Three centers experience with device closure of congenital Gerbode-type perimembranous ventricular septal defects.

OBJECTIVES: We aim to evaluate our experience with interventional closure of Gerbode-type perimembranous ventricular septal defects (pmVSDs). METHODS: We performed three-center retrospective data review of patients with congenital indirect Gerbode-type pmVSDs treated percutaneously between August 2017 and May 2021. Standard safety and latest follow-up outcomes were assessed. RESULTS: Ten patients (six females) were identified with a median age of 6.8 years (range: 2.5-54) and a median weight of 26.5 kg (range: 12-88). The median left ventricular defect size was 10 mm (range: 3-15.5). On baseline ultrasound, 6 patients had absent subaortic rim , 6 patients had trivial aortic regurgitation, and 3 patients had tear-drop-type (small) aortic cusp prolapse. The tricuspid regurgitation was graded II (n = 5) and III (n = 5). Five Lifetech Konar-Multifunctional occluders, four Amplatzer duct occluders II and one Amplatzer duct occluder I were implanted. The median fluoroscopy time was 10.4 min (range: 4.3-20.2). Pre-existing aortic regurgitations remained identical. One new aortic regurgitation was identified before discharge and remained trivial after 48 months of follow-up. No heart block or tricuspid stenosis was observed on a median follow-up of 17 months (range: 3-48). All patients are symptom-free with complete shunt closure and significant regression or resolution of tricuspid regurgitation. CONCLUSIONS: Despite anatomical challenges, interventional closure of congenital indirect Gerbode-type pmVSD appears to be feasible, safe, and most importantly clinically effective using different commercially available devices. Amplatzer duct occluder II and Lifetech Konar-Multifunctional occluder offer interesting specifications to retrogradely target this specific defect with success.

Feasibility and accuracy of printed models of complex cardiac defects in small infants from cardiac computed tomography.

BACKGROUND: Three-dimensional (3-D) printed models are increasingly used to enhance understanding of complex anatomy in congenital heart disease. OBJECTIVE: To assess feasibility and accuracy of 3-D printed models obtained from cardiac CT scans in young children with complex congenital heart diseases. MATERIALS AND METHODS: We included children with conotruncal heart anomalies who were younger than 2 years and had a cardiac CT scan in the course of their follow-up. We used cardiac CT scan datasets to generate 3-D models. To assess the models' accuracy, we compared four diameters for each child between the CT images and the printed models, including the largest diameters (Dmax) of ventricular septal defects and aortic annulus and their minimal diameters (Dmin). RESULTS: We obtained images from 14 children with a mean age of 5.5 months (range 1-24 months) and a mean weight of 6.7 kg (range 3.4-14.5 kg). We generated 3-D models for all children. Mean measurement difference between CT images and 3-D models was 0.13 mm for Dmin and 0.12 mm for Dmax for ventricular septal defect diameters, and it was 0.16 mm for Dmin and -0.13 mm for Dmax for aortic annulus diameter, indicating a non-clinically significant difference. CONCLUSION: Three-dimensional printed models could be feasibly generated from cardiac CT scans in a small pediatric population with complex congenital heart diseases. This technique is highly accurate and reliably reflects the same structural dimensions when compared to CT source images.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018­2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

The usefulness of 3D printed heart models for medical student education in congenital heart disease.

BACKGROUND: Three-dimensional (3D) printing technology enables the translation of 2-dimensional (2D) medical imaging into a physical replica of a patient's individual anatomy and may enhance the understanding of congenital heart defects (CHD). We aimed to evaluate the usefulness of a spectrum of 3D-printed models in teaching CHD to medical students. RESULTS: We performed a prospective, randomized educational procedure to teach fifth year medical students four CHDs (atrial septal defect (ASD, n = 74), ventricular septal defect (VSD, n = 50), coarctation of aorta (CoA, n = 118) and tetralogy of Fallot (ToF, n = 105)). Students were randomized into printing groups or control groups. All students received the same 20 min lecture with projected digital 2D images. The printing groups also manipulated 3D printed models during the lecture. Both groups answered an objective survey (Multiple-choice questionnaire) twice, pre- and post-test, and completed a post-lecture subjective survey. Three hundred forty-seven students were included and both teaching groups for each CHD were comparable in age, sex and pre-test score. Overall, objective knowledge improved after the lecture and was higher in the printing group compared to the control group (16.3 ± 2.6 vs 14.8 ± 2.8 out of 20, p < 0.0001). Similar results were observed for each CHD (p = 0.0001 ASD group; p = 0.002 VSD group; p = 0.0005 CoA group; p = 0.003 ToF group). Students' opinion of their understanding of CHDs was higher in the printing group compared to the control group (respectively 4.2 ± 0.5 vs 3.8 ± 0.4 out of 5, p < 0.0001). CONCLUSION: The use of 3D printed models in CHD lectures improve both objective knowledge and learner satisfaction for medical students. The practice should be mainstreamed.

