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Background: Lymphatic disorders such as protein-losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers., Methods and Results: A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty-one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178 - 313) cardiopulmonary bypass procedures per year. Chylothorax emerged as the most prevalent lymphatic disorder followed by protein-losing enteropathy and plastic bronchitis. The most common diagnostic investigation method was noncontrast magnetic resonance lymphangiography (52%). Eleven centers (35%) conducted lymphatic interventions with a median of 3 (interquartile range, 1 - 4) procedures per year and 12 (interquartile range, 5 - 15) interventions in total per center., Conclusions: This study confirms the rarity of and variation in treatment approaches for lymphatic disorders across Europe. With at least 11 centers offering lymphatic interventions, the adoption of these procedures is on the rise in Europe. To improve the quality of care and treatment outcomes for these complex patients, it is crucial to consider evidence-based lymphatic diagnostics, interventional lymphatic procedures, and the centralization of services in Europe.

The CardioMEMS HF System (Abbott, Abbott Park, IL) is the first FDA- and CE-Mark-approved device for monitoring patients with heart failure, significantly reducing hospitalizations and improving the quality of life for NYHA class III non-congenital adult patients. This device, implanted percutaneously, allows the direct monitoring of pulmonary arterial pressure with the wireless transfer of pressure data to the clinician, who can adjust the therapy remotely. Limited experience exists regarding its use in patients with congenital heart disease (CHD). CardioMEMS device implantation is feasible and safe in selected adults and children with CHD. The potential of the device to reduce heart failure hospitalizations in this population is enormous, but further multi-center studies are needed to demonstrate its efficacy.

Background: Stent implantation has become the preferred method of treatment for treating vessel stenosis in congenital heart diseases. The availability of covered stents may decrease complications and have an important role in the management of patients with complex anatomy., Aim: This study aims to evaluate the feasibility and safety of the pre-mounted cobalt-chromium stent-graft-covered ePTFE Aortic BeGraft in a broad spectrum of vascular lesions., Methods: This is a multicenter retrospective results analysis of 107 implanted BeGraft stents between 2016 and 2022 in six different European centers., Results: One hundred and four patients with a mean age of thirteen years (range 1-70 years) and with the body weight of 56.5 kg (range 11-115 kg) underwent the BeGraft stent implantation. Stents were implanted in the following conditions: aortic coarctation (74 patients), RVOT dysfunction (12 patients), Fontan circulation (7 patients), and miscellaneous (11 subjects with complex CHD). All the stents were implanted successfully. The median stent diameter was 16 mm (range 7-24 mm), and the median length was 39 mm (range 19-49 mm). Major complications occurred in five subjects (4.7%). During a median follow-up of fourteen (1-70) months, stents' re-dilatation was performed in five patients., Conclusions: The BeGraft stent can be used safely and effectively in a wide spectrum of congenital heart diseases. Whether these good results will be stable in the longer term still needs to be investigated in a follow-up given its recent introduction into clinical practice, in particular regarding stent fracture or neointimal proliferation., Competing Interests: The authors declare no conflict of interest.

Findings of eosinophilic and lymphomonocytic inflammatory infiltrates in endomyocardial biopsies (EMBs) may help in myocardial disease diagnosis identification. Eosinophilic myocarditis (EM), a rare condition, is fatal if left untreated and has rarely been described in heart transplant recipients. An extensive work up is necessary to achieve an early etiological diagnosis; however, the underlying cause remains unexplained in nearly one-third of the patients. The cornerstone of treatment is corticosteroids, comprehensive therapy and heart failure management (including advanced mechanical support for fulminant myocarditis). We have described the case of a 17-year-old heart transplant recipient who presented with a cardiogenic shock. He was admitted to our intensive care unit and treated with inotropic drugs, such as milrinone, adrenaline, vasopressin, and levosimendan; the doses of these drugs were in accordance with our internal protocol. The patient underwent cardiac catheterization, coronarography, and right ventricular EMB. EMB revealed inflammatory lymphomonocytic and eosinophil granulocyte infiltrates; thus, steroid therapy was initiated, with complete recovery achieved after 15 days. Performing an early differential diagnosis among eosinophilic infiltration, acute cellular rejection (ACR), and possible chemotherapeutic damage is emerging as an important challenge. To our knowledge, this is the first reported case of a lymphomonocytic inflammatory infiltration with numerous eosinophilic granulocytes in the interstitium in a surviving heart transplant recipient., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Placidi, Francalanci, Adorisio, Girardi, Vinti, Panebianco, Rebonato, Amodeo and Grutter.)

