Cor Triatriatum Sinister With a Decompressing Levoatriocardinal Vein: A Rare Association.

Levoatriocardinal vein has rarely been described with cor triatriatum. We report a case of a newborn with respiratory distress who was found to have cor triatriatum sinister with a decompressing levoatriocardinal vein on transthoracic echocardiography. The pulmonary venous confluence received all pulmonary veins and drained to the proximal left atrial chamber through significant communication. Moreover, the cor triatriatum membrane separated the left atrial body into proximal and distal left atrial chambers with a restrictive opening in the membrane, causing severe flow limitation to the cardiac output, severe pulmonary arterial hypertension, and significant right ventricular dilatation. In addition, a sizeable levoatriocardinal vein decompressed the pulmonary venous confluence drained superiorly to the left innominate vein. She had a successful surgical repair by resectioning the cor triatriatum membrane and ligating the levoatriocardinal vein. The patient had improved pulmonary arterial pressures and right ventricular dimensions at the one-month follow-up. The case highlights the rare association of the levoatriocardinal vein and cor triatriatum, and its presence signifies severe obstruction at the level of cor triatriatum.

The Effectiveness of Leadership Interventions on Cardiologists' Performance Using Benchmarking as a Tool.

BACKGROUND: Aligned with the Health Sector Transformation Strategy of Saudi Vision 2030, the study analyzed the attitudes and behaviors of cardiologists toward change and identified factors that could either facilitate or hinder the success of leadership interventions. At our cardiac center, the cath lab department is at the forefront of operations, accounting for 80% of the procedures. Our team members may not be fully equipped with the necessary attitudes and behaviors to drive successful improvement projects. Therefore, our top priority is ensuring they remain productive and engaged throughout the process. This is especially crucial because 60% of our budget is allocated to the cath lab department. OBJECTIVES: The study aimed to assess the effectiveness of a leadership intervention on cardiologists' performance in terms of safety, speed, and cost. The research analyzed the behavior and attitude of cardiologists towards change and encouraged progress and collaborative learning between doctors, using benchmarking as a tool. Besides, the study sought to determine the contribution of the interventions used to overall efficiency in performing interventions. This case study focuses on four main aspects of the program. First, it aims to explore an innovative approach to improving the PSCCQ cath lab for patients. Second, it assesses the collective effort of all participants involved in the program. Third, it analyzes the program results and compares them with those of international experiences. And finally, it examines the program's potential benefits for our patients. METHODS: The study's objectives were evaluated through qualitative analysis of in-depth interviews and quantitative data analysis of three variables in the cath lab: radiation dose, time, and inventory. The reason for using mixed methods was to comprehensively understand the same concept from different angles. RESULTS: According to the study, participants improved the safety and effectiveness of our cath lab by reducing the radiation dose and its cost. The study revealed a 52% decrease in the radiation dose for diagnostic cases and an 11% decrease for interventional cases. Similarly, the cost of the radiation dose decreased by 28% for diagnostic cases and 11% for interventional cases. During the observation, it was noted that the participants were highly engaged and willing to adapt to the situation. Some even viewed it as an opportunity for personal growth and improvement in the cath lab. However, they stressed the significance of awareness as a crucial element in improving their behavior and reinforcing it as the foundation for maintaining progress. Furthermore, the study revealed that collaborative work among the participants could have been more optimal. CONCLUSION: The study concludes that implementing innovative improvements to the cath lab was a necessary yet complex undertaking. Participants were more inclined to embrace the changes when they were easily understandable and motivating. The study recommends the appointment of a change agent, the establishment of benchmarks, and the creation of a collaborative working environment between leaders and staff. Above all, the leader should support and sponsor the change to facilitate the transition at various levels.

The Burden of Neonatal Referrals on a Pediatric Cardiology Service: A Local Center Experience.