Image of a left atrial mass after a cardiopulmonary bypass in a child.

Invagination of an appendage into the left atrium is a rare complication. It occurs spontaneously or after open-heart surgery. In our case, a postoperative transesophageal echocardiogram, after closure of a ventricular septal defect in a 5-month-old infant, revealed a large mass in the left atrium. A diagnosis of a left appendage inversion was confirmed after external examination of the heart. Herein, we provide echocardiographic images before, during, and after manual reversion of the left appendage. Misdiagnosis of this complication could have led to an additional unnecessary surgical procedure that could have impacted on the patient's morbidity.

Three-dimensional printing of a complex CHD to plan surgical repair.

We used three-dimensional printing technology to create an anatomical three-dimensional model of a very rare and complex cyanotic CHD in a newborn, consisting of double-outlet left ventricle, ventricular septal defect, and pulmonary stenosis. This case demonstrates how this new innovative technology allows better understanding of the anatomy in complex CHDs and permits to better plan the surgical repair.

Endothelial Function and Vascular Properties in Children with Sickle Cell Disease.

BACKGROUND: Sickle cell disease (SCD) is an inherited disorder characterized by recurrent painful crises with ischemia resulting from vascular occlusion. Adults with SCD have increased arterial stiffness and reduced flow-mediated dilation (FMD), due to impaired release of substances such as nitric oxide. AIM: We aimed to assess the vascular properties of carotid and brachial arteries in children with SCD compared with a control group without cardiovascular risk factors. METHODS: Thirty patients with SCD, mean age 12.3 ± 4.5 years, were prospectively enrolled. A control group was made up of 30 age- and gender-matched healthy subjects. Carotid intima-media thickness (IMT), cross-sectional compliance (CSC), cross-sectional distensibility (CSD), diastolic wall stress (DWS), incremental elastic modulus (Einc), and FMD were determined in both groups. RESULTS: There was no significant difference in FMD between the two groups (8.2 ± 5.0% in the SCD group vs. 9.3 ± 4.2% in the control group, P = 0.15). There was no significant correlation between FMD and age, hemoglobin, LDH level, or transcranial Doppler findings. CSD was significantly elevated in the SCD group (0.96 ± 0.44 vs. 0.59 ± 0.21, P = 0.0002), whereas DWS and Einc were significantly lower in the SCD group. CSC did not differ significantly between the two groups. CONCLUSIONS: Children with SCD have no marked endothelial dysfunction or change in arterial stiffness. These manifestations may be related to disease severity and duration. Changes may become evident later in life as the disease progresses.

Bis-chlorination of a hexapeptide-PCP conjugate by the halogenase involved in vancomycin biosynthesis.

Vancomycin is an important nosocomial antibiotic containing a glycosylated, cross-linked and doubly chlorinated heptapeptide backbone. During the biosynthesis of the vancomycin aglycone, two ß-hydroxytyrosine (Bht) residues are inserted at positions-2 and -6 into the heptapeptide backbone by a non-ribosomal peptide synthetase. A single flavin-dependent chlorinase (VhaA) is responsible for chlorinating both Bht residues at some ill-defined point in the assembly process. We show here using in vitro assays that VhaA is able to introduce a chlorine atom into each aromatic ring of both Bht residues at positions-2 and -6 of a peptide carrier protein-bound hexapeptide. The results suggest that VhaA can recognize and chlorinate two quite different sites within a linear hexapeptide intermediate during vancomycin biosynthesis.

Usefulness of perioperative transoesophageal echocardiography during paediatric cardiac surgery.