Aortic wall injuries may occur after interventional treatment of aortic coarctation (CoA), especially after balloon angioplasty. We reported on a patient who presented with an intra-stent aneurysm formation after direct stenting of a native near atretic aortic CoA by using a BeGraft Aortic stent. This evidence supports the need to maintain a strict follow-up protocol. A computed tomography scan is mandatory, after covered stent implantation as well, especially in high-risk cases and even in the absence of any immediate apparent complication., (© 2023 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

Background: The aim of this study is to identify echocardiographic predictors of transient left ventricle dysfunction after pulmonary valve balloon dilatation (PVBD), in neonates with pulmonary valve stenosis (PVS) and atresia with intact septum (PAIVS) at birth., Methods: The study includes patients admitted at the Bambino Gesù Children Hospital from January 2012 to January 2017. Clinical, echocardiographic and cardiac catheterization data before and after PVBD were retrospectively analyzed., Results: Twenty-nine infants were included in the study (21 male and eight female). The median age was 5.8 ± 7.1 days. Eight patients developed transient LV dysfunction (three PAIVS and five PVS) and comparing data before and after the procedure, there was no difference in right ventricle geometrical and functional parameters except for evidence of at least moderate pulmonary valve regurgitation after PVBD., Conclusion: Moderate to severe degree pulmonary valve regurgitation was significant associated to LV dysfunction ( p < 0.05) in PVS and PAIVS patients., Competing Interests: The authors declare that they have no competing interests., (© 2022 D’Anna et al.)

Endomyocardial biopsy (EMB) is a well-known diagnostic tool for the investigation and treatment of myocardial diseases and remains the gold standard for the diagnosis of myocarditis. Due to its invasiveness, with a complication rate ranging from 1 to 15%, its role in the diagnostic work-up of pediatric heart failure is not well established. The aim of this review is to define the role of EMB as diagnostic technique in the work up of children presenting with severe left ventricular dysfunction with the support of our center experience.

: We report the case of a 56-year-old male patient affected by a severe form of Ebstein's anomaly (type C of Carpentier classification) with secundum atrial septal defect, who presented to the emergency department with impaired consciousness, seizures, and trismus. The brain computed tomography scan showed evidence of a mass located in the frontal lobe, confirmed by brain MRI consistent with brain abscess. Both echocardiography and cardiac MRI showed no evidence of valvular vegetation. This case shows how the combination of increased atrial pressure and bidirectional shunt through atrial septal defect may lead to paradoxical embolization.

Objective: This study analyses dynamicchangesin dispersion of ventricularrepolarization over the time course of takotsubo cardiomyopathy (TC), and their relationships with clinical features and life-threatening arrhythmias., Methods and Results: All consecutive patients admitted to our division between January 2008 and December 2011 with a diagnosis of TC were analysed. Patients with prior myocardial infarction, symptoms-onset-to-admission time greater than 12 hours, an implanted pacemaker, or under treatment with drugs affecting QTinterval, were excluded. Standard 12-lead ECG recordings during the acute, subacute and chronic phases were collected for each patient. Twenty-four patients (23 women, 63 +/- 14 years) were includedin our analysis. Only one patient experienced ventricular arrhythmias (4.2%). Significant increases were observed in QT and QTc intervals (from 420 ?423 to 505 +/- 66 ms, P < 0.00001, and from 479 +/- 33 to 551 +/- 51 ms, P < 0.00001, respectively), QTdispersion (from 59 +/- 18 to 100 +/- 44 ms, P=0.0006), Tpeak-to-Tend (from 82 +/- 20 to 123 +/- 39 ms, P=0.00006) and Tpeak-to-Tend/QT (from 0.20 +/- 0.33 to 0.26 +/- 0.57, P=0.0003) during the subacute phase. All these parameters returned to baseline values in the chronic phase and did not show any significant differences between the acute and chronic phases., Conclusions: A marked increase in QTc, QT dispersion, Tpeak-to-Tend and Tpeak-to-Tend/QT was observed during the subacute phase; this increase was transient and reverted in allpatients before hospital discharge. Ofnote, these findings were not associated with an increased risk of life-threatening arrhythmias.

