Abstract Background Infective endocarditis is a rare complication of aortic valve replacement with high morbidity and mortality. Data of randomized trials comparing the incidence and outcomes between surgical (SAVR) and transcatheter aortic valve replacement (TAVR) are scarce. Purpose To compare the frequency, timing and outcomes of infective endocarditis after TAVR and SAVR from 3 prospective randomized trials and examine the clinical outcomes. Methods Clinical data from the CoreValve Pivotal High-Risk, the intermediate-risk SURTAVI and the Evolut Low Risk randomized trials, which compared TAVR with a supra-annular, self-expanding transcatheter valve to SAVR, was pooled. Cases of infective endocarditis were independently adjudicated based on Duke's criteria necessitating 2 major criteria, or 1 major and 3 minor criteria, or 5 minor criteria. Baseline clinical and procedural characteristics for patients with and without endocarditis were obtained. The cumulative incidence of endocarditis through 5 years after TAVR or SAVR was determined using death as a competing risk. Kaplan-Meier estimates of all-cause mortality and the composite of all-cause mortality or stroke through 2 years were calculated for both treatment groups. Results Among 2249 TAVR patients, 12 cases of endocarditis (0.5%) were documented and among 1828 SAVR patients, 21 (1.1%) over a mean follow-up time of 2.25±1.58 years. Baseline characteristics were well-balanced between the TAVR and SAVR patients with endocarditis. The cumulative incidence of endocarditis at 5 years was significantly different between the two groups (figure). The prevalence of diabetes was significantly higher in patients with endocarditis than in those without (57.6% vs. 34.2%, p=0.005). In endocarditis patients the rate of all-cause mortality was 39.4% for TAVR patients and 67.8% for SAVR patients at 2 years (log-rank p=0.133). The rates of all-cause mortality or stroke were 55.0% for TAVR and 64.6% for SAVR patients (log-rank p=0.078). Conclusions In this pooled analysis of three randomized trials comparing TAVR with a supra-annular, self-expanding bioprosthesis to SAVR, overall rates of endocarditis were low. The cumulative incidence of infective endocarditis at 5 years was lower in the TAVR group. Mortality after endocarditis was high. Figure 1 Funding Acknowledgement Type of funding source: Private company. Main funding source(s): Medtronic

Background: The application of uniportal video-assisted thoracic surgery (VATS) for both minor and major thoracic procedures is gaining widespread use across the globe. Believing its advantages, both in superb surgical results and less morbidity, our center has the privilege to be one of the first centers in the Middle East to introduce this surgical technique into our standard practice. This study presents our initial experience using this technique in 192 procedures and demonstrates the results of postoperative pain level in a sample of 90 patients.Methods: In a retrospective study of prospectively collected data, 192 uniportal VATS procedures were analyzed between November 2013 and June 2016. The level of early post-operative pain (postoperative days 1-4) was analyzed in the first 90 cases between November 2013 and March 2015. Uniportal technique was used for a wide array of procedures: blebectomies, pleurectomies, wedge resections, anatomical major lung resections, mediastinal tumors, empyema drainage and decortications.Results: The mean age of patients was 49.6 years, and 72 patients were females (37%). Thirty-five (18.2%) patients underwent anatomical resections with conversion to thoracotomy in three patients (8%). Six (3%) patients had air leak > 4 days. The average chest drain duration was 3.25 days. The average length of stay was 4.2 days. Postoperative pain level was low in the first 4 days following the surgery and 30 days mortality was 0%.Conclusions: Uniportal VATS surgery is a safe and established technique with a minimal invasive thoracic surgery. Excellent results with minimal morbidity, short hospital stay and low postoperative pain are amongst its strong points. Thoracic surgeons experienced in thoracic surgical approaches can safely perform uniportal VATS.

