Significance of colonization by antibiotic-resistant organisms prior to congenital heart disease surgery in children from low- to middle-income countries sent by non-governmental organizations to Switzerland.

PURPOSE: Children with congenital heart disease (CHD) from low- to middle-income countries (LMIC) are suspected to have a high prevalence of antibiotic-resistant microorganisms (ARMOs) carriage, but data are currently lacking. Carriage of ARMOs could impact the post-operative course in pediatric intensive care unit (PICU). The aim of the study was to assess the prevalence of ARMOs carriage in children with CHD from LMIC and its impact on post-operative outcomes. METHODS: This was a retrospective monocentric study from 01/2019 to 12/2022. Included patients were children (0-18 years) from a LMIC admitted after CHD surgery and with AMRO screening performed the week before. Infections and post-operative evolution were compared based on ARMOs carriage status. FINDINGS: Among 224 surgeries (median age 38.5 months (IQR 22-85.5)), ARMOs carriage was evidenced in 95 cases (42.4%). Main organisms isolated were Extended Spectrum Beta-Lactamase (ESBL) producing E. coli (75/224) 33.5%)) and ESBL-K. pneumoniae (30/224) 13.4%)). Median mechanical ventilation duration was 1 day (IQR 0-1), PICU stay 3 days (IQR 2-4) and hospital stay 6.5 days (IQR 5-10). A total of 17 infectious episodes occurred in 15 patients, mostly consisting in hospital-acquired pneumonia (HAP) (12/17). Only two infections were caused by a colonizing ARMO. Occurrence of infections and patients' outcome were similar between ARMO carriers and non-carriers. Higher use of carbapenems (6 (6.3%) vs 1 (0.8%), p = 0.04) and a trend to a higher use of vancomycin (14 (13.7%) vs 9 (6.9%), p = 0.04) in case of ARMOs carriage. Applying current guidelines, negative swab screening could have led to sparing most of empirical vancomycin therapy (11/12) for HAP based on current guidelines. CONCLUSION: Prevalence of AMROs carriage is high in children from LMIC and has a limited impact on patients' outcome. However, ARMOs carriage leads to higher consumption of antibiotics. Screening may help saving use of broad-spectrum antibiotic in non-carrier patients.

Comparison of Perioperative and Postoperative Outcomes Among 3 Left Atrial Incisions: Conventional Direct, Transseptal, and Superior Septal Left Atriotomy.

BACKGROUND: Achieving optimal exposure of the mitral valve during surgical intervention poses a significant challenge. This study aimed to compare perioperative and postoperative outcomes associated with 3 left atriotomy techniques in mitral valve surgery-the conventional direct, transseptal, and superior septal approaches-and assess differences during the surgical procedure and the postoperative period. METHODS: Inclusion criteria were patients undergoing mitral valve surgery from January 2010 to December 2020, categorized into 3 cohorts: group 1 (conventional direct; n = 115), group 2 (transseptal; n = 33), and group 3 (superior septal; n = 59). To bolster sample size, the study included patients undergoing mitral valve surgery independently or in conjunction with other procedures (eg, coronary artery bypass grafting, aortictricuspid surgery, or maze procedure). RESULTS: No substantial variance was observed in the etiology of mitral valve disease across groups, except for a higher incidence of endocarditis in group 3 (P = .01). Group 1 exhibited a higher frequency of elective surgeries and isolated mitral valve procedures (P = .008), along with reduced aortic clamping and cardiopulmonary bypass durations (P = .002). Conversely, group 3 patients represented a greater proportion of emergency procedures (P = .01) and prolonged intensive care unit and hospital stays (P = .001). No significant disparities were detected in terms of permanent pacemaker implantation, postoperative complications, or mortality among the groups. CONCLUSION: Mitral valve operations that employed these 3 atriotomy techniques demonstrated a safe profile. The conventional direct approach notably reduced aortic clamping and cardiopulmonary bypass durations. The superior septal method was primarily employed for acute pathologies, with no significant escalation in postoperative arrhythmias or permanent pacemaker implantation, although these patients had prolonged intensive care unit and hospital stays. These outcomes may be linked to the underlying pathology and nature of the surgical intervention rather than the incision method itself.

Infective Endocarditis Due to Kingella kingae.

