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A middle-aged woman with rheumatoid arthritis presented with treatment refractory pericarditis. Symptoms persisted despite escalation of immunosuppression and she had recurrent admissions for heart failure. Imaging revealed minimal pericardial effusion and a thickened pericardium. Invasive hemodynamics confirmed constrictive physiology and a pericardiectomy was required. Pathology confirmed cholesterol pericarditis, a rare condition of inflammatory cholesterol deposits within the pericardium. Previous reports describe moderate-to-large volumes of gold-coloured pericardial fluid. This case illustrates that cholesterol pericarditis can present with minimal pericardial effusion and rapidly progress to pericardial constriction.

Objective Aortic valve replacement is the standard of care for severe, symptomatic aortic valve stenosis (AS); however, anatomy or preexisting comorbidities may preclude conventional or alternative transcatheter approaches. Aortic valve bypass (AVB) may be performed as a salvage procedure for the relief of symptomatic aortic stenosis in patients who are not suitable candidates for aortic valve replacement. Methods At our institution, seven patients underwent AVB using the Correx automated coring and apical connector system. All patients had severe AS with New York Heart Association functional class 3 symptoms and were not candidates for conventional or transcatheter approaches. Via a left anterolateral thoracotomy to access the descending aorta and left ventricular apex, we used the Correx system (Correx, Waltham, MA USA) to anastomose a valve conduit to the left ventricular apex proximally and the descending aorta distally. Three patients required cardiopulmonary bypass. Results In all seven patients, the automated coring and apical connector was successfully deployed. There were two in-hospital deaths in this series. Immediately postoperatively and at 3 months, there was a significant reduction in mean and peak valve gradients, and all surviving patients performed at New York Heart Association functional class 1. Conclusions Aortic valve bypass seems to be an acceptable alternative for the treatment of severe AS in high-risk patients who are not candidates for aortic valve replacement. The Correx automated system may improve the clinical applicability and surgical repro-ducibility of AVB in appropriately selected patients in which conventional or transcatheter aortic valve replacement is not a feasible options.

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). Intraoperative pulmonary vein isolation was confirmed by the inability to pace the heart via the pulmonary veins after ablation. All patients received postoperative β-blocker. Results There was no difference in the incidence of POAF in the treatment group who underwent adjuvant pulmonary vein ablation (group A; 37.1%) compared with the control group who did not (group B; 36.1%) ( P = .887). There were no differences in postoperative inotropic support, antiarrhythmic drug use, need for oral anticoagulation, and complication rates. The mean length of postoperative hospital stay was 8.2 ± 6.5 days in the ablation group and 6.7 ± 4.6 days in the control group ( P Conclusion Adjuvant pulmonary vein isolation does not decrease the incidence of POAF or its clinical impact but increases the mean length of stay in the hospital. The mechanism of POAF does not appear to depend on the pulmonary veins.

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. There is no benefit to the early use of high-frequency oscillatory ventilation (HFOV) in patients with moderate/severe ARDS, although it may be considered as rescue therapy. Further investigations of novel methods of bedside monitoring of mechanical ventilation may help identify the optimal ventilatory strategy.