BACKGROUND: Congenital heart disease (CHD) is a common occurrence in live births, with some exhibiting critical congenital heart disease; therefore, cardiology services should be available around the clock to ensure timely diagnosis and management. This study aims to describe the workload and the need for pediatric cardiac services in a maternity hospital for newborn referrals. Moreover, the study describes the indications for neonatal cardiology consultations. METHODS: The prospective cohort study was conducted over four months, from January to April 2022, in the Prince Sultan Cardiac Center Al Qassim region of Saudi Arabia. Prince Sultan Cardiac Center's pediatric cardiology department provides cardiac services to the Maternity and Children Hospital Buraidah Al Qassim. Out of the total 2,606 live births during the study period, the cardiology team evaluated 352 neonates. Neonates less than 30 days of age who were born in the maternity hospital were enrolled in the study. The outborn babies referred from other centers as suspected congenital heart disease for whom a cardiac evaluation was done were excluded. In addition, babies assessed in the emergency room and born elsewhere were excluded. Only new consultations have been considered, excluding follow-up consultations. STATISTICAL ANALYSIS: Data about patients' demographic, clinical and echocardiographic findings were recorded on Google Forms and converted to a Google spreadsheet. The Google spreadsheet's inbuilt statistical software was used for analysis. Categorical data were presented as percentages, and numerical data as median and range. RESULTS: The cardiology team evaluated 352 neonates from 2,606 live births over four months, accounting for 13.5 per 100 live births. The median weight was 2.8 kilograms, with a 0.5-4.3 kilogram range. Males comprised 187 (53%), and females comprised 165 (47%). Moreover, full-term, preterm, and post-term accounted for 236 (67%), 113 (32%), and 3 (0.8%) of patients, respectively. The common indications for neonatal cardiac referral were respiratory distress 60 (17%), infants born to diabetic mothers 50 (14%), abnormal fetal echocardiogram 49 (13.9%), family history of abortion or neonatal death 31 (8.8%), and congenital anomalies 30 (8.5%). Systolic murmur was the commonest clinical finding that prompted cardiology referrals 82 (23.2%), followed by desaturation 38 (10.7%) and dysmorphic features 31 (8.8%). Among the congenital cardiac defects, an isolated atrial septal defect (ASD) was seen in 66 (18.5%), isolated patent ductus arteriosus in 50 (14.2%), and ventricular septal defect in 21 (5.9%). Moreover, 13 (4.4%) lesions were critical CHDs. Finally, 27 (7.6%) had a diagnosis of pulmonary hypertension. CONCLUSION: Knowing the burden of neonatal cardiac assessment on pediatric cardiology services in any maternity center may help the healthcare authorities to allocate resources and optimize the delivery of cardiac services among the neonatal population. Properly allocating pediatric cardiologists to the needed centers may optimize neonatal cardiac services. Moreover, it may decide on the number of pediatric cardiologists that need to be trained each year to meet the requirements of neonatal cardiac services.

Comparison of Demographic, Clinical, and Echocardiographic Features Between Complete and Incomplete, and Early and Late Presenters of Kawasaki Disease: A 10-Year Single-Center Experience.

INTRODUCTION:  The diagnosis of Kawasaki disease (KD) is based mainly on clinical findings and supported by laboratory tests. Complete KD fulfills the main clinical criteria, while incomplete KD includes patients with fewer main criteria and compatible laboratory or echocardiographic findings. The study compares the demographic, clinical, laboratory, and echocardiographic parameters between the complete and incomplete KD and early and late presenters. Moreover, it describes the coronary manifestations of the study population. METHODOLOGY: A retrospective review of all patients admitted with a diagnosis of KD during the period from January 2010 to September 2020 was conducted. Clinical presentation, laboratory features, echocardiographic observations, and follow-up data were examined. Moreover, the patients were further classified as early presenters (presented within 10 days of fever onset) and late presenters (presented after 10 days of disease onset). A comparison between complete and incomplete KD and early and late presenters was performed for demographic, clinical, and echocardiographic features. RESULTS: A total of 76 patients were admitted with a diagnosis of KD. The median age of presentation was 28 months, with a range of five to 144 months, and the median timing was seven days, with a range of one to 30 days. The median follow-up period was six weeks, with a range of one to 192 weeks. Complete KD was present in 38 patients (50%), and 38 (50%) had incomplete KD. Skin manifestations, oral mucosal changes, skin desquamation, conjunctivitis, and lymphadenopathy were present more in patients with complete KD than incomplete ones. Complete and incomplete diseases did not differ regarding coronary artery lesions. Of the patients, 53 (70%) presented 10 days or less after the onset of fever, and 23 (30%) presented after the 10th day of disease onset. Comparison between early and late presenters revealed significantly greater mucus membrane changes and lymphadenopathy manifestations among the early presenters and coronary artery lesions among the late presenters. CONCLUSION: The clinical features of KD should prompt early referral for evaluation, echocardiography, and early administration of intravenous immunoglobulin to prevent coronary artery complications. The complete form of Kawasaki does not have more frequent coronary artery lesions than the incomplete form. Additionally, late presenters may be at increased risk for coronary artery abnormalities than early presenters.

A Combined DNA/RNA-based Next-Generation Sequencing Platform to Improve the Classification of Pancreatic Cysts and Early Detection of Pancreatic Cancer Arising From Pancreatic Cysts.