Infective endocarditis due to Kingella kingae is a rare but serious invasive infection that occurs mostly in children. Recent advances in nucleic acid amplification testing as well as in cardiac imaging have enabled more accurate diagnosis. A good understanding of the epidemiology and virulence factors remains crucial to guide the therapeutic approach. Here, we synthesize the current state of knowledge on epidemiological features, pathophysiological insights, complications, and therapy regarding Kingella kingae endocarditis in children and adults. Finally, throughout this comprehensive review, knowledge gaps and areas for future research are also identified.

Triple switch' aortic and pulmonary root inversion and modified Senning procedure for anatomically complex left transposition of the great arteries with inlet ventricular septal defect and pulmonary stenosis.

Left transposition of the great arteries with inlet ventricular septal defect and pulmonary stenosis is a relatively uncommon cardiac malformation. Two surgical treatments are available: double switch or physiological correction. The choice of surgical technique depends on the results of a discussion between the family and the surgeon. Choosing the appropriate technique is challenging because all options present various complications and benefits. We present a 'triple switch' aortic and pulmonary root inversion and modified Senning procedure for an anatomically complex left transposition of the great arteries with an inlet ventricular septal defect and pulmonary stenosis.

Aortic Dilatation on the Edge of Dissection-Do We Operate Too Late? The Ratio between Ascending and Descending Aorta DiameteR (RADAR).

(1) Background: There is a need for a novel surrogate marker to ease decision making when facing ascending aortic dilatation. In this article, we study the ratio between ascending and descending aorta diameters as a potential one. (2) Methods: Retrospective observational cohort study, including all the patients who underwent surgery for acute type A aorta dissection (aTAAD) between January 2014 and September 2020 at our center. A total of 50 patients were included. Clinical and demographic data were collected. The anatomical measurements were made including orthogonal maximal diameters of the ascending and descending aorta, post-dissection whole circumference length (post-wCL), post-dissection true lumen circumference length (post-tCL), and surface and sphericity indices of the ascending and descending aorta. Pre-dissection ascending aorta diameter (pre-AAD) and pre-dissection descending aorta diameter (pre-DAD) were calculated as well as the ratio between them and compared with reference values. (3) Results: Of the pre-AAD patients, 96% had smaller than the recommended 55 mm. The ratio between the descending and ascending aorta pre-dissection diameters was significantly smaller compared to the reference value (0.657 ± 0.125 versus 0.745 ± 0.016 with a mean difference of -0.088 and a p < 0.001). (4) Conclusions: The 55 mm threshold for aorta maximal diameter is an insufficient criterion when assessing the risk of dissection. The ratio between DAD and AAD is a parameter worthy of analysis as a tool to stratify the risk of dissection.

Decompressive hemicraniectomy in pediatric malignant arterial ischemic stroke: a case-based review.

PURPOSE: Malignant stroke is a life-threatening emergency, with a high mortality rate (1-3). Despite strong evidence showing decreased morbidity and mortality in the adult population, decompressive hemicraniectomy (DCH) has been scarcely reported in the pediatric stroke population, and its indication remains controversial, while it could be a potential lifesaving option. METHODS AND RESULTS: We performed an extensive literature review on pediatric malignant arterial ischemic stroke (pmAIS) and selected 26 articles reporting 97 cases. Gathering the data together, a 67% mortality rate is observed without decompressive therapy, contrasting with a 95.4% survival rate with it. The median modified Rankin score (mRS) is 2.1 after surgery with a mean follow-up of 31.8 months. For the 33% of children who survived without surgery, the mRS is 3 at a mean follow-up of 19 months. As an illustrative case, we report on a 2-year-old girl who presented a cardioembolic right middle cerebral artery stroke with subsequent malignant edema and ongoing cerebral transtentorial herniation in the course of a severe myocarditis requiring ECMO support. A DCH was done 32 h after symptom onset. At the age of 5 years, she exhibits an mRS of 3. CONCLUSION: Pediatric stroke with malignant edema is a severe condition with high mortality rate if left untreated and often long-lasting consequences. DCH might minimize the vicious circle of cerebral swelling, increasing intracranial pressure and brain ischemia. Our literature review underscores DCH as an efficient therapeutic measure management of pmAIS even when performed after a significant delay; however, long-lasting morbidities remain high.

