: results from a randomized trial.

Background: Although often short-lived and self-limiting, postoperative atrial fibrillation (POAF) is a well-recognized postoperative complication of cardiac surgery and is associated with a 2-fold increase in cardiovascular mortality and morbidity.

Objective: Our aim was to determine whether intraoperative bilateral pulmonary vein radiofrequency ablation decreases the incidence of POAF in patients undergoing coronary artery bypass grafting (CABG).

Methods: A total of 175 patients undergoing CABG was prospectively randomized to undergo adjuvant bilateral radiofrequency pulmonary vein ablation in addition to CABG (group A; n = 89) or CABG alone (group B; n = 86).

Beyond low tidal volumes: ventilating the patient with acute respiratory distress syndrome.

The cornerstone of lung protective ventilation in patients with acute respiratory distress syndrome (ARDS) is a pressure- and volume-limited strategy. Other interventions have also been investigated. Although no method for positive end-expiratory pressure (PEEP) titration has proven most advantageous, experimental and clinical data support the use of higher PEEP in patients with moderate/severe ARDS.

Clinical outcomes of minimally invasive endoscopic and conventional sternotomy approaches for atrial septal defect repair.

Background: Concerns remain that minimally invasive atrial septal defect (ASD) repair may compromise patient outcomes. We compared clinical outcomes of adult patients undergoing ASD repair via a minimally invasive endoscopic approach versus a "gold standard" sternotomy.

Methods: We retrospectively reviewed the clinical outcomes of consecutive patients who underwent ASD patch repair at our institution between 2002 and 2012.

Extracorporeal membrane oxygenation in the acute treatment of cardiovascular collapse immediately post-partum.

We describe the use of extracorporeal membrane oxygenation (ECMO) in a 30-year old woman at 37 weeks' gestation, following cardiac arrest from pulmonary embolism immediately post-partum from an emergent Caesarean section. In this case, ECMO was initiated though modified techniques with only the equipment available in a delivery room as a last resort to save a new mother after a significant downtime of 83 min. The patient received tissue plasminogen activator during the resuscitation resulting in significant blood loss.

VMA21 deficiency prevents vacuolar ATPase assembly and causes autophagic vacuolar myopathy.

X-linked Myopathy with Excessive Autophagy (XMEA) is a childhood onset disease characterized by progressive vacuolation and atrophy of skeletal muscle. We show that XMEA is caused by hypomorphic alleles of the VMA21 gene, that VMA21 is the diverged human ortholog of the yeast Vma21p protein, and that like Vma21p, VMA21 is an essential assembly chaperone of the vacuolar ATPase (V-ATPase), the principal mammalian proton pump complex. Decreased VMA21 raises lysosomal pH which reduces lysosomal degradative ability and blocks autophagy.

Constrictive pericarditis associated with atypical antipsychotics.

We report the successful surgical intervention in a case of constrictive pericarditis after long-term use of atypical antipsychotics. Pericarditis developed in our patient with a longstanding history of schizophrenia treated with atypical antipsychotics. Pericardiectomy was undertaken, and the patient's presenting symptom of shortness of breath resolved subsequently with an uneventful postoperative course.

Perceptions of emergency medicine residents and fellows regarding competence, adverse events and reporting to supervisors: a national survey.

Objective: We sought to characterize the perceptions of emergency medicine (EM) residents and fellows of their clinical and procedural competence, as well as their attitudes, practices and perceived barriers to reporting these perceptions to their supervisors.

Methods: A Web-based survey was distributed to residents and fellows, via their residency directors, in all Canadian EM residency programs outside of Quebec.

Results: Of 220 residents and fellows contacted in 9 of 10 EM programs of the Royal College of Physicians and Surgeons of Canada and 12 of 13 EM programs of The College of Family Physicians of Canada, 82 (37.

Biatrial reduction plasty with reef imbricate technique as an adjunct to maze procedure for permanent atrial fibrillation associated with giant left atria.

Success of the modified maze procedure after valvular operation with giant atria and permanent atrial fibrillation (AF) remains suboptimal. We report an aggressive approach for these patients utilizing biatrial reduction plasty with a reef imbricate suture technique concomitantly with valvular and maze procedure for AF. From January 1999 to December 2006, 122 consecutive Chinese patients with permanent AF and biatrial enlargement who required mitral valve+/-tricuspid valve (TV) surgery underwent aggressive left atrial reduction combined with radiofrequency bipolar full maze procedure.

VMA21 deficiency causes an autophagic myopathy by compromising V-ATPase activity and lysosomal acidification.

X-linked myopathy with excessive autophagy (XMEA) is a childhood-onset disease characterized by progressive vacuolation and atrophy of skeletal muscle. We show that XMEA is caused by hypomorphic alleles of the VMA21 gene, that VMA21 is the diverged human ortholog of the yeast Vma21p protein, and that like Vma21p it is an essential assembly chaperone of the V-ATPase, the principal mammalian proton pump complex. Decreased VMA21 raises lysosomal pH, which reduces lysosomal degradative ability and blocks autophagy.

Does the trainee's level of experience impact on patient safety and clinical outcomes in coronary artery bypass surgery?

Background: There is a relative dearth of information on how the resident's level of training affects patient outcomes in cardiac surgery. We designed this study to determine if there were any significant differences in patient demographics and clinical outcomes of coronary artery bypass procedures (CABG) performed by residents of PGY 4/lower, residents of PGY 5/6, fellows, or consultants.

Methods: Standardized preoperative, intraoperative, and postoperative variables were prospectively collected and analyzed on 2906 isolated CABG procedures, performed between July 1999 and March 2006 with the primary surgeon prospectively classified as PGY4/lower, PGY5/6, fellow, and consultant.

Sequence variants within exon 1 of MECP2 occur in females with mental retardation.

A new splice variant of the Rett syndrome gene, MECP2, was recently identified, that includes coding sequence from exon 1, and is the predominant transcript in the central nervous system. This sequence encodes polyalanine and polyglycine stretches within the N-terminal portion of MeCP2, and may confer novel functional properties to the protein. We screened autism, mental retardation (MR), and control populations for sequence variation within this region, and identified variation in approximately 1% of MR cases screened (N = 1,410).

A prospective randomized trial of endoscopic versus conventional harvesting of the saphenous vein in coronary artery bypass surgery.

Objectives: Our objectives were (1) to determine whether minimally invasive endoscopic harvesting of the saphenous vein reduces morbidity due to postoperative wound infection and pain with improved cosmetic results and mobilization as compared with the conventional technique and (2) to compare the histologic properties of the saphenous veins harvested conventionally and endoscopically.

Methods: One hundred forty-four patients undergoing coronary artery bypass grafting were randomized to have vein harvesting performed by either the conventional (n = 72) or an endoscopic (n = 72) minimally invasive technique.

Results: Vein harvest time (open leg wound time) was significantly reduced in the endoscopic group (27.