OBJECTIVE: We report the development and validation of a combined DNA/RNA next-generation sequencing (NGS) platform to improve the evaluation of pancreatic cysts. BACKGROUND AND AIMS: Despite a multidisciplinary approach, pancreatic cyst classification, such as a cystic precursor neoplasm, and the detection of high-grade dysplasia and early adenocarcinoma (advanced neoplasia) can be challenging. NGS of preoperative pancreatic cyst fluid improves the clinical evaluation of pancreatic cysts, but the recent identification of novel genomic alterations necessitates the creation of a comprehensive panel and the development of a genomic classifier to integrate the complex molecular results. METHODS: An updated and unique 74-gene DNA/RNA-targeted NGS panel (PancreaSeq Genomic Classifier) was created to evaluate 5 classes of genomic alterations to include gene mutations (e.g., KRAS, GNAS, etc.), gene fusions and gene expression. Further, CEA mRNA ( CEACAM5 ) was integrated into the assay using RT-qPCR. Separate multi-institutional cohorts for training (n=108) and validation (n=77) were tested, and diagnostic performance was compared to clinical, imaging, cytopathologic, and guideline data. RESULTS: Upon creation of a genomic classifier system, PancreaSeq GC yielded a 95% sensitivity and 100% specificity for a cystic precursor neoplasm, and the sensitivity and specificity for advanced neoplasia were 82% and 100%, respectively. Associated symptoms, cyst size, duct dilatation, a mural nodule, increasing cyst size, and malignant cytopathology had lower sensitivities (41-59%) and lower specificities (56-96%) for advanced neoplasia. This test also increased the sensitivity of current pancreatic cyst guidelines (IAP/Fukuoka and AGA) by >10% and maintained their inherent specificity. CONCLUSIONS: PancreaSeq GC was not only accurate in predicting pancreatic cyst type and advanced neoplasia but also improved the sensitivity of current pancreatic cyst guidelines.

Prospective, Multi-Institutional, Real-Time Next-Generation Sequencing of Pancreatic Cyst Fluid Reveals Diverse Genomic Alterations That Improve the Clinical Management of Pancreatic Cysts.

BACKGROUND & AIMS: Next-generation sequencing (NGS) of pancreatic cyst fluid is a useful adjunct in the assessment of patients with pancreatic cyst. However, previous studies have been retrospective or single institutional experiences. The aim of this study was to prospectively evaluate NGS on a multi-institutional cohort of patients with pancreatic cyst in real time. METHODS: The performance of a 22-gene NGS panel (PancreaSeq) was first retrospectively confirmed and then within a 2-year timeframe, PancreaSeq testing was prospectively used to evaluate endoscopic ultrasound-guided fine-needle aspiration pancreatic cyst fluid from 31 institutions. PancreaSeq results were correlated with endoscopic ultrasound findings, ancillary studies, current pancreatic cyst guidelines, follow-up, and expanded testing (Oncomine) of postoperative specimens. RESULTS: Among 1933 PCs prospectively tested, 1887 (98%) specimens from 1832 patients were satisfactory for PancreaSeq testing. Follow-up was available for 1216 (66%) patients (median, 23 months). Based on 251 (21%) patients with surgical pathology, mitogen-activated protein kinase/GNAS mutations had 90% sensitivity and 100% specificity for a mucinous cyst (positive predictive value [PPV], 100%; negative predictive value [NPV], 77%). On exclusion of low-level variants, the combination of mitogen-activated protein kinase/GNAS and TP53/SMAD4/CTNNB1/mammalian target of rapamycin alterations had 88% sensitivity and 98% specificity for advanced neoplasia (PPV, 97%; NPV, 93%). Inclusion of cytopathologic evaluation to PancreaSeq testing improved the sensitivity to 93% and maintained a high specificity of 95% (PPV, 92%; NPV, 95%). In comparison, other modalities and current pancreatic cyst guidelines, such as the American Gastroenterology Association and International Association of Pancreatology/Fukuoka guidelines, show inferior diagnostic performance. The sensitivities and specificities of VHL and MEN1/loss of heterozygosity alterations were 71% and 100% for serous cystadenomas (PPV, 100%; NPV, 98%), and 68% and 98% for pancreatic neuroendocrine tumors (PPV, 85%; NPV, 95%), respectively. On follow-up, serous cystadenomas with TP53/TERT mutations exhibited interval growth, whereas pancreatic neuroendocrine tumors with loss of heterozygosity of ≥3 genes tended to have distant metastasis. None of the 965 patients who did not undergo surgery developed malignancy. Postoperative Oncomine testing identified mucinous cysts with BRAF fusions and ERBB2 amplification, and advanced neoplasia with CDKN2A alterations. CONCLUSIONS: PancreaSeq was not only sensitive and specific for various pancreatic cyst types and advanced neoplasia arising from mucinous cysts, but also reveals the diversity of genomic alterations seen in pancreatic cysts and their clinical significance.

The Impact of Integration of a Genetic Clinic Into a Pediatric Cardiac Unit.