Three-dimensional printing and virtual reconstruction in surgical planning of double-outlet right ventricle repair.

Objectives: For more than a decade, 3-dimensional (3D) printing has been identified as an innovative tool for the surgical planning of double-outlet right ventricle (DORV). Nevertheless, lack of evidence concerning its benefits encourages us to identify valuable criteria for future prospective trials. Methods: We conducted a retrospective study involving 10 patients with DORV operated between 2015 and 2019 in our center. During a preoperative multidisciplinary heart team meeting, we harvested surgical decisions following a 3-increment step process: (1) multimodal imaging; (2) 3D virtual valvular reconstruction (3DVVR); and (3) 3D-printed heart model (3DPHM). The primary outcome was the proportion of predicted surgical strategy following each of the 3 steps, compared with the institutional retrospective surgical strategy. The secondary outcome was the change of surgical strategy through 3D modalities compared with multimodal imaging. The incremental benefit of the 3DVVR and 3DPHM over multimodal imaging was then assessed. Results: The operative strategy was predicted in 5 cases after multimodal imaging, in 9 cases after 3DVVR, and the 10 cases after 3DPHM. Compared with multimodal imaging, 3DVVR modified the strategy for 4 cases. One case was correctly predicted only after 3DPHM inspection. Conclusions: 3DVVR and 3DPHM improved multimodal imaging in the surgical planning of patients with DORV. 3DVVR allowed a better appreciation of the relationships between great vessels, valves, and ventricular septal defects. 3DPHM offers a realistic preoperative view at patient scale and enhances the evaluation of outflow tract obstruction. Our retrospective study demonstrates benefits of preoperative 3D modalities and supports future prospective trials to assess their impact on postoperative outcomes.

Three-dimensional printed moulds to obtain silicone hearts with congenital defects for paediatric heart-surgeon training.

OBJECTIVES: Many types of congenital heart disease are amenable to surgical repair or palliation. The procedures are often challenging and require specific surgical training, with limited real-life exposure and often costly simulation options. Our objective was to create realistic and affordable 3D simulation models of the heart and vessels to improve training. METHODS: We created moulded vessel models using several materials, to identify the material that best replicated human vascular tissue. This material was then used to make more vessels to train residents in cannulation procedures. Magnetic resonance imaging views of a 23-month-old patient with double-outlet right ventricle were segmented using free open-source software. Re-usable moulds produced by 3D printing served to create a silicone model of the heart, with the same material as the vessels, which was used by a heart surgeon to simulate a Rastelli procedure. RESULTS: The best material was a soft elastic silicone (Shore A hardness 8). Training on the vessel models decreased the residents' procedural time and improved their grades on a performance rating scale. The surgeon evaluated the moulded heart model as realistic and was able to perform the Rastelli procedure on it. Even if the valves were poorly represented, it was found to be useful for preintervention training. CONCLUSIONS: By using free segmentation software, a relatively low-cost silicone and a technique based on re-usable moulds, the cost of obtaining heart models suitable for training in congenital heart defect surgery can be substantially decreased.

Sterile peri-graft abscess formation following aortic replacement: A word of caution for usage of BioGlue®.

We report sterile peri-graft abscess formation following an ascending aortic and hemiarch replacement for acute type A aortic dissection, possibly caused as a reaction to BioGlue®. The patient was successfully treated by drainage, cleaning, removal of BioGlue® remnants, three sessions of negative-pressure wound dressing, and secondary chest closure.

Fulminant Infective Endocarditis Due to Kingella Kingae and Several Complications in a 6-Year-Old Girl: A Case Report.

Kingella kingae is a gram-negative coccobacillus belonging to the HACEK group (Haemophilus species, Aggregatibacter actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, and Kingella species) and is a common oropharyngeal colonizer of healthy young children. Osteoarticular infection is the most commonly reported invasive Kingella kingae infection in children, usually presenting a mild clinical picture. However, it can also cause severe invasive infections, especially infective endocarditis, with a high complication rate. We report the case of a 6-year-old girl, with no past medical history, who presented with fulminant infective endocarditis due to Kingella kingae. She received emergency venoarterial extracorporeal membrane oxygenation support, rapidly underwent cardiac surgery, and was then treated using ceftriaxone for 4 weeks as recommended by the American Heart Association. The patient's postoperative course was marked by a cerebral ischemic stroke consistent with septic embolism. She also presented with a para-aortic pseudoaneurysm that required a secondary surgical procedure, with a good postoperative result. This report illustrates a case of fulminant infective endocarditis due to Kingella kingae and responsible for two major complications. We also describe the preventive valve surgery performed to ensure the preservation of valve function and its capacity for growth.

