Organisation of paediatric echocardiography laboratories and governance of echocardiography services and training in Europe: current status, disparities, and potential solutions. A survey from the Association for European Paediatric and Congenital Cardiology (AEPC) imaging working group.

BACKGROUND: There is limited data on the organisation of paediatric echocardiography laboratories in Europe. METHODS: A structured and approved questionnaire was circulated across all 95 Association for European Paediatric and Congenital Cardiology affiliated centres. The aims were to evaluate: (1) facilities in paediatric echocardiography laboratories across Europe, (2) accredited laboratories, (3) medical/paramedical staff employed, (4) time for echocardiographic studies and reporting, and (5) training, teaching, quality improvement, and research programs. RESULTS: Respondents from forty-three centres (45%) in 22 countries completed the survey. Thirty-six centres (84%) have a dedicated paediatric echocardiography laboratory, only five (12%) of which reported they were European Association of Cardiovascular Imaging accredited. The median number of echocardiography rooms was three (range 1-12), and echocardiography machines was four (range 1-12). Only half of all the centres have dedicated imaging physiologists and/or nursing staff, while the majority (79%) have specialist imaging cardiologist(s). The median (range) duration of time for a new examination was 45 (20-60) minutes, and for repeat examination was 20 (5-30) minutes. More than half of respondents (58%) have dedicated time for reporting. An organised training program was present in most centres (78%), 44% undertake quality assurance, and 79% perform research. Guidelines for performing echocardiography were available in 32 centres (74%). CONCLUSION: Facilities, staffing levels, study times, standards in teaching/training, and quality assurance vary widely across paediatric echocardiography laboratories in Europe. Greater support and investment to facilitate improvements in staffing levels, equipment, and governance would potentially improve European paediatric echocardiography laboratories.

Evaluation of the total hydrodynamic energy loss using 4D flow MRI in a case with Fontan failure.

Fontan Failure (FF) is a common problem for single-ventricle patients as they reach adulthood. Although several mechanisms may cause FF, an optimized blood flow stream through the surgical conduits is essential to avoid excessive energy loss (EL). Recent clinical studies showed EL is related to the quality of life, exercise capacity, and hepatic function since the single-ventricle feeds pulmonary and systemic circulation serially. 4D flow MRI effectively estimates EL in Fontan circulation and allows clinicians to compare the effectiveness of the treatment strategy concerning pre-intervention. Here, we present 26-year-old women with FF who had normal cardiac catheterization findings and were treated according to high EL definitions that are measured through 4D flow MRI.

Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.

BACKGROUND AND AIMS: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. METHODS: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. RESULTS: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively. CONCLUSIONS: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs.

Approach to red blood cell transfusions in post-operative congenital heart disease surgery patients: when to stop?

BACKGROUND: The best transfusion approach for CHD surgery is controversial. Studies suggest two strategies: liberal (haemoglobin ≤ 9.5 g/dL) and restrictive (waiting for transfusion until haemoglobin ≤ 7.0 g/dL if the patient is stable). Here we compare liberal and restrictive transfusion in post-operative CHD patients in a cardiac intensive care unit. METHODS: Retrospective analysis was conducted on CHD patients who received liberal transfusion (2019-2021, n=53) and restrictive transfusion (2021-2022, n=43). RESULTS: The two groups were similar in terms of age, gender, Paediatric Risk of Mortality-3 score, Paediatric Logistic Organ Dysfunction-2 score, Risk Adjustment for Congenital Heart Surgery-1 score, cardiopulmonary bypass time, vasoactive inotropic score, total fluid balance, mechanical ventilation duration, length of cardiac intensive care unit stay, and mortality. The liberal transfusion group had a higher pre-operative haemoglobin level than the restrictive group (p < 0.05), with no differences in pre-operative anaemia. Regarding the minimum and maximum post-operative haemoglobin levels during a cardiac intensive care unit stay, the liberal group had higher haemoglobin levels in both cases (p<0.01 and p=0.019, respectively). The number of red blood cell transfusions received by the liberal group was higher than that of the restrictive group (p < 0.001). There were no differences between the two groups regarding lactate levels at the time of and after red blood cell transfusion. The incidence of bleeding, re-operation, acute kidney injury, dialysis, sepsis, and systemic inflammatory response syndrome was similar. CONCLUSIONS: Restrictive transfusion may be preferable over liberal transfusion. Achieving similar outcomes with restrictive transfusions may provide promising evidence for future studies.

Carotid artery cut-down technique for ductus arteriosus stenting.

Background: This study aims to evaluate early and mid-term outcomes of ductal stenting via carotid artery surgical cut-down technique in neonates. Methods: Between January 2015 and January 2022, a total of 17 neonates (12 males, 5 females; median age: 14 days, range, 5 to 34 days) who underwent carotid artery surgical cut-down technique for ductal stenting were retrospectively analyzed. Diagnoses of the patients, demographics, procedural success/failure, access-related complications, and neuroimaging findings were recorded. Results: The primary indication for ductal stenting was pulmonary atresia in all patients. All patients who underwent carotid cut-down had vertical anatomy, with or without tortuous ductal anatomy, and they were not suitable for the femoral approach. The median body weight was 3 (range, 2 to 3.4) kg. Fifteen of the 17 interventions (88.2%) were successful. Two patients whose stenting failed underwent a systemic-to-pulmonary shunt operation. The early in-hospital mortality rate was 17.6% (n=3). No neurological or accessrelated complications were observed in any of the patients. Conclusion: Stenting the ductus arteriosus with challenging anatomy is feasible and safe with carotid artery cut-down, particularly in small neonates. Based on our study findings, this technique may offer an effective and less invasive alternative to the systemic-to-pulmonary shunt operation.

A rare complication after an interventional procedure using the common carotid: carotid pseudoaneurysm in an infant.

The carotid artery is a valuable vascular access that can be used in patients who have undergone repetitive interventional and surgical procedures and premature babies. In the past, cut-down was used but nowadays, mostly the procedure is performed under ultrasonographic guidance. Complications such as bleeding, haematoma, and pseudoaneurysm may occur when the carotid artery is used as a vascular access for the procedures such as aortic balloon valvuloplasty, coarctation balloon angioplasty, or after interventional or surgical treatments to the carotid artery. Although pseudoaneurysm is very rare, prompt diagnosis and accurate treatment planning are life-saving. In this article, the diagnosis and treatment of pseudoaneurysm in the left common carotid after transcatheter coarctation balloon angioplasty in a 6-month-old infant will be presented.

Early results of Pulsta® transcatheter heart valve in patients with enlarged right ventricular outflow tract and severe pulmonary regurgitation due to transannular patch.

OBJECTIVE: The purpose of this study is to assess the feasibility, effectivity, and safety of a novel self-expandable valve system, Pulsta® transcatheter heart valve in patients with tetralogy of fallot and severe pulmonary regurgitation after transannular patch repair. BACKGROUND: Severe pulmonary regurgitation after tetralogy of fallot repair is a life-threatening problem and should be treated by pulmonary valve implantation. Although percutaneous pulmonary valve implantation has been ever increasingly used for this purpose