Ectopic pancreas in the mediastinum is extremely rare. We are reporting on a case of a twenty two year old woman who presented to our clinic with a large cervical mass. The CT scan revealed a cystic lesion in the anterior mediastinum. The patient underwent surgical resection by cervical approach. A Cystic mass with pseudocysts, cysts and complete pancreatic tissue were found in pathology. There were no signs of pancreatitis or malignancy. No recurrence was observed after a follow up of four years. We reviewed the case reports describing this rare condition in the medical literature.We conclude that the possibility of ectopic pancreatic tissue should be included in the differential diagnosis of anterior mediastinal cystic mass, though as a remote possibility. Surgery is probably needed for the diagnosis and treatment. Posterior mediastinal pseudocyst is a different entity associated with acute pancreatitis. In those cases surgery is not recommended. Our third conclusion is that pancreatic tissue should be actively sought, if a structure resembling a pseudocyst is found in an unexpected location.VIRTUAL SLIDES:The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1849369005957671.

Enzyme replacement therapy is the standard of care for symptomatic Gaucher disease. Velaglucerase alfa is a human beta-glucocerebrosidase produced in a well-characterized human cell line. A 9-month phase 1/2 open-label, single-center trial and ongoing extension study were conducted to evaluate safety and efficacy of velaglucerase alfa. Twelve symptomatic adult type 1 Gaucher patients (intact spleens) received velaglucerase alfa (60 U/kg per infusion) during phase 1/2. An extension study was offered to patients completing the trial; step-wise dose reduction (to 30 U/kg per infusion) was instituted. Eleven patients completed phase 1/2; 10 entered the extension; 9 patients reached 39 months of extension. No drug-related serious adverse events or withdrawals, and no antibodies were observed. Home therapy was successfully implemented during the extension. Statistically significant improvements (P < .004) were noted in mean percentage change from baseline to 9 months and baseline to 48 months for hemoglobin (+19.2%, +21.7%, respectively), platelet counts (+67.6%, +157.8%, respectively), normalized liver volume (-18.2%, -42.8%, respectively), and normalized spleen volume (-49.5%, -79.3%, respectively). These significant clinical changes and safety profile led to phase 3 trials and highlight the potential of velaglucerase alfa as alternative therapy for type 1 Gaucher disease. The extension trial is registered at http://www.clinicaltrials.gov as NCT00391625.

Objective. Our large tertiary clinic for patients with Gaucher disease has used sonography as the preferred modality to monitor hepatosplenomegaly in hundreds of patients for more than 18 years. With the advent of specific enzyme replacement therapy (ERT), sonographic monitoring of changes in both hepatomegaly and the echogenicity of the hepatic tissue may highlight features that are amenable to ERT. Methods. All patients (500) seen at presentation and at annual or semiannual routine visits have undergone sonographic examinations by a single senior radiologist (I.H.-H.). Results. Thirty-nine patients (7.8%) had sonographic evidence of hepatic disease (21 male and 18 female; age range, 18-90 years); 26 (66.7%) of these received ERT, and 10 (25.6%) were splenectomized. Conclusions. Liver findings are relatively rare. Among 500 patients, there was no instance of computed tomographic findings that had not been previously shown by sonography. Radiologists should be acquainted with the variable sonographic spectrum of the Gaucher liver. If hepatic lesions are small, hyperechoic, and slowly evolving, one may surmise that they are due to Gaucher cell accumulation. However, special attention should be paid to progressive deterioration and irregularities in liver texture because other metabolic processes and cancers must be ruled out.

Background: Conduction disturbances requiring permanent pacemaker implantation after heart surgery occur in about 1.5% of patients. Early pacemaker implantation may reduce morbidity and postoperative hospital stay. We reviewed our experience with patients undergoing surgery to try and identify predictors for pacemaker requirements and patients who will remain pacemaker dependent.