Background: Acute appendicitis is the most common emergency in abdominal surgery, but remains a continuing controversy regarding the most appropriate method of removing the inflamed appendix., Materials and Methods: From January 2002 to December 2012, 1037 women underwent appendectomy (average age: 25±15.7 y; range: 6 to 91 y). Of these, 519 underwent open appendectomy (OA) and 518 underwent laparoscopic appendectomy (LA). For all the patients we determined the postoperative hospital stay, the eventual readmissions within 30 days after discharge, the length of surgical procedures (data were available only for the period from January 2008 to December 2012), the costs for the OA and LA, and the rate of negative appendicitis., Results: In our cohort of patients, 189 women (18.2%) had a negative appendectomy. Considering the postoperative hospital stay (average: 4.2±3.6 d; range: 1 to 32 d in OA group and average: 3.9±3.1 d; range: 1 to 21 d in LA group; P=0.15) there were no statistical differences between 2 groups. The average length of surgical procedures in LA group was 42.3±18.4 minutes (range: 8 to 135 min) and 43.2±19 minutes in the OA group (range: 10 to 135 min) (P=0.63). The average net cost of LA was 1203.61 euros, whereas for OA it was 95.18 euros. In this study, we considered only the surgical materials., Conclusions: LAs are not associated with a lower complication rate than the OAs and, above all, LAs are more expensive than OAs. Also we believe that laparoscopic approach should be used only in case of unclear abdominal pain and not for the treatment of clear acute and uncomplicated appendicitis.

The association between truncus arteriosus and total anomalous pulmonary venous return is exceedingly rare. Here described is a neonate presenting with a common arterial trunk and a previously unreported "scimitar-like" mixed total pulmonary venous return who underwent a successful one-stage repair. The unprecedented anatomic pattern of the systemic and pulmonary venous connection and the original surgical strategy are discussed., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Introduction: Disaster is a collective responsibility requiring coordinated response from all parts of society. This theme focused on coordination and management issues in a diverse range of scenarios., Methods: Details of the methods used are provided in the preceding paper. The chairs moderated all presentations and produced a summary that was presented to an assembly of all of the delegates. Although the main points developed in Themes 1 and 4 were different from each other (as reported in the Results section), their implementation was similar. Therefore, the chairs of both groups presided over one workshop that resulted in the generation of a set of Action Plans that then were reported to the collective group of all delegates., Results: The main points developed during the presentations and discussions included: (1) the need for evidence-based assessments and planning, (2) the need for a shift in focus to health-sector readiness, (3) empowerment of survivors, (4) provision of relief for the caregivers, (5) address the incentives and disincentives to attain readiness, (6) engage in joint preparation, response, and training, (7) focus on prevention and mitigation of the damage from events, and (8) improve media relations. There exists a need for institutionalization of processes for learning from experiences obtained from disasters., Discussion: Action plans presented include: (1) creation of an Information and Data Clearinghouse on Disaster Management, (2) identification of incentives and disincentives for readiness and develop strategies and interventions, and (3) act on lessons learned from evidence-based research and practical experience., Conclusions: There is an urgent need to proactively establish coordination and management procedures in advance of any crisis. A number of important insights for improvement in coordination and management during disasters emerged.

