Diagnostic work-up and current management strategies for infective endocarditis in the pediatric population.

Infective endocarditis (IE) remains a serious disease that is associated with significant morbidity and mortality, and despite the significant advances that have been made in understanding the disease process in past decades, its incidence appears to be on the rise recently. Endocarditis in children is no longer a rare occurrence. This appeared to be related to a combination of the improved survival of children with congenital heart diseases (CHDs), increase use of intracardiac protheses, and catheter-related interventions. The American Heart Association (AHA) 2007 guidelines reduced the recommendations for use of prophylactic antibiotics in those with CHDs which occurred despite the noticeable increase in endocarditis incidence around that time. In general, the recommendations for managing children with IE are derived from the adults' guidelines, and the evidence-base is lacking in many clinical scenarios. Understanding the epidemiology, clinical presentations, microbiology, and outcomes of different management strategies for endocarditis is needed to have a clear and optimal plan for these children. In the current narrative review, we discuss IE in the pediatric population in terms of etiology, predisposing factors, and different treatment strategies for this unique population.

Aortic root anatomy: insights into annular and root enlargement techniques.

The introduction of the Y(ang)-technique for aortic root enlargement has sparked a renewed interest in annular and root enlargement procedures world-wide. In order to execute these procedures proficiently however, it's important to understand the complex three-dimensional structure of the aortic root and left ventricular outflow tract, and also be familiar with the different enlargement techniques. Herein, we are providing a description of the aortic root anatomy and the most commonly utilized root enlargement procedures. This should facilitate clinical decision making and guidance of patients towards the most appropriate procedure, which should not only treat the patients' acute symptoms, but should also set the patient up for potentially needed future procedures and respective life-time management of aortic valve disease.

Improved Early Outcomes With Off-Pump Coronary Artery Bypass Grafting in Patients With Left Ventricular Dysfunction: A Systematic Review and Meta-Analysis.

The ongoing debate surrounding coronary artery bypass grafting (CABG) with or without cardiopulmonary bypass persists, particularly in individuals with left ventricular dysfunction. The objective of this study was to evaluate the safety and efficacy of these 2 strategies through a comprehensive meta-analysis of existing studies. A systematic search of PubMed, EMBASE, Web of Science, and the Cochrane Central Registry was conducted from inception to July 2023. The primary focus was on studies comparing on-pump versus off-pump CABG as the primary treatment for multivessel coronary artery disease in patients with left ventricular dysfunction (ejection fraction ≤40%), with mortality as the primary outcome. The meta-analysis included 26 studies with a total of 35,863 patients. The results revealed a significant reduction in mortality risk [risk ratio (RR), 0.75; 95% confidence interval (CI), 0.60-0.93; P = 0.009] and other perioperative morbidities associated with off-pump CABG. These included stroke (RR, 0.67; 95% CI, 0.54-0.82; P = 0.0002), myocardial infarction (RR, 0.74; 95% CI, 0.56-0.97; P = 0.03), pulmonary complications (RR, 0.71; 95% CI, 0.55-0.92; P = 0.010), postoperative transfusion (RR, 0.70; 95% CI, 0.55-0.88; P = 0.002), neurological dysfunction (RR, 0.80; 95% CI, 0.64-1.00; P = 0.05), infection (RR, 0.74; 95% CI, 0.56-0.97; P = 0.03), renal failure (RR, 0.79; 95% CI, 0.67-0.95; P = 0.010), and reoperation for bleeding (RR, 0.66; 95% CI, 0.52-0.84; P = 0.0006). However, no significant difference was observed between the 2 groups regarding postoperative atrial fibrillation (RR, 0.97; 95% CI, 0.84-1.12; P = 0.69). In conclusion, off-pump CABG demonstrates a lower perioperative mortality risk and improved overall early outcomes compared with on-pump techniques in individuals with reduced left ventricular function.

Efficacy of automated fasteners versus hand-tied knots in cardiac surgery: a systematic review and meta-analysis.