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.We retrospectively reviewed the clinical outcomes of consecutive patients who underwent ASD patch repair at our institution between 2002 and 2012. We compared in-hospital/30-day mortality, postoperative complications, length of stay in hospital and in the intensive care unit and blood product requirements between patients who underwent right mini-thoracotomy (MT) and those who underwent conventional sternotomy.During the study period, 73 consecutive patients underwent ASD patch repair at our institution: 51 (age 47 ± 16 yr, 66.7% women) in the MT group and 22 (age 46 ± 21 yr, 59.1% women) in the sternotomy group. In-hospital mortality was similar between the 2 groups (MT 0% v. sternotomy 4.5%, p = 0.30). There were no significant differences in any postoperative complications or blood product requirements. No patients in the MT group suffered stroke, retrograde aortic dissection or leg ischemia. Mean intensive care unit (MT 1.2 ± 1.2 d v. sternotomy 1.7 ± 2.2 d, p = 0.26) and hospital length of stays (MT 5.1 ± 2.2 d v. sternotomy 6.3 ± 3.6 d, p = 0.17) were similar between the groups; however, there was a trend toward fewer patients requiring prolonged hospital stays (10 d) in the MT group (3.9% v. 18.2%, p = 0.06).Repair of ostium secundum and sinus venosus ASD can be performed safely via MT endoscopic approach with similar outcomes as sternotomy. Patient preference for a more cosmetically appealing incision may be considered without concern of compromised outcomes.Des inquiétudes persistent au sujet des résultats potentiellement négatifs chez les patients soumis à une intervention de réparation de communication interauriculaire (CIA) minimalement effractive. Nous avons comparé les résultats cliniques chez des patients adultes soumis à une réparation de CIA par approche endoscopique minimalement effractive ou par sternotomie classique — « l’étalon-or ».Nous avons passé en revue de manière rétrospective les résultats cliniques chez des patients consécutifs qui ont subi un traitement d’occlusion de leur CIA dans notre établissement, entre 2002 et 2012. Nous avons comparé la mortalité en cours d’hospitalisation et à 30 jours, les complications postopératoires, la durée des séjours à l’hôpital et aux soins intensifs et le recours aux produits sanguins chez les patients selon qu’ils avaient subi une mini-thoracotomie (MT) ou une sternotomie classique.Durant la période de l’étude, 73 patients consécutifs ont subi un traitement d’occlusion de leur CIA dans notre établissement : 51 (âge 47 ± 16 ans, 66,7 % femmes) dans le groupe MT et 22 (âge 46 ± 21 ans, 59,1 % femmes) dans le groupe sternotomie. La mortalité perhospitalière a été similaire entre les 2 groupes (MT 0 % c. sternotomie 4,5 %,La réparation de la CIA au niveau de l’ostium secundum et du sinus veineux peut se faire de manière sécuritaire par approche endoscopique MT, avec des résultats similaires à ceux de la sternotomie. On peut tenir compte de la préférence des patients pour une incision plus acceptable au plan esthétique sans crainte de compromettre les résultats.

Introduction: Ascertaining the biological age of patients with advanced vascular disease could advance risk assessment and management. The extent to which telomeres shorten in leukocytes could be a marker of biological age because it reflects the accumulation of replication stresses imposed on leukocyte progenitors. However, because of wide, genetic variability in leukocyte telomere length (TL), a single leukocyte TL measurement does not reliably indicate telomere shortening. Hypothesis: We hypothesized that the difference in length of telomeres in “non-replicating” muscle-rich tissue and that of circulating leukocytes provides a patient-specific index of telomere shortening in patients with advanced vascular disease. Methods: TL in leukocytes, skeletal muscle, and right atrial cardiac muscle were measured from 134 patients undergoing coronary or thoracic aortic surgery, using quantitative polymerase chain reaction. Relationships between leukocyte TL or the muscle-leukocyte TL difference (ΔTL) and early post-operative outcomes were tested using Cox proportional hazard and binary logistic regression analyses. Results: Telomeres in cardiac muscle and skeletal muscle were significantly longer than those in leukocytes (p Conclusions: Right atrium-leukocyte ΔTL provides an index of telomere shortening and may inform outcomes in patients with advanced vascular disease. This two-component telomere measurement may reflect the biological age of individuals with chronic vascular disease.

Purpose The technical difficulty of performing the left ventricular apical anastomosis has limited the adoption of aortic valve bypass surgery for the treatment of aortic stenosis. We report the successful use of an automated coring and apical connector device to perform aortic valve bypass surgery. Description A 74-year-old man, with a history of prior coronary bypass surgery with patent grafts and a porcelain ascending aorta, presented with symptomatic critical aortic stenosis. Through a left anterolateral thoracotomy, a valved conduit was anastomosed to the descending thoracic aorta. The automated coring and apical connector insertion device was used to core a plug of apical myocardium and simultaneously insert an 18-mm apical connector into the left ventricular apex. Evaluation There were no procedural complications, cardiopulmonary bypass was not used, and estimated blood loss was minimal. The patient was discharged on postoperative day 5, and at 3-month follow-up demonstrated significant clinical and hemodynamic improvement. Conclusions The automated coring and apical connector insertion device facilitated the safe and effective performance of aortic valve bypass surgery.