BACKGROUND: Paediatric transoesophageal echocardiography probes allow perioperative evaluation during paediatric congenital heart disease surgery. AIM: To assess the usefulness of perioperative transoesophageal echocardiography in evaluating the severity of residual lesions, based on the type of congenital heart disease repaired in paediatric patients. METHODS: A retrospective analysis was conducted on paediatric patients who underwent open-heart surgery at our tertiary centre over a four-year period. Perioperative transoesophageal echocardiography studies were performed, and residual lesions were classified as mild, moderate or severe. RESULTS: Overall, 323 procedures involving 310 patients with a median age of 13.8 (0.07-214.4) months and a median weight of 8.2 (2-96) kg at intervention were enrolled in the study. Twenty-one (6.5%) residual lesions led to immediate reintervention: severe right ventricular outflow tract obstruction (n=12); severe aortic regurgitation (n=3); superior vena cava stenosis (n=2); moderate residual ventricular septal defect (n=2); severe mitral regurgitation (n=1); and severe mitral stenosis (n=1). Three (0.9%) neonates had ventilation difficulties caused by the transoesophageal echocardiography probe having to be removed, but experienced no sequelae. CONCLUSION: Perioperative transoesophageal echocardiography is a safe procedure, providing information on severe residual lesions, leading to the immediate revision of several paediatric congenital heart disease cases.

Advancing paediatric cardiac imaging: a comprehensive analysis of the feasibility and accuracy of a novel 3D paediatric transoesophageal probe.

Aims: Pediatric transoesophageal echocardiography (TOE) probes have remained two-dimensional (2D) limiting their use compared to adults. While critical in pediatrics for interventions and post-surgery assessments, technological advancements introduced a three-dimensional (3D) pediatric TOE probe. This study assessed the new 3D pediatric TOE probe (GE 9VT-D) for feasibility, handling, and imaging quality. Methods and results: At Children's Hospital of Toulouse, 2-month prospective study enrolled children undergoing TOE with the new probe. All imaging modalities were rated by 2 operators using a 5-point Likert-type scale from 1 (very poor) to 5 (very good) quality. Forty-five children, median age 3.7 (range: 2 months-14.7 years) median weight 7.8 kg (range: 4.3-48 kg) underwent 60 TOEs: 25% pre-surgery, 45% post-surgery, 28% during percutaneous procedures, and 2% in intensive care. Probe handling was "very easy" in all cases without adverse events. The median score of 2D, 2D colour, pulsed Doppler and 3D were noted 5 out of 5 and continuous Doppler and 3D colour 4 out of 5. The 3D image quality remained consistent irrespective of the patient weighing above or below 7.8 kg (p = 0.72). Postoperative TOEs identified two cases needing further interventions, emphasizing its value in evaluating surgical outcomes and also for guiding percutaneous interventions. Conclusion: Our comprehensive evaluation demonstrates that the new 3D pediatric TOE probe is feasible and provides high-quality imaging in pediatric patients. The successful integration of this novel probe into clinical practice has the potential to enhance diagnostic accuracy and procedural planning, ultimately optimizing patient outcomes in pediatric cardiac care.

Tetralogy of Fallot: T-shaped infundibulotomy for pulmonary valve-sparing procedure.

This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14-64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.

Impact of 3D-printed models in meetings with parents of children undergoing interventional cardiac catheterisation.

Background: Paediatric interventional catheterisation has consistently improved in recent decades, with often highly successful outcomes. However, progress is still required in terms of the information delivered to parents and how parental anxiety is managed. Aim: To investigate the impact of cardiac printed models on improving parental understanding and alleviating anxiety before interventional catheterisation. Methods: The parents of children undergoing interventional cardiac catheterisation were prospectively enrolled in the study. A questionnaire highlighting knowledge and understanding of the condition and cardiac catheterisation per se was scored on a scale of 1-30. The State-Trait Anxiety Inventory (STAI), which generates current anxiety scores, was also used before and after the pre-catheterisation meeting. The "printing group" received an explanation of catheterisation using the device and a three-dimensional (3D) model, while the "control group" received an explanation using only the device and a manual drawing. Results: In total, 76 parents of 50 children were randomly assigned to a "control group" (n = 38) or "printing group" (n = 38). The groups were comparable at baseline. The level of understanding and knowledge improved after the "control group" and "printing group" meetings (+5.5±0.8 and +10.2±0.8; p < 0.0001 and p < 0.0001, respectively). A greater improvement was documented in the "printing group" compared to the "control group" (p < 0.0001). The STAI score also improved after the explanation was given to both groups (-1.8±0.6 and -5.6±1.0; p < 0.0001 and p < 0.0001). The greatest improvement was noted in the "printing group" (p = 0.0025). Most of the parents (35/38 from the "printing group") found the models to be extremely useful. Conclusion: 3D-printed models improve parental knowledge and understanding of paediatric cardiac catheterisation, thereby reducing anxiety levels.