BACKGROUND:The choice between a mechanical or bioprosthetic valve replacement device is not always clear, although patient age is most often the determining factor. We reviewed our experience with patients undergoing aortic valve replacement (AVR) in order to assess and compare long-term outcomes between patients receiving a mechanical valve and those receiving a bioprosthesis.METHODS:Three hundred fifty-two patients underwent AVR with or without coronary artery bypass between 1993 and 2004: 189 received a mechanical valve and 163 a bioprosthesis. Events included: late mortality, thrombo-embolic events, stroke, bleeding events, valve thrombosis, endocarditis, reoperation, and coronary catheterization.RESULTS:Patients in the bioprosthesis group were older (71 +/- 11 vs. 65 +/- 13) than in the mechanical group (p < 0.0001). There was no difference in operative mortality (6.8%) or morbidity. Follow-up (61 +/- 40 months) was available in 87%. For mechanical valves and bioprostheses, respectively: 3-, 5-, and 10-year survival was 92%, 86%, and 69% versus 90%, 86%, and 71% (p = n.s.); and event-free survival was 79%, 68%, and 41% versus 79%, 68%, and 44% (p = n.s.). Five patients (3%) in each group required re-replacement of their aortic valve (p = n.s.). Coronary artery disease requiring bypass surgery did not affect long-term survival. Age at operation and renal failure were the only predictors for late mortality.CONCLUSIONS:Survival and event-free survival are similar for patients receiving a mechanical or biological aortic valve substitute. Selection of a valve replacement device should be based on life expectancy, patient preference, ability to take anticoagulants, lifestyle, risk of bleeding, and risk of reoperation. Patient age alone should not be the determining factor.

BACKGROUND Sarcoidosis is a multisystemic disorder of unknown cause that primarily affects the lungs. The diagnosis is based on the clinical manifestations, radiological findings and histological examination. OBJECTIVES To review and illustrate the typical and atypical radiological findings of sarcoidosis in the chest. METHODS We analyzed the radiographic thoracic findings of all patients who had biopsy-proven sarcoidosis over the last 10 years. RESULTS There were 100 consecutive patients (36 men and 64 women, age 20-84 years) with an established diagnosis of sarcoidosis. Thoracic lymphadenopathy was detected in 89 patients (89%). Lung parenchyma involvement was found in 60 patients. These changes were variable and included: ground-glass attenuation (n=39), multiple small nodules (n=44) and irregular thickening of the interlobular septa (n=16). Larger nodules (1-3 cm) were identified in 12 patients and frank consolidations were seen in 12 patients. Pleural thickening with subpleural nodules was identified in 17 patients. CONCLUSIONS Sarcoidosis has a wide variety of radiological manifestations in the chest. Familiarity with the various radiographic findings is important for diagnosis and management.

BACKGROUND Pleural effusion is a common finding after coronary artery bypass surgery (CABG). We sought to determine the incidence and patient characteristics predictive of its appearance, in addition to the effect of post-operative treatment with enoxaparin. METHODS We retrospectively examined 893 patients undergoing CABG: 520 consecutive patients did not receive enoxaparin, and 373 patients received it. All early (up to 10 days) peri-operative chest radiograms were examined and graded by the amount of pleural effusion: (i) small--obliteration of the costophrenic angle; (ii) moderate-- < 50% of lung field; (iii) large-- > 50% of lung field. Patient characteristics as well as operative and post-operative parameters were analyzed in order to identify predictors for pleural effusion. RESULTS Pleural effusion was small in 415 patients (46%), moderate in 346 (39%) and large in 132 (15%). Older age, female gender and congestive heart failure were found to be predictors for pleural effusion by multi-variate analysis (p <0.05). Routine use of enoxaparin was not found to be associated with pleural effusion after CABG. CONCLUSIONS Over 50% of patients will develop a significant pleural effusion following CABG. Prophylactic treatment with enoxaparin does not increase the risk for pleural effusion.