BACKGROUND: Previously published studies suggest that genetic or environmental causes can be observed in 20-30% of congenital heart disease (CHD) patients, which include aneuploidy, single gene defects, pathological copy number variations, and de novo autosomal dominant and recessive inheritance. Moreover, the genetic background of childhood cardiomyopathies (CMs) has not been elucidated well. OBJECTIVE: The study highlights the value of genetic assessment in diagnosing and family counseling for CHD and pediatric CM patients referred to the genetic clinic in a pediatric cardiology department. METHODS: The study involved patients less than 18 years of age attending the cardiogenetic clinic in the pediatric cardiology department between December 2010 and February 2019. The following patient categories who had genetic evaluation were included: CHD in the presence of a syndromic phenotype, patients with CHD having extracardiac congenital anomalies or delayed development, hypertrophic and dilated CM patients, patients with dilated aortic root and ascending aorta, significant CHD in siblings or first-degree relatives, suspected channelopathies; and interrupted aortic arch abnormalities. RESULTS: A total of 285 patients were evaluated in the cardiogenetic clinic. The mean age was 20.2 months, with a range of 0-168. Females and males constituted 153 (53.7%) and 132 (46.3%), respectively. The most common cause of referral to the genetic clinic was the presence of CM (N=134 (46.3%)): hypertrophic CM in 24% and dilated CM in 20% of cases. Seventy-six patients (26.7%) had positive genetic results. The most common genetic abnormality was familial infantile hypertrophic CM-causing gene ELAC2 in 19 (23.5%) cases. CONCLUSION: It may be beneficial for any pediatric cardiology unit to provide an established genetic clinic. Using a genetic clinic will enhance understanding of CHD pathophysiology, family education, and genetic counseling. Agreement on a well-written protocol and the way forward to specify what congenital heart conditions require genetic investigation should be clarified.

Can Echocardiography Aid in Diagnosing Vascular Rings?

Even though cardiac computed tomography and magnetic resonance imaging are the gold standard for evaluating the aortic arch in the context of vascular rings in children, echocardiography is usually the first-line modality. The echocardiographic evaluation of the aortic arch in the context of vascular rings in children has received little attention. This article details the step-by-step echocardiographic assessment of the aortic arch in vascular ring patients.

Radiation Dose Exposure to Patients During Transcatheter Patent Ductus Arteriosus Closure via the Arterial Route.

Objective This study aims to evaluate the radiation dose for transcatheter patent ductus arteriosus (PDA) via the arterial route and compare it with previously published benchmarks. Background Exposure to radiation in the catheterization lab can cause skin injury and cancer in the long run, especially in pediatric patients with complex heart conditions, which necessitate serial catheterizations. Therefore, measuring the patient radiation dose and establishing a benchmark for each cardiac interventional procedure is essential. Material and methods In this prospective study, 34 patients with transcatheter patent ductus arteriosus closure via an arterial route were included. Patients who had silent PDAs, no left heart dilatation and PDA size of less than 2mm had PDA closed via an arterial route. All the study group patients received an Amplatzer duct occluder II-additional size device (St. Jude Medical Corp, St. Paul, MN) using biplane flat-panel fluoroscopy equipment adjusted in accordance with the pediatric parameters. Patients' dose area product, air kerma and fluoroscopy time were recorded in the catheterization lab and finally compared with internationally published reference data. Results Of 73 patients who had transcatheter patent ductus arteriosus closure between April 2021 to December 2021, 34 patients who had a PDA closure via an arterial route were enrolled. The median age and weight were 11.5 (4-168) months and 10.5 (6-31) kg. Twenty-one (61.8%) were males, and 13 (38.2%) were males. The median radiation dose parameters were as follows: air kerma 11 (3-42) milliGray, dose area product 131 (33-443) microGray per m2, median dose area product indexed to weight 12 (1-48) microGray m2 per kg, fluoroscopy time 2 (2-4) min and frame rate 15 (7.5-15) frames per second. Due to many factors, our radiation dose parameters were less than internationally published reference values for transcatheter PDA closure. Conclusion Patient selection, detailed pre-catheterization echocardiography and procedure planning are essential for accomplishing device closure of PDA with a significant reduction in radiation dose. Hemodynamic assessment in the catheterization lab is unnecessary for most PDA patients. Additionally, a next-generation imaging platform equipped with flat-panel detectors and adjusted for pediatric settings and a fluoro recording option can be used to reduce radiation exposure.

Catheter Closure of Clinically Silent Patent Ductus Arteriosus Using the Amplatzer Duct Occluder II-Additional Size: A Single-Center Experience.