Cannabis and Its Permissibility Status.

Cannabis has been used and misused to treat many disorders. Δ9-Tetrahydrocannabinol (THC) and cannabidiol (CBD) are the most important components of cannabis and could be used for recreational and medical purposes. The permissibility (Halal) status of cannabis is controversial, and its rational use is ambiguous. Global awareness and interest in cannabis use are increasing and its permissibility status, especially for recreational and medical purposes, needs to be addressed. Rationalizing the scientific value and Halal status of cannabis is useful for the rational use and maintenance of the compatible system. It is rare in Muslim countries to discuss the permissibility status of cannabis from the perspective of its value and composition. Using the analogy concept, the CBD component extracted from a cannabis plant in a clean and pure form is permissible to use in industry, particularly in cosmetics and pharmaceuticals. If THC component is present in <1% and CBD is >99%, the mixture is considered permissible as long as THC is not intentionally added or intentionally left, but is mainly present due to the limited capabilities and efficiency of the purification methods. However, any amount of THC prepared with the intention to be used as an intoxicant is considered non-Halal.

Transcatheter closure of a perimembranous ventricular septal defect with Nit-Occlud Lê VSD Coil: A French multicentre study.

BACKGROUND: Transcatheter perimembranous ventricular septal defect (pmVSD) closure remains challenging and is seldom used in France given the risk of atrioventricular block (AVB). pmVSD closure with the Nit-Occlud Lê VSD coil was recently introduced in France as an alternative to occluder devices. AIMS: To study the safety and feasibility of pmVSD closure with the Nit-Occlud Lê VSD coil. METHODS: All consecutives cases of pmVSD closure with the Nit-Occlud Lê VSD coil in 20 tertiary French centres were included between January 2015 and December 2018. RESULTS: Among 46 procedures in five centres, indications for pmVSD closure were left ventricle overload (76.1%), exertional dyspnoea (17.4%), history of infective endocarditis (4.3%) and mild pulmonary hypertension (2.2%). The median (interquartile [IQR]) age of the patients was 13.9 (5.7-31.8) years. Aneurismal tissue was identified in 91.3% of patients. VSD median (IQR) size was 8 (7-10) mm on the left ventricle side and 5 (4-6) mm on the right ventricle side. Implantation was successful in 40 patients (87.0%; 95% confidence interval [CI] 73.7-95.1%). Severe complications occurred in six patients (13.0%, 95% CI 4.9-26.3%), mainly severe haemolysis (8.7%, 95% CI 2.4-20.8%). One aortic valve lesion required surgical aortic valvuloplasty. Occurrence of severe complications was significantly related to the presence of haemolysis (P=0.001), residual shunt (P=0.007) and multi-exit VSD (P=0.005). Residual shunt was observed in 40% of cases with the implanted device shortly after closure and 15% after a median follow-up of 27 months. No immediate or delayed device embolization or complete AVB was recorded. CONCLUSION: pmVSD closure with the Nit-Occlud Lê VSD Coil is feasible in older children and adults. However, residual shunting (leading to haemolysis) is a dreaded complication that should not be tolerated. pmVSD closure with the Nit-Occlud Lê VSD as a therapeutic strategy remains controversial and is limited to selected patients.

Impact of Sophrology on cardiopulmonary fitness in teenagers and young adults with a congenital heart disease: The SOPHROCARE study rationale, design and methods.

BACKGROUND: Recent advances in the field of congenital heart disease (CHD) have significantly improved the overall prognosis. Now more attention is being given to health-related quality of life (HRQoL) and promotion of physical activity. Non-invasive relaxation therapy may be effective in cardiac patients concerned with exercise-induced dyspnoea. The SOPHROCARE randomised trial aims to assess the impact of Caycedian Sophrology on cardiopulmonary fitness in adolescents and young adults with CHD. METHODS: The SOPHROCARE trial is a nationwide, multicentre, randomised, controlled study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (8 Sophrology group sessions vs. no intervention). The primary outcome is the change in percent predicted maximum oxygen uptake (VO2max) between baseline and 12-month follow-up. A total of 94 patients in each group is required to observe a significant increase of 10% in VO2max with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (VE/VCO2 slope, ventilatory anaerobic threshold, oxygen pulse, respiratory response to hypercapnia), health-related quality of life score (PedsQL), physical and psychological status. CONCLUSION: After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. We sought to assess in the SOPHROCARE trial, if a Sophrology program, could improve exercise capacity and quality of life in youth with CHD. TRIAL REGISTRATION: Clinicaltrials.gov (NCT03999320).