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 ∼1% of MR cases screened (N = 1,410). No variants were identified in the autism sample (N = 401). Most of these variants occur within a trinucleotide repeat region and result in change in number of alanine or glycine residues within the repeat stretches. We suggest some of these variants may be a relatively frequent cause of non-specific MR or developmental delay. © 2006 Wiley-Liss, Inc.

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. However, the patient was stabilized on ECMO and after 5 weeks in the intensive care unit achieved complete physical and neurologic recovery. To our knowledge, this is the first reported case where ECMO has been used in a resuscitation from massive pulmonary embolism immediately post-partum, after thombolytics were administered. Here, we discuss our strategies for emergent cannulation in a suboptimal environment, management of profound bleeding and oxygenation strategies in this hostile setting. Given the potential for success and the significant life-years gained, aggressive measures, such as ECMO, should be considered in such extreme life-threatening cases.

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.

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. Left atrial dimensions were measured by TTE or TEE. There were 71 women (58.1%) and 51 men (41.9%) and their mean age was 45+/-9.5 years. Mean duration of AF was 48.4+/-21.4 months. All patients underwent left atrial reduction plasty with reef imbricate suture technique and full maze procedure. Their preoperative left atria measured 64+/-12 mm in the enlarged left atria (ELA) group and 86+/-17 mm in the giant left atria (GLA). Mitral valve replacement (MVR) combined with TV repair was performed in 102 patients (83%) while 21 patients underwent MVRs combined with aortic valve replacements (17%). Sixty-six (54%) patients required additional procedures and 61 (50%) of the patients also underwent left atrial appendage clot evacuation. Postoperative left atrial size was reduced to 49+/-8 mm (ELA) and 51+/-11 mm (GLA), respectively (P

Objectives The complexity of surgical treatment for acute type A dissection contributes to the variability in patient management. This study was designed to elucidate the contemporary practice preferences of cardiac surgeons regarding different phases of management of acute type A aortic dissection. Methods A 34-item questionnaire was distributed to all Canadian adult cardiac surgeons addressing the preoperative, intraoperative, and postoperative management of acute type A dissection. A total of 100 responses were obtained (82% of active surgeons in Canada). Outcomes were compared between high- and low-volume aortic surgeons. Results Seventy-six percent of respondents favored axillary artery cannulation. High-volume surgeons (>150 cases) were more likely to indicate a target lowest nasopharyngeal temperature more than 20°C (53% vs 25%, P = .02). The majority of surgeons (65%) recommended using selective antegrade cerebral perfusion, with a significantly greater proportion for higher-volume aortic surgeons ( P = .03). In addition, high-volume aortic surgeons were more likely to recommend aortic root replacement at smaller diameters (73% vs 55%, P = .02), to recommend more extensive distal aortic resection with routine open hemiarch anastomosis (85% vs 65%, P = .04), and to more commonly perform total arch reconstruction when needed (93% vs 77%, P = .04). In the follow-up period, frequency of serial imaging of the residual aorta was significantly higher for high-volume aortic surgeons ( P = .04). Conclusions This study identified some commonalities in practice preferences among Canadian cardiac surgeons for the management of acute type A aortic dissection. However, it also highlighted significant differences in temperature management, cerebral protection strategies, and extent of resection between high-volume and low-volume aortic surgeons.

Myocardial infarction that is attributed to native coronary artery spasm in the early postoperative phase has rarely been documented. We report three cases of postoperative myocardial infarction secondary to angiographically demonstrated coronary spasm. Native coronary artery spasm is a rare, but important cause of postoperative ischemia and infarction. Suspicious electrocardiographic changes warrant consideration of transesophageal echocardiography to detect unexpected wall motion abnormalities. Established treatments include intravenous or intracoronary infusion of nitroglycerin and calcium channel antagonists, although several new therapeutic agents may also be beneficial. Prompt coronary angiography is the only definitive modality for early diagnosis and targeted treatment.

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. This reduces cellular free amino acids, which upregulates the mTOR pathway and mTOR-dependent macroautophagy, resulting in proliferation of large and ineffective autolysosomes that engulf sections of cytoplasm, merge together, and vacuolate the cell. Our results uncover macroautophagic overcompensation leading to cell vacuolation and tissue atrophy as a mechanism of disease.