Double-root inversion for complex malposition of the great arteries with tricuspid valve straddling.

Dextro-transposition of the great vessels associated with pulmonary stenosis, double-outlet right ventricle, and straddling of the tricuspid valve is an uncommon condition. Several treatment options are available for this malformation, but most of them are not optimal. For patients with transposition of the great vessels, the gold standard procedure, which is an arterial switch procedure, would usually be performed, whereas for patients with pulmonary stenosis, a Rastelli operation or a Nikaidoh procedure would be proposed. Both of these methods have several advantages and disadvantages. Selected patients can qualify for the double-root rotation procedure, which is limited by the function of the pulmonary and aortic valves, the position of the coronary arteries, and the skill of the surgeon[1]. After a thorough analysis of all the preoperative test results, our patient qualified for a surgical correction of the malformation. Due to preexisting pulmonary regurgitation and severe dilation of the pulmonary root, the patient was not considered a good candidate for the arterial switch operation. Therefore, it was decided that the double-root inversion was the best option. Introduction The double-root inversion gives the patient the possibility of avoiding a reoperation. If the patient were to have the Nikaidoh or the Rastelli procedure, we know that the pulmonary graft would eventually have to be replaced. For this reason, we would like to share our experience with the double-root inversion method.

Cardiac Neural Crest Cells: Their Rhombomeric Specification, Migration, and Association with Heart and Great Vessel Anomalies.

Outflow tract abnormalities are the most frequent congenital heart defects. These are due to the absence or dysfunction of the two main cell types, i.e., neural crest cells and secondary heart field cells that migrate in opposite directions at the same stage of development. These cells directly govern aortic arch patterning and development, ascending aorta dilatation, semi-valvular and coronary artery development, aortopulmonary septation abnormalities, persistence of the ductus arteriosus, trunk and proximal pulmonary arteries, sub-valvular conal ventricular septal/rotational defects, and non-compaction of the left ventricle. In some cases, depending on the functional defects of these cells, additional malformations are found in the expected spatial migratory area of the cells, namely in the pharyngeal arch derivatives and cervico-facial structures. Associated non-cardiovascular anomalies are often underestimated, since the multipotency and functional alteration of these cells can result in the modification of multiple neural, epidermal, and cervical structures at different levels. In most cases, patients do not display the full phenotype of abnormalities, but congenital cardiac defects involving the ventricular outflow tract, ascending aorta, aortic arch and supra-aortic trunks should be considered as markers for possible impaired function of these cells. Neural crest cells should not be considered as a unique cell population but on the basis of their cervical rhombomere origins R3-R5 or R6-R7-R8 and specific migration patterns: R3-R4 towards arch II, R5-R6 arch III and R7-R8 arch IV and VI. A better understanding of their development may lead to the discovery of unknown associated abnormalities, thereby enabling potential improvements to be made to the therapeutic approach.

Cylinder mitral and tricuspid valve replacement in neonates and small children.

OBJECTIVES: Atrioventricular valve replacement in small children is associated with high morbidity and mortality. There are no prostheses available with a diameter ˂15 mm. This study reports our initial experience with a cylinder valve for mitral and tricuspid valve replacement in infants and small children. METHODS: Our cylinder valve was hand-made for patients requiring atrioventricuclar valve replacement with an annulus of <15 mm. A 12-mm Contegra valve was prepared and placed inside a 14-mm Gore-Tex tube graft and sutured on both extremities. RESULTS: Eight patients were included, with a median age of 6.9 months (range 1 day to 38 months). Four had mitral and 4 had tricuspid valve replacement. All implants were technically successful, with no significant regurgitation, no stenosis and no left ventricular outflow tract obstruction. There were 3 early deaths from low cardiac output, in patients with significant associated lesions (severe neonatal Ebstein's, pulmonary artery-intact ventricular septum, biventricular conversion from Norwood stage 1). Two patients required early reintervention: 1 for balloon dilatation for stenosis and 1 for reoperation for paravalvular leak. During follow-up, 2 patients had mitral valve replacement with a 16-mm mechanical valve at 9 and 20 months from the cylinder valve implantation. The remaining 2 patients are alive and well 2 years and 2 months after the procedure. CONCLUSIONS: Cylinder valve replacement of atrioventricular valves was feasible without any technical issues. It was successful in getting out of a difficult situation and allows for somatic growth and implantation of a reasonably-sized mechanical prosthesis on the annulus.