Objectives Transcatheter closure is the treatment of choice for most patent ductus arteriosus (PDA) in infants, children, and adults. However, there is a controversy regarding transcatheter closure of clinically silent PDAs. Some authors favor device closure to eliminate the lifelong risk of infective endarteritis while others recommend avoiding PDA closure in such patients. The study describes our experience of closing the silent PDAs using the Amplatzer duct occluder II-additional size (ADO II-AS) (St. Jude Medical Corp, St. Paul, MN). Materials and methods From April 2018 through March 2021, 52 consecutive pediatric patients aged 18 years and less with clinically silent PDA who had transcatheter closure at our center were enrolled. Patients were excluded if they had clinically detected PDAs; had surgical ligation of PDA with no residual shunt; had left heart dilatation on echocardiography; or moderate-sized PDAs closed with ADO II-AS. In addition, patients with an innocent murmur or murmur due to an associated lesion were included. This study was retrospective, and all of the 52 patients underwent PDA device closure using ADO II-AS. Results Fifty-two consecutive patients were enrolled with a median age of 17 months, range (97-2.5) 94.5 months. Mean weight was 11.29 kilogram, range (24.8-3.5) 21.3 kilogram, and mean follow-up was 13.5 months, range (29-0) 29 months. Thirty-one (59.6%) were females, and 21 (40.4%) were males. The mean procedure time was 30.6 min, range (60-10) 50 min, and mean fluoroscopic time was 5.5 min, range (28-1.7) 26.3 min. The mean volume of contrast given was 9.1 milliliter, range (30-4) 26 milliliter. Forty-five (45; 88.2%) patients had immediate closure of PDA. No patients had anesthetic or vascular complications; however, two patients had procedural complications. Device placement was unsuccessful in one patient with Downs syndrome. The mean follow-up for our patients was 13.5 months, range (29-0) 29 months; the patients were asymptomatic at the follow-up, and none of the patients had any residual leak. None of the patients showed coarctation or left pulmonary artery stenosis at the latest follow-up. Conclusion The usefulness of catheter-based therapy for silent PDA is less well-established by current evidence. Further studies are needed to justify the intervention solely based on the premise that the silent duct is a substrate for infective endarteritis; however, our reason to close silent PDA was to do so primarily because of social reasons. This study found that device closure of silent PDA is safe and effective using an ADO II-AS device with minimal risk of embolization and a low residual shunt rate. Coils have been used to close small PDAs, however, with higher rates of embolization and device malpositioning. We believe ADO-II AS offers an advantage of safety and efficacy over coils. In addition, the study highlights the advantage of using an ADO II-AS device, which can be delivered via a four French delivery system with no arterial complications.

Safety and efficacy of EUS-guided coil and glue injection for the primary prophylaxis of gastric variceal hemorrhage.

BACKGROUND AND AIMS: Gastric variceal bleeding (GVB) is associated with high morbidity and mortality. EUS-guided coil and cyanoacrylate (CYA) injection (EUS-CCI) has been shown to be an effective therapy in acute bleeding and secondary prophylaxis; however, there is a paucity of data on primary prophylaxis. METHODS: In this single-center observational study, adult patients with high-risk gastric varices (GV; size >10 mm or cherry red spot) without prior bleeding underwent EUS-CCI for the primary prophylaxis of GVB between June 2009 and December 2019. The primary outcome was post-treatment GVB. RESULTS: Eighty patients with a mean variceal size of 22.5 ± 9.4 mm and a mean length of follow-up of 3.0 ± 2.4 years were included. The etiology of portal hypertension was cirrhosis in 71 patients (88.7%) and noncirrhotic in 9 (11.3). The mean model for end-stage liver disease score was 12.3 ± 3.7 in patients with cirrhosis. The mean coil number was 1.5 (range, 1-3) and mean glue volume injected 2 mL (range, .5-5). Technical success was achieved in 100%, 96.7% had EUS confirmation of GV obliteration, and 67.7% were obliterated with 1 treatment session. Post-treatment GVB occurred in 2 patients (2.5%) and adverse events in 4 (4.9%). No deaths related to GVB occurred, and emergent transjugular intrahepatic shunts were not needed during the follow-up period. CONCLUSIONS: In patients with high-risk GV, EUS-CCI for primary prophylaxis is highly effective at preventing GVB with a low rate of adverse events. Primary prophylaxis of high-risk GV with coil and CYA glue injection should be considered in centers with the appropriate expertise.

Effectiveness of combination endoscopic therapy for colonic anastomotic leaks.