Background: Ischemic mitral regurgitation is associated with reduced survival after coronary artery bypass surgery.Objectives: To compare long-term survival among patients undergoing coronary surgery for reduced left ventricular function and severe ischemic MR in whom the valve was repaired, replaced, or no intervention was performed.Methods: Eighty patients with severe left ventricular dysfunction and severe MR underwent coronary bypass surgery. The mean age of the patients was 65 years (range 42-82), and 63 (79%) were male. Sixty-three (79%) were in preoperative NYHA functional class III-IV (mean NYHA 3.3), and 26 (32%) were operated on an urgent/emergent basis. Coronary artery bypass surgery was performed in all patients. The mitral valve was repaired in 38 and replaced in 14, and in 28 there was no intervention. The clinical profile was similar in the three groups, although patients undergoing repair were slightly younger.Results: Operative mortality was 15% (8%, 14%, and 25% for the repair, replacement and no intervention respectively; not significant). Long-term follow up was 100% complete, for a mean of 38 months (range 2-92). Twenty-nine patients (57%) were in NYHA I-II (mean NYHA 2.3). Among the surgery survivors, late survival was improved in the repair group compared to the other groups (P < 0.05). Predictors for late mortality were non-repair of the mitral valve, residual MR, and stroke (P = 0.005).Conclusions: Patients with severe ischemic cardiomyopathy and severe MR undergoing coronary bypass surgery should have a mitral procedure at the time of surgery. Mitral valve repair offers a survival advantage as compared to replacement or no intervention on the valve. Patients with residual MR had the worst results.

BACKGROUND:Ischemic mitral regurgitation is associated with reduced survival after coronary artery bypass surgery.OBJECTIVES:To compare long-term survival among patients undergoing coronary surgery for reduced left ventricular function and severe ischemic MR in whom the valve was repaired, replaced, or no intervention was performed.METHODS:Eighty patients with severe left ventricular dysfunction and severe MR underwent coronary bypass surgery. The mean age of the patients was 65 years (range 42-82), and 63 (79%) were male. Sixty-three (79%) were in preoperative NYHA functional class III-IV (mean NYHA 3.3), and 26 (32%) were operated on an urgent/emergent basis. Coronary artery bypass surgery was performed in all patients. The mitral valve was repaired in 38 and replaced in 14, and in 28 there was no intervention. The clinical profile was similar in the three groups, although patients undergoing repair were slightly younger.RESULTS:Operative mortality was 15% (8%, 14%, and 25% for the repair, replacement and no intervention respectively; not significant). Long-term follow up was 100% complete, for a mean of 38 months (range 2-92). Twenty-nine patients (57%) were in NYHA I-II (mean NYHA 2.3). Among the surgery survivors, late survival was improved in the repair group compared to the other groups (P < 0.05). Predictors for late mortality were non-repair of the mitral valve, residual MR, and stroke (P = 0.005).CONCLUSIONS:Patients with severe ischemic cardiomyopathy and severe MR undergoing coronary bypass surgery should have a mitral procedure at the time of surgery. Mitral valve repair offers a survival advantage as compared to replacement or no intervention on the valve. Patients with residual MR had the worst results.

OBJECTIVES Massive refractory pleural effusions are uncommon in patients with systemic lupus erythematosus. Describing such a patient, the literature was reviewed to report the various therapeutic options in such cases.METHODS MEDLINE search using the terms "lupus" and "pleural effusion," inclusion of cases with refractory massive effusions with emphasis on treatment.RESULTS Only 10 such cases (including the patient described here) were reported in the English literature over the past 25 years. Those 10 patients suffered symptoms related to pleural effusion for a long period of time until resolution, ranging between 2 months to 2.5 years (median 6 months). During that period of time they underwent multiple fluid aspirations. Seven different types of therapy were reported in these case descriptions. They can be divided into 2 major groups: systemic therapy (immunosuppressive therapy, plasmapheresis, and intravenous immunoglobulin) and local therapy (intrapleural steroid injections, pleurodesis with talc or tetracycline, and pleurectomy). Pleurodesis with talc seemed to be the most effective treatment modality.CONCLUSIONS Due to the small number of reported patients, the best type of intervention is uncertain. When refractory pleural effusion is part of lupus exacerbation, the treatment of choice would be systemic, such as immunosuppressive therapy with high-dose steroids and cyclophosphamide. Intravenous immunoglobulin may also be considered. Local measures such as talc pleurodesis should be employed if systemic measures fail, or when pleural effusion is the only manifestation of lupus. Semin Arthritis Rheum 34:744-749 (c) 2005 Elsevier Inc. All rights reserved.