Valve surgery is common in cardiac procedures, with fasteners like COR-KNOT® and hand-tied knots used for knot securing. This study compares their efficacy in valve surgery patients. We searched PubMed, SCOPUS, and Cochrane Central until August 2023. Outcomes assessed included aortic cross-clamp time (AXT), cardiopulmonary bypass (CPB) time, valvular regurgitation, mortality, prolonged ventilatory support, atrial fibrillation, postoperative left ventricular ejection fraction (LVEF), and renal failure. Subgroup analysis was performed for minimally invasive and open cardiac surgery. We used a random effects model for analysis. We included eight observational studies and two randomized controlled trials (RCTs) with a total of 1.411 participants. COR-KNOT significantly reduced AXT [MD -15.14, 95 % CI (-18.57, -11.70), P<0.00001] and CPB time [MD -12.38, 95 % CI (-14.99, -9.77), P<0.00001]. Valvular regurgitation [RR 0.40, 95 % CI (0.26, 0.61), P<0.0001] and need for prolonged ventilatory support [RR 0.29, 95 % CI (0.13, 0.65), P=0.003] were significantly lower with COR-KNOT. There were no significant differences in mortality [RR 0.39, 95 % CI (0.09, 1.69), P=0.44], atrial fibrillation [RR 1.03, 95 % CI (0.83, 1.27), P=0.81], LVEF changes [MD 0.05, 95 % CI (-1.37, 1.47), P = 0.95], or renal failure [RR 0.87, 95 % CI (0.16, 4.80), P = 0.87]. COR-KNOT devices reduce operative time and valvular regurgitation without increasing mortality or adverse outcomes. This supports their use in enhancing surgical efficiency and patient outcomes. However, ongoing discussions about suturing techniques, especially in minimally invasive procedures, highlight the need for further research and consensus among practitioners. See also the graphical abstract(Fig. 1).

Combined transaortic/transapical left ventricular septal myectomy for subaortic and midventricular obstruction in a paediatric patient.

The patient is a 15-year-old girl who was diagnosed with hypertrophic cardiomyopathy and has been symptomatic due to severe left ventricular outflow tract obstruction. Combined transaortic and transapical left ventricular septal myectomy was performed to relieve the left ventricular outflow tract obstruction and address both subaortic and midventricular gradients.

Partial anomalous venous connection with intrapulmonary dual drainage: Transcatheter treatment of a rare entity.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital condition, and dual-drainage connection PAPVR to the left atrium has been reported in a few cases in the literature; in which cases, percutaneous catheterization was successfully used in lieu of surgery. We, hereby, describe a 7-month-old boy with a functional single-ventricle physiology with dual drainage of the left upper pulmonary vein to the left atrium and the innominate vein. Appropriate recognition of this entity allowed safe occlusion of the anomalous draining vein.

What an adult multiple sclerosis registry can tell us about pediatric onset multiple sclerosis?

BACKGROUND: Multiple Sclerosis (MS) is an immune-mediated, chronic disease of the central nervous system that affects mainly adults. However, it is increasingly recognized that MS may start in childhood resulting in a relentlessly progressive disability and cognitive impairment. Registries across the globe are reporting inconstant data about their Pediatric-Onset Multiple Sclerosis (POMS) patients. Moreover, newer lines of treatments are emerging and showing efficacy in controlling the MS disease regardless of the onset. Therefore, there is a requirement for more research into the clinical profile of POMS in different populations and ethnicities. METHODS: This study was a cross-sectional study that included MS patients who visited the MS unit at Alexandria University from January 2019 to January 2021. We analyzed their epidemiological, clinical, radiological data, and cerebrospinal fluid (CSF) results from their updated records as well as follow-up interviews. RESULTS: Annual Relapse Rate (ARR) was marginally less in POMS than AOMS (0.72 ± 0.57 vs 1.04 ± 0.78 relapse/year, P =.008). POMS patients had a bigger gap to their first relapse (40.0 ± 47.35 vs 22.71 ± 34.33 months, p= .066). The difference in relapse rate between the two groups was abolished after the exclusion of patients who had a gap of more than 5 years to their first relapse. AOMS patients were significantly more likely to start with a second-line disease-modifying treatment (DMT) than POMS patients (11.5% vs 31%, p= .04), whereas POMS patients were more likely to be escalated to the second line (34.6% vs 19.3%, p= .07). ARR had a positive and significant correlation with expanded disability status scale (EDSS) progression per year (rs(24)= .57 p=.003). A Mann-Whitney test indicated that POMS patients who had infratentorial involvement in the initial MRI brain had higher EDSS (3.08 ± 1.99) than POMS who did not (1.07 ± 0.79) U=24 P =.013. IgG index had a significant and positive correlation with annual EDSS progression rate rs (8) = 0.8 p=.001. CONCLUSION: Early disease onset does not mean a higher relapse rate when including the full spectrum of POMS and longer follow-up duration. POMS patients relapsed more on the first-line DMT and escalation should be considered early. Infratentorial involvement in the initial magnetic resonance imaging (MRI) brain and high IgG index are potential predictors for aggressive disease course in POMS.