BACKGROUND: Colonic anastomotic leaks are associated with significant morbidity and mortality. Whereas small case series suggest that fully covered self-expandable metal stents (FCSEMS) are effective, no larger studies have examined the impact of combination endoscopic therapy on colonic anastomotic leaks. METHODS: Our retrospective cohort study reviewed 51 patients undergoing endoscopic therapy for colonic anastomotic leaks between 2011 and 2018. Patients receiving combination therapy involving FCSEMS plus local closure (n = 24) were compared with patients receiving FCSEMS alone (n = 18) or endoscopic suturing alone (n = 9). The primary outcomes were technical and clinical success (resolution of leak, removal of percutaneous drains, avoidance of surgical reoperation, and reversal of temporary diversion). RESULTS: Clinical success was achieved in 55 % of patients. Clinical success was achieved in 18/24 patients (75 %) with combination therapy compared with 6/18 patients receiving FCSEMS alone (33 %, adjusted risk ratio [RR] 2.16, 95 % confidence interval [CI] 1.10 - 4.24; P = 0.02) and 4 /9 patients undergoing endoscopic suturing alone (44 %, RR 1.91, 95 %CI 0.84 - 4.31; P = 0.10). Stent migration occurred in 40 % of patients. CONCLUSIONS: This large series demonstrates that combination therapy was associated with a higher rate of clinical success, and future prospective studies are warranted.

Impact of observational training on endoscopic mucosal resection outcomes and competency for large colorectal polyps: single endoscopist experience.

Background and study aims Endoscopic mucosal resection (EMR) is standard treatment for large colorectal polyps. However, it is a specialized technique with limited data on the effectiveness of training methods to acquire this skill. The aim of this study was to evaluate the impact of observational training on EMR outcomes and competency in an early-stage endoscopist. Patients and methods A single endoscopist completed comprehensive EMR training, which included knowledge acquisition and direct observation of EMR cases, and proctored supervision, during the third year of gastroenterology fellowship. After training, EMR was independently attempted on 142 consecutive, large (i. e., ≥ 20 mm), non-pedunculated colorectal polyps between July 2014 and December 2017 (mean age 61.7 years; mean polyp size 30.4 mm; en-bloc resection 55 %). Surveillance colonoscopy for evaluation of residual neoplasia was available for 86 % of the cases. Three primary outcomes were evaluated: endoscopic assessment of complete resection, rate of adverse events (AEs), and rate of residual neoplasia on surveillance colonoscopy. Results Complete endoscopic resection was achieved in 93 % of cases, the rates of AEs and residual neoplasia were 7.8 % and 7.3 %, respectively. The rate of complete resection remained stable (at 85 % or greater) with increasing experience while rates of AEs and residual neoplasia peaked and decreased after 60 cases. Conclusions An early-stage endoscopist can acquire the skills to perform effective EMR after completing observational training. At least 60 independent EMRs for large colorectal polyps were required to achieve a plateau for clinically meaningful outcomes.

Placement of lumen-apposing metal stents to drain pseudocysts and walled-off pancreatic necrosis can be safely performed on an outpatient basis: A multicenter study.

BACKGROUNDS AND OBJECTIVES: No study on the use of lumen-apposing fully covered self-expanding metal stent (LAMS) to drain pancreatic fluid collections (PFCs) has evaluated outcomes of patients in the outpatient setting. The objective of this multicenter study was to evaluate the clinical outcomes, success rate, and adverse events of the LAMS for endoscopic ultrasound (EUS)-guided transmural drainage of patients with symptomatic PFCs on an inpatient versus an outpatient basis. METHODS: This was a multicenter, retrospective study conducted at 4 tertiary care centers. RESULTS: We identified eighty patients with PFCs in whom EUS-guided transmural drainage using the LAMS was performed. The mean age of the patients was 53.1 years old. Mean size of the PFC was 11.8 ± 5.1 cm. A total of 33 patients had PFCs drained in an outpatient setting while 47 patients underwent PFC drainage as inpatients. The overall technical success (ability to access and drain a PFC by placement of transmural stents) was 98.7% (79 patients). There was no statistically significant difference in the technical success rate between the inpatient and outpatient groups (100% vs. 98%, respectively, P = 1). There was no significant difference in resolution of PFCs in the inpatient and outpatient groups (91% vs. 87% respectively; P = 1). The number of procedures required for PFC resolution was significantly lower in the inpatient group as compared to the outpatients (2.3 vs. 3.1 respectively, P = 0.025). Procedure-related adverse events were significantly lower in the inpatient group compared to the outpatient group (P < 0.01). There was no significant difference in the 2 groups in terms of development of adverse events requiring endoscopic reintervention within 30 days of initial stent placement (P = 0.69). CONCLUSION: This study shows that LAMS placement for PFCs can be performed safely on an outpatient basis with overall technical and clinical outcomes that are comparable to those seen in inpatients.

Double-outlet left atrium: Successful repair of an extremely rare anomaly.

The clinical detail, echocardiographic features, angiographic evaluation, and surgical repair of double-outlet left atrium (LA) in a 2-year-old boy are discussed. In a solitus position and normally connected systemic and pulmonary venous drainage setting, the only outlet of the right atrium was a secundum atrial septal defect. The LA thus received the entire venous return and then drained into both ventricles through a common atrioventricular valve.

Endoscopic ultrasound-guided biliary access versus precut papillotomy in patients with failed biliary cannulation: a retrospective study.