We report our first experience with a laparoscopic treatment of congenital choledochal cysts involving the total cyst resection and the reconstruction of the biliary and gastrointestinal tracts through a transmesocolic hepatic-jejunal Roux-en-Y loop anastomosis. The procedure was carried out in a 14-kg 6-year-old girl with a congenital choledochal cyst of the first type, according to the Alonso-Lej classification. The cyst was divided using a Multifire EndoGIA 30 stapler. Hepatic-jejunal and jejunojejunal anastomoses were made with 4.0 chrome catgut interrupted sutures. Intestinal recanalization occurred on the 2nd postoperative day and the postoperative course was uneventful. The laparoscopic approach affords several advantages: excellent intraoperative visualization of tiny structures and, therefore, great surgical accuracy; early resumption of peristalsis; no postoperative pain; no laparocele; prevention of adhesions; excellent esthetics; and quicker resumption of school and sports activities.

The authors describe the technique for the treatment of gallbladder stones using a laparoscopic approach and discuss the diagnostic and operative flow chart stressing complications and ways to avoid them. A total of 2517 non-selected patients underwent surgery since october 1990 up to september 1995. 252 were affected by acute cholecystitis (10%); 172 underwent emergency laparoscopic cholecystectomy. ERCP was performed in 278 patients (11.04%): 177 underwent endoscopic sphincterotomy and laparoscopic cholecystectomy, 21 underwent laparoscopic cholecystectomy before sphincterotomy, 8 laparoscopic cholecystectomy and ESWL. Laparoscopic cholecystectomy was converted into laparotomy in 37 patients (1.4%); surgery was abandoned in 3 patients following to onset of intense bradycardia. Major complications were observed in 0.63%; bile duct injury occurred in four patients (0.15%). One patient died following a massive intraoperative myocardial infarction. Average operative time was 21 minutes. Only 22.8% of patients required mild analgesia on the first day after surgery. The average hospital postoperative stay was 2.6 days. Return to work took place in 98% of non complicated patients within one week of being discharged from hospital.

Eleven patients underwent choledochoduodenostomy under laparoscopic control: 5 for adenocarcinoma of head of pancreas, including 2 with extension into duodenum, 3 for chronic pancreatitis. 1 for gastric carcinoma with pancreatic infiltration 1 for carcinoma of ampulla and 1 for stenosing papillitis. Mean duration of operation was 97.9 minutes and mean hospital stay 7.8 days. No immediate or delayed postoperative complications were reported. The advantages of this method are the marked reduction in recovery time, especially in severely debilitated elderly patients, and the absence of postoperative pain.

A total of 92 patients were submitted to laparoscopic cholecystectomy during the period Autumn 1990-Spring 1991. The dissection of the gallbladder from the hepatic bed was performed in 40 patients using the Argon beam coagulator, in 25 using the monopolar electrocoagulator and in 27 using the Holmio laser beam. In average, with the Argon beam coagulator time procedure was respectively 2.7 and 5.4 minutes shorter than monopolar electrocoagulator and Holmio laser. Only one complication (pneumomediastinum) was correlated with the use of the Argon beam coagulator.

Between May 1978 and July 1981 at the Department of Cardiovascular Surgery, Padua University Medical School in Verona, 10 patients with coarctation of the aorta underwent surgical treatment with a by-pass between the left subclavian artery and the descending aorta. A synthetic fibre graft was employed. The patient's age varied between 20 and 46 years and all were hypertensive: 160-220 mmHg systolic, 90-110 diastolic pressure. No hospital deaths occurred. Blood pressure decreased significantly in all patients as well as their electrocardiographic signs of left ventricular hypertrophy.

Pulmonary Sequestration is a congenital anomaly rarely seen in the pediatric age. Clinical manifestations commonly appear with a respiratory symptomatology and less frequently with cardiac signs especially in the case of an associated congenital heart disease. In the presented case, isolated Pulmonary Sequestration manifested itself as a congestive heart failure, and a diastolic overloading of the left ventricle. After surgical removal of the Sequestration was demonstrated a normalization of both clinical and haemodynamic findings.