Repair of Partial Anomalous Pulmonary Venous Connection to the Azygous Vein via Right Thoracotomy.

A 21-month-old boy was diagnosed with partial anomalous pulmonary venous connection, with the right upper pulmonary veins draining to the superior vena cava (SVC). Intraoperatively, it became evident that the right upper pulmonary veins connected to the azygous vein before draining to the SVC.

Late complications after Norwood stage 1 palliation: surgical strategies and a word of caution.

The current case represents one of the complications that may occur when inserting a stent in the left pulmonary artery after a previous Norwood procedure and in the presence of an aneurysmal neo-aorta and large Damus-Kaye-Stansel connection. We demonstrate the technique of a fourth sternotomy with reconstruction of the left pulmonary artery and the neo-aorta in a 12-year-old boy with a functional single ventricle who underwent all three previous stages of palliation of hypoplastic left heart syndrome.

Safety and Efficacy of Right Axillary Thoracotomy for Repair of Congenital Heart Defects in Children.

BACKGROUND: Minimally invasive repair of congenital heart defects in children has not gained wide popularity yet compared to minimally invasive approaches in adults. We sought to review our experience with this approach in children. METHODS: This study included a total of 37 children (24 girls, 64.9%) with a mean age of 6.5 ± 5.1 years, who underwent vertical axillary right minithoracotomy for repair of a variety of congenital heart defects between May 2020 and June 2022. RESULTS: The mean weight of these children was 25.66 ± 18.3 kg. Trisomy 21 syndrome was present in 3 patients (8.1%). The most common congenital heart defects that were repaired via this approach were atrial septal defects (secundum in 11 patients, 29.7%; primum in 5, 13.5%; and unroofed coronary sinus in 1, 2.7%). Twelve patients (32.4%) underwent repair of partial anomalous pulmonary venous connections with or without sinus venosus defects, while 4 patients (10.8%) underwent closure of membranous ventricular septal defects. Mitral valve repair, resection of cor triatriatum dexter, epicardial pacemaker placement, and myxoma resection occurred in 1 patient (2.7%) each. No early mortality or reoperations. All patients were extubated in the operating room, and the mean length of hospital stay was 3.3 ± 2.04 days. Follow-up was complete (mean 7 ± 5 months). No late mortality or reoperations. One patient required epicardial pacemaker placement due to sinus node dysfunction 5 months after surgery. CONCLUSIONS: Vertical axillary right thoracotomy is a cosmetically superior approach that is safe and effective for repair of a variety of congenital heart defects in children.

Single-incision, off-pump repair of a right aortic arch with an aberrant left subclavian artery and diverticulum of Kommerell in an adult.

Repairing a complete vascular ring in adults can be challenging. A right aortic arch with an aberrant retro-oesophageal left subclavian artery and persistent diverticulum of Kommerell represents one of the most common variants seen in adults, and the ring is completed by the left-sided ligamentum arteriosum. Most presentations in adults occur secondary to oesophageal compression, resulting in varying degrees of dysphagia. Owing to the difficulty and challenges associated with exposure in adults, it is not unusual for surgeons to offer a two-incision approach or to stage the procedure. We present a detailed surgical technique for a single-incision repair of a right aortic arch with an aberrant retro-oesophageal left subclavian artery via a left posterolateral thoracotomy.