Background and aims Precut papillotomy is widely used after failed biliary cannulation. Endoscopic ultrasound (EUS)-guided biliary access techniques are newer methods to facilitate access and therapy in failed cannulation. We evaluated the impact of EUS-guided biliary access on endoscopic retrograde cholangiopancreatography (ERCP) success and compared these techniques to precut papillotomy. Patients and methods We retrospectively compared two ERCP cohorts. One cohort consisted of biliary ERCPs (n = 1053) attempted in patients with native papillae and surgically unaltered anatomy in whom precut papillotomy and/or EUS-guided biliary access were routinely performed immediately after failed cannulation. This cohort was compared with a similar ERCP cohort (n = 1062) in which only precut papillotomy was available for failed cannulation. The following outcomes were compared: conventional cannulation success, rates of attempted advanced access techniques (precut or EUS), precut success, EUS-guided biliary access success, and ERCP failure rates. Results Although conventional cannulation success, rates of attempted advanced access technique (precut or EUS), and precut success were similar, the ERCP failure rate was lower when both EUS-guided biliary access and precut were available (1.0 % [95 % confidence interval (CI) 0.4 - 1.6]), compared with when only precut was possible for failed access (3.6 % [95 %CI 2.5 - 4.7]; P < 0.001). Success for EUS-guided biliary access (95.1 % [95 %CI 89.7 - 100]) was significantly higher than for precut (75.3 % [95 %CI 68.2 - 82.4]; P < 0.001), and mainly due to superiority in malignant obstruction (93.5 % vs. 64 %; P < 0.001). Conclusions EUS-guided biliary access decreases the rate of therapeutic biliary ERCP failure. Our results support the use of EUS-guided biliary access to optimize single-session ERCP success. In experienced hands, these techniques appear as effective, if not more so, than precut papillotomy.

A randomized controlled cross-over trial and cost analysis comparing endoscopic ultrasound fine needle aspiration and fine needle biopsy.

BACKGROUND AND STUDY AIMS: Techniques to optimize endoscopic ultrasound-guided tissue acquisition (EUS-TA) in a variety of lesion types have not yet been established. The primary aim of this study was to compare the diagnostic yield (DY) of endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) to endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) for pancreatic and non-pancreatic masses. PATIENTS AND METHODS: Consecutive patients referred for EUS-TA underwent randomization to EUS-FNA or EUS-FNB at four tertiary-care medical centers. A maximum of three passes were allowed for the initial method of EUS-TA and patients were crossed over to the other arm based on on-site specimen adequacy. RESULTS: A total of 140 patients were enrolled. The overall DY was significantly higher with specimens obtained by EUS-FNB compared to EUS-FNA (90.0 % vs. 67.1 %, P = 0.002). While there was no difference in the DY between the two groups for pancreatic masses (FNB: 91.7 % vs. FNA: 78.4 %, P = 0.19), the DY of EUS-FNB was higher than the EUS-FNA for non-pancreatic lesions (88.2 % vs. 54.5 %, P = 0.006). Specimen adequacy was higher for EUS-FNB compared to EUS-FNA for all lesions (P = 0.006). There was a significant rescue effect of crossover from failed FNA to FNB in 27 out of 28 cases (96.5 %, P = 0.0003). Decision analysis showed that the strategy of EUS-FNB was cost saving compared to EUS-FNA over a wide range of cost and outcome probabilities. CONCLUSIONS: RESULTS of this RCT and decision analysis demonstrate superior DY and specimen adequacy for solid mass lesions sampled by EUS-FNB.

Feasibility of nonradiation EUS-based ERCP in patients with uncomplicated choledocholithiasis (with video).

BACKGROUND AND AIMS: ERCP inherently involves radiation exposure. Nonradiation ERCP has been described in pregnancy. Theoretically, the same techniques could be applied to the general population. We prospectively assessed the feasibility of nonradiation, EUS-based ERCP in nonpregnant patients with choledocholithiasis. METHODS: Consecutive patients referred for ERCP for choledocholithiasis were recruited over a 1-year period. Patients providing study consent underwent the following procedural protocol. First, EUS was performed to verify the presence, size, and number of stones. Second, biliary cannulation was attempted without fluoroscopy for a maximum of 10 minutes. Selective cannulation was based on deep insertion with visible bile in the catheter on aspiration. Third, for stone removal, sphincterotomy was performed and stones were removed using a basket or balloon. The number of stones exiting the papilla was matched to the number seen on EUS. Finally, once the duct was deemed clear by the endoscopist, a final occlusion cholangiogram and ductal sweep served as the reference standard for confirmation of stone clearance. RESULTS: Nonradiation ERCP was attempted in 31 patients. Cannulation without fluoroscopy was successful in 26 patients (84%). Complete stone removal without fluoroscopy was achieved in all 26 of these cases. The 5 patients with failed nonfluoroscopic cannulation required double guidewire (n = 2) or precut papillotomy (n = 3) for deep biliary access and subsequent stone clearance. One patient who required precut papillotomy for access developed moderate post-ERCP pancreatitis (3%). CONCLUSIONS: Nonradiation, EUS-based ERCP for uncomplicated choledocholithiasis appears to be successful and safe. (Clinical trial registration number: NCT01678391.).