"Double-Parachute" Mitral Valve.

A 38-year-old asymptomatic man was referred by his general practitioner for a 3/6 systolic heart murmur, which was detected during a routine consultation. Echocardiography revealed a parachute mitral valve associated with a parachute-like membrane, causing significant subaortic obstruction that was eventually surgically resected with an excellent postoperative outcome. (Level of Difficulty: Beginner.).

"Micro-Bentall" procedure.

Truncus arteriosus, an anomaly of the conotruncus, is an extremely rare congenital heart disease that affects 1.19% of all patients with congenital heart diseases.  We present a surgical technique using an 8-mm cryopreserved aortic root homograft in the aortic position and a 12-mm pulmonary valved conduit in the right position that allowed us to correct this rare congenital malformation.  The cryopreserved aortic root homograft was considered a priority option for surgical correction.  The neonatal Bentall (micro-Bentall) procedure is a surgically demanding procedure but can be performed successfully by an experienced surgeon.  If we were performing a non-salvage procedure, we would have chosen a decellularized allograft.

Pretreatment with glucose-insulin-potassium improves ventricular performances after coronary artery bypass surgery: a randomized controlled trial.

Heart failure is the main cause of poor outcome following open heart surgery and experimental studies have demonstrated that glucose-insulin-potassium (GIK) infusion exerts cardioprotective effects by reducing myocardial ischemia-reperfusion injuries. This randomized controlled trial was designed to assess the effects of GIK on left ventricular function in moderate-to-high risk patients undergoing on-pump isolated coronary artery bypass surgery (CABGS), or combined with aortic valve replacement. The primary outcomes were the effects of GIK on two- and three-dimensional left ventricular ejection fraction (2D and 3D-LVEF), and on transmitral flow propagation velocity (Vp), that occurred between the pre- and post-CPB periods. GIK administration was associated with favorable interaction effects (p < 0.001) on 2D-LVEF, 3D-LVEF and Vp changes over the study periods. In GIK pretreated patients (N = 54), 2-D and 3D-LVEF and Vp increased slightly during surgery (mean difference [MD] + 3.5%, 95% confidence interval [95% CI] - 0.2 to 7.1%, MD + 4.0%, 95% CI 0.6-7.4%, and MD + 22.2%, 95% CI 16.0-28.4%, respectively). In contrast, in the Placebo group (N = 46), 2D-and 3D-LVEF, as well as Vp all decreased after CPB (MD - 7.5% [- 11.6 to - 3.4%], MD - 12.0% [- 15.2 to - 8.8%] and MD - 21.3% [- 25.7 to - 16.9%], respectively). In conclusion, the administration of GIK resulted in better preservation of systolic and diastolic ventricular function in the early period following weaning from CPB.

Non-visible scar surgery: Right axillary thoracotomy approach for partial atrioventricular canal repair.

As the outcomes of the repair of congenital heart defects have improved, minimizing the long-term morbidity associated with these repairs has become more important. Avoiding a midline incision and hiding the incision in the axilla allows a virtual "non-visible scar" repair, which can be beneficial for avoiding future psychological morbidity. In this tutorial, we present our technique for right axillary incision for the repair of a partial common atrioventricular defect.

Pediatric Orthotopic Heart Transplantation.

Since the first pediatric orthotopic heart transplant was performed by Dr Adrian Krantowicz in 1967, just days after the first ever human transplant by Dr Christiaan Barnard, the technique for orthotopic heart transplantation has evolved from biatrial anastomosis to separate caval anastomoses, leaving the right atrium intact. In this video tutorial, we present our technique for standard orthotopic heart transplantation in children.