Outcomes of heart-lung transplantation in Eisenmenger syndrome compared to primary pulmonary hypertension.

OBJECTIVE: Heart-Lung Transplantation (HLTX) is required both in primary pulmonary hypertension (PPH) and Eisenmenger syndrome (ES) when there is associated end-stage heart disease. Although PPH is associated with an otherwise structurally normal heart, ES is associated with congenital heart defects, which may increase the complexity of the operation. This study analyzes if the diagnosis (PPH vs. ES) is related to short-term outcomes after HLTX. METHODS: Patients ≥18 years of age with PPH and ES who underwent HLTX were identified in the United Network for Organ Sharing database from 2005 to 2021. Patients were propensity score matched on heart and lung listing status at the time of transplant. Univariable, multivariable, and Kaplan-Meir survival analyses were performed. RESULTS: The unmatched cohort had 128 PPH and 44 ES patients, and the matched cohort had 44 patients in each group. PPH patients had lower waitlist times and PA pressures but higher FEV1, heart, and lung listing status and ECMO bridge. There were no differences in immediate postoperative outcomes such as dialysis, stroke, and airway dehiscence. PPH patients had a higher treatment rejection in the first year. The 30-day, 1-year, and 3-year survival were better in the PPH group. However, a landmark analysis excluding deaths within 30 days eliminated differences in survival between the groups. Post-transplant dialysis and postoperative mechanical ventilation >5 days were risk factors for 1-year mortality in ES. CONCLUSION: The short-term outcomes of HLTX are inferior in ES compared to PPH and much of the attrition in ES occurs in the immediate postoperative period.

Surgical Experience With Crossed and Anomalous Origin of the Pulmonary Arteries From the Pulmonary Trunk: A Single Center Report of 24 Cases.

BACKGROUND: Anomalies of the pulmonary origin of the pulmonary arteries are uncommon; however, the true incidence is unknown and may be underestimated. We sought to review our experience with this unusual pathology and examine its surgical implications. METHODS: We reviewed medical records between 2011 and 2022. RESULTS: A total of 24 patients were identified. Genetic syndromes were present in 12 patients (50%). The patients were divided into two main groups. Those with septal defects (14 patients,58.3%), and those without (10 patients, 41.7%). Aortic arch hypoplasia with/without coarctation was present in nine patients (37.5%). Pulmonary arterial branch hypoplasia and/or obstruction was present in six patients (25%) with resultant preoperative systemic or suprasystemic right ventricular pressure. Four patients (16.7%) underwent pulmonary artery branch and/or right ventricular outflow tract interventions prior to surgery. All patients underwent surgical interventions for their associated cardiac defects. The crossed pulmonary arteries were uncrossed for those four patients (16.7%) who required concomitant pulmonary arterioplasties and pulmonary arterial branch rehabilitation and who had elevated right ventricular pressures preoperatively. No early or late mortalities. One Alagille's syndrome underwent liver transplant prior to discharge. Late transcatheter interventions were needed in four (16.7%) during the follow-up period, while four patients required repeat aortic arch augmentation for recurrent arch obstruction. CONCLUSIONS: Anomalous origin of the pulmonary arteries from the pulmonary trunk including crossed pulmonary arteries is an uncommon anomaly that may be underrecognized. It can occur in isolation or in association with other heart defects. Recognition of this malpositional anomaly is important as it has specific surgical and/or transcatheter implications.

Early Concerning Outcomes for the Edwards Inspiris Resilia Bioprosthesis in the Pulmonary Position.