EUS-guided treatment of gastric fundal varices with combined injection of coils and cyanoacrylate glue: a large U.S. experience over 6 years (with video).

BACKGROUND AND AIMS: Conventional endoscopic treatment of gastric fundal varices (GFV) with cyanoacrylate (CYA) glue may be complicated by embolization and rebleeding. We evaluated the long-term outcomes of EUS-guided injection of coils and CYA glue for therapy of GFV. METHODS: A retrospective chart review of patients treated for GFV was performed. The main outcomes measured were hemostasis, obliteration on surveillance EUS, post-treatment bleeding rate, and adverse events. RESULTS: From March 2009 to 2015, 152 patients with GFV were treated. Seven (5%) had active hemorrhage, 105 (69%) had recent bleeding, and 40 (26%) were treated for primary prophylaxis. Treatment was technically successful in 151 patients (>99%). Mean number of coils was 1.4 (range, 1-4 coils), and mean volume of CYA was 2 mL (range, 0.5-6). Follow-up was available for 125 patients with treated GFV (mean, 436 days; range, 30-2043). Among 100 patients with follow-up EUS examinations, complete obliteration (on Doppler study) of GFV was confirmed in 93 (93%). Post-treatment bleeding from obliterated GFV occurred in 3 of 93 patients (3%). Twenty-five patients who had clinical and/or EGD follow-up had 3 post-treatment bleeding episodes after a median follow-up of 324 days (range, 41-486). Among the 40 patients treated for primary prophylaxis, 28 underwent follow-up EUS and 27 (96%) had confirmed obliteration. Mild postprocedure abdominal pain occurred in 4 of 125 patients (3%), and clinical signs of pulmonary embolization were seen in 1 patient (1%). Another 4 of 125 patients (3%) presented with minor delayed upper GI bleeding from coil/glue extrusion. CONCLUSIONS: EUS-guided combined coil and CYA glue injection of high-risk GFV appears to be highly effective for hemostasis in active bleeding and primary and secondary bleeding prophylaxis. Once obliteration was achieved, post-treatment bleeding from GFV occurred in only 3% during long-term follow-up. Combination therapy appears safe and may reduce the risk of CYA embolization.

EUS-guided drainage of peripancreatic fluid collections and necrosis by using a novel lumen-apposing stent: a large retrospective, multicenter U.S. experience (with videos).

BACKGROUND AND AIMS: EUS-guided drainage of peripancreatic fluid collection (PFC) (pancreatic pseudocyst [PP] or walled-off necrosis [WON]) by using a novel lumen-apposing, fully covered, self-expandable metal stent (LAMS) has been promising, but few of these data are from the United States. The aim of this study was to evaluate clinical outcomes and safety of EUS-guided drainage of pancreatic pseudocysts and WON by using the LAMS. METHODS: We conducted a multicenter, retrospective study on 82 patients with symptomatic PFC who underwent EUS-guided drainage by using the LAMS at 4 U.S. tertiary care centers. Outcomes evaluated included successful placement of the LAMS, the number of patients in whom complete resolution of PPs or WON was achieved, the number of procedures performed per patient to achieve PFC resolution, and adverse events. RESULTS: The mean size of the PFC was 11.8 cm. LAMSs were successfully placed in 80 patients (97.5%). Twelve patients had PP and 68 had WON. The median stent in-dwelling time was 2 months (range 1-3 months). Endoscopic debridement with the LAMS in WON was performed in 54 patients. The patency of the stent was maintained in 98.7% of the patients (77/78). There was spontaneous dislodgment of 2 LAMSs. Successful endoscopic therapy by using the LAMS was successful in 12 of 12 patients (100%) with PP compared with 60 of 68 patients (88.2%) with WON. All stents were endoscopically removed from all patients after peripancreatic fluid collection (PFC) resolution. There was 1 PFC recurrence during the 3-month median follow-up period. Procedure-related adverse events occurred in 8 patients (9.8%), and included stent maldeployment (n = 2), and self-limited bleeding (n = 6). In 1 patient with stent maldeployment gastric perforation developed, and the patient underwent surgical repair. CONCLUSION: EUS-guided drainage of PFCs by using the novel LAMS has high technical and long-term success rates. Due to its ease of use, the LAMSs may simplify and streamline EUS-guided management of PFCs, particularly for the endoscopic debridement of WON, and may help in its widespread adoption as an alternative to surgery.