One hundred and twenty two patients with ischemic mitral regurgitation grade 3-4 underwent surgery for mitral valve repair with annuloplasty ring. Patient age was 65 +/- 10 and 89 (73%) were male. Eighty-six (70%) were in NYHA class III-IV, 77 (63%) had reduced LV function, and mean MR grade (+/- SD) was 3.8 +/- 0.4. One hundred and thirteen operative survivors were followed for a mean of 33 months. Overall MR grade improved and was 1.6 +/- 1. Twenty-eight patients (25%) remained with MR grade 3-4. Survival was 70% at 5 years. Predictors for late mortality were LV function, NYHA class IV, and older age. Residual MR did not emerge as a predictor for late mortality.

Chemokines play a pivotal role in homeostatic and inflammatory migration of naive and activated natural killer (NK) subsets. Recent studies have shown that aberrant chemokine receptor expression on certain immune cells underlies the pathogenesis of clinical conditions in which recruitment of such cells is altered. Progressive accumulation of activated NK cells, subsequently resulting in the formation of chronic granulomatous lesions in the respiratory tract and the skin, has been described in a number of patients with transporter associated with antigen processing 2 (TAP-2) deficiency in the later stages of disease. Therefore, the goal of the present study was to elucidate whether the dysregulation of chemoattracting receptor expression on NK cells could explain abnormal navigation of these cells in TAP-2 deficiency. High-throughput proteomic comparison, followed by verification with flow cytometry, revealed that chronically activated NK cells derived from 3 newly identified patients with TAP-2 deficiency consistently expressed aberrant levels of CC chemokine receptor 2 (CCR2) chemokine receptor in vitro and in vivo. This expression pattern translated into specific responsiveness of chronically activated NK cells derived from patients with TAP-2 deficiency to multiple ligands of CCR2. Moreover, the in vivo elevated levels of interleukin-2 (IL-2) and monocyte chemoattractant protein-1 (MCP-1) detected in serum and bronchoalveolar lavage samples derived from these patients highlight the potential involvement of the CCR2 pathway in aberrant NK-cell retention at chronic inflammatory sites.

remained stable. No further fluid was suctioned from the trachea. The estimated blood loss was 15 ml. Recovery was uneventful compared with the preceding event. He was ventilated on oxygen and reversed, awakened after three minutes and was extubated after five minutes and transferred to an incubator and spent an hour in the Recovery Room. The hemoglobin was found to be 8 gÆdl )1 and blood was transfused in the immediate postcatheter period. Oxygen was required to keep the saturation at the precatheter level of 85% over the first night, but he was discharged on the second hospital day. Cardiac catheterization of small children has a mortality of approximately 0.6%. There is a higher mortality in children with congenital cyanotic heart disease, and this may be related to the fact that they are also more likely to be in heart failure and have abnormal anatomy. The signs of heart failure in infants have a different pattern to adults. Lung signs are not common; feeding difficulties, long feeding time, sweating during feeding are prominent. The liver may be palpable. Cardiovascular signs, tachycardia and gallop may be difficult for anesthetists to identify. The child, we describe, was in treated heart failure. Uncommon events may occur during catheterization which are life threatening and our policy is that consultant anesthetists should give these anesthetics. We have identified a previous case report in the European literature (1). Surprisingly, that child also had a worsened cough for the first postcatheter day and no other sequelae identified. Goldstein reported another from America (2). We have also found a case report where the flow is in the opposite direction to our case, that is, from a bronchogram to a vertebral embolus (3). We thought that an account of inadvertent bronchography during catheterization with successful conservative management would be of interest, considering intuitively one would expect the results to be disastrous. A Almenrader F R C A Oliver Dearlove F R C A Royal Manchester Children’s Hospital, Manchester M27 4HA, UK (email: o.dearlove@man.ac.uk)