BACKGROUND: The Inspiris Resilia (Edwards Lifesciences LLC) bioprosthesis has gained widespread use in the aortic position; however no robust data are available about its use in the pulmonary position. METHODS: We reviewed our outcomes for the Inspiris Resilia in the pulmonary position between August 2019 and October 2021. RESULTS: Twenty-seven patients (15 female patients [56%]; mean age, 22.26 ± 14.99 years) were included. The most common original pathology was tetralogy of Fallot (13 patients, 48%). Five patients (18.5%) had prior transcatheter interventions. The mean right ventricular end-diastolic volume index was 164.25 ± 45.1 mL/m2. Sternotomy or repeat sternotomy was the most common approach (21 patients, 77.8%). The standard technique for pulmonary valve replacement was used in 22 patients (81.5%), whereas the prosthesis was implanted as a conduit in the remaining 5 (18.5%). Trivial to mild prosthetic regurgitation was present in 6 patients (22.2%) at the time of discharge. There was no early mortality. Follow-up was complete in all patients (mean, 16 ± 8 months), with no late mortality or late reoperations. New prosthetic regurgitation developed in 13 patients (48%), all of whom underwent replacement with the standard surgical technique. No regurgitation occurred in the conduit cases. This progressed to moderate regurgitation in 6 patients (22%) and severe in 3 (11%). Three patients (11%) underwent transcatheter valve-in-valve after their surgical pulmonary valve replacement. CONCLUSIONS: The early data regarding the Inspiris Resilia bioprosthesis use in the pulmonary position is concerning. The prosthesis design may not be suitable for low-pressure circulation, or modification of its implantation technique may be needed.

Towards Zero Phrenic Nerve Injury in Reoperative Pediatric Cardiac Surgery: The Value of Intraoperative Phrenic Nerve Stimulation.

BACKGROUND: Phrenic nerve injury is a devastating complication that results in significant morbidity and mortality. We developed a novel technique to localize the phrenic nerve and evaluate its success. METHODS: Two groups of children underwent repeat sternotomy for a variety of indications. Group I (69 patients, nerve stimulator) and Group II (78 patients, no nerve stimulator). RESULTS: There was no significant difference in the mean age and weight between the two groups: (6.4 ± 6.5 years vs. 5.6 ± 6.4 years; p = 0.65) and (25.2 ± 24.1 vs. 22.6 ± 22.1; p = 0.69), respectively. The two groups were comparable in the following procedures: pulmonary conduit replacement, bidirectional cavopulmonary anastomosis, aortic arch repair, and Fontan, while Group I had more pulmonary arterial branch reconstruction (p = 0.009) and Group II had more heart transplant patients (p = 0.001). There was no phrenic nerve injury in Group I, while there were 13 patients who suffered phrenic nerve injury in Group II (p < 0.001). No early mortality in Group I, while five patients died prior to discharge in Group II. Eleven patients underwent diaphragm plication in Group II (p = 0.001). The mean number of hours on the ventilator was significantly higher in Group II (137.3 ± 324.9) compared to Group I (17 ± 66.9), p < 0.001. Group II had a significantly longer length of ICU and hospital stays compared to Group I (p = 0.007 and p = 0.006 respectively). CONCLUSION: Phrenic nerve injury in children continues to be associated with significant morbidities and increased length of stay. The use of intraoperative phrenic nerve stimulator can be an effective way to localize the phrenic nerve and avoid its injury.

How Early Is Early Multiple Sclerosis?

The development and further optimization of the diagnostic criteria for multiple sclerosis (MS) emphasize the establishment of an early and accurate diagnosis. So far, numerous studies have revealed the significance of early treatment administration for MS and its association with slower disease progression and better late outcomes of the disease with regards to disability accumulation. However, according to current research results, both neuroinflammatory and neurodegenerative processes may exist prior to symptom initiation. Despite the fact that a significant proportion of individuals with radiologically isolated syndrome (RIS) progress to MS, currently, there is no available treatment approved for RIS. Therefore, our idea of "early treatment administration" might be already late in some cases. In order to detect the individuals who will progress to MS, we need accurate biomarkers. In this review, we present notable research results regarding the underlying pathology of MS, as well as several potentially useful laboratory and neuroimaging biomarkers for the identification of high-risk individuals with RIS for developing MS. This review aims to raise clinicians' awareness regarding "subclinical" MS, enrich their understanding of MS pathology, and familiarize them with several potential biomarkers that are currently under investigation and might be used in clinical practice in the future for the identification of individuals with RIS at high risk for conversion to definite MS.