A case report of mycotic pseudoaneurysm in childhood: an unusual complication of coarctation of the aorta.

BACKGROUND: We report on an unusual case of a 3 year-old girl with coarctation of the aorta complicated by mycotic pseudoaneurysm and infected with Streptococcus pneumoniae. CASE SUMMARY: The only symptoms and signs were fever and weak femoral pulses. The echocardiography confirmed a localised isthmic's coarctation. In order to complete the evaluation, a CT scan was performed. This proved crucial in terms of the diagnosis and decision to perform emergency surgery. The diagnosis was confirmed surgically. An aortic rupture was contained by the parietal pleura. Bacteriological analysis of surgical specimens revealed bacterial DNA that tested positive for Streptococcus pneumoniae. The post-operative course was free from any cardiovascular or neurological complications after six weeks of antibiotic therapy. DISCUSSION: Surgical repair of coarctation of the aorta is frequently performed in children. However, complicated forms are less common with a potentially fatal outcome. Indeed, there are no recommendations concerning the management and surgical timing of mycotic pseudoaneurysm. These rare complications should be kept in mind. Although short- and medium-term follow-up of these children is good, caution should be exercised with long-term follow-up because of complications in childhood and adulthood.

Feasibility of New Transthoracic Three-Dimensional Echocardiographic Automated Software for Left Heart Chamber Quantification in Children.

BACKGROUND: New three-dimensional echocardiographic automated software (HeartModel) is now available to quantify the left heart chambers. The aims of this study were to assess the feasibility, reproducibility, and analysis time of this technique and its correlation with manual three-dimensional echocardiography (3DE) and cardiac magnetic resonance (CMR) in children. METHODS: Ninety-two children (5-17 years of age) were prospectively included in two separate protocols. In protocol 1, 73 healthy children underwent two-dimensional and three-dimensional transthoracic echocardiography. Left ventricular (LV) end-diastolic volume (LVEDV), LV end-systolic volume (LVESV), LV ejection fraction (LVEF), and left atrial volume at ventricular end-systole (LAV) by automated 3DE were compared with the same measurements obtained using manual 3DE. In protocol 2, automated three-dimensional echocardiographic measurements from 19 children with cardiomyopathy were compared with CMR values. RESULTS: Automated 3DE was feasible in 77% of data sets and significantly reduced the analysis time compared with manual 3DE. In protocol 1, there were excellent correlations for LVEDV, LVESV, and LAV between automated 3DE and manual 3DE (r = 0.89 to 0.99, P < .0001 for all) and a weak correlation for LVEF, despite contour adjustment (r = 0.57, P < .0001). Automated 3DE overestimated LVEDV, LVEF, and LAV with small biases and underestimated LVESV with wider bias. With contour adjustment, the biases and limits of agreement were reduced (bias: LVEDV, 0.9 mL; LVESV, -1.2 mL; LVEF, 2.2%). In protocol 2, correlations between automated 3DE with contour edit and CMR were good for LV volumes and LAV (r = 0.76 to 0.94, P < .0003 for all) but remained weak for LVEF (r = 0.46, P = .05). Automated 3DE slightly underestimated LV volumes (relative bias, -7.2% to -7.8%) and significantly underestimated LAV (relative bias, -31.6%). The limits of agreement were clinically acceptable only for LVEDV. Finally, test-retest, intraobserver, and interobserver variability values were low (<12%). CONCLUSIONS: HeartModel is feasible, reproducible, faster than manual 3DE, and comparable with manual 3DE for measurements of LV and left atrial volumes in children >5 years of age. However, compared with CMR, only LVEDV measured by automated 3DE with contour edit seems applicable for clinical practice.

Cardiac strangulation: An atypical complication from epicardial pacemaker leads in a newborn.

We report a rare and serious complication of cardiac strangulation arising from the implantation of epicardial pacing leads in a newborn. Patient's follow-up 9-month postsurgery revealed compression under the pulmonary valve annulus by a pacemaker lead, causing progressive stenosis of the right ventricular outflow tract. The epicardial leads were replaced to relieve compression, and stenosis of the right ventricular outflow tract was rectified. Pacemaker implantation in newborns is not without challenges; epicardial leads should be carefully positioned to avoid any compression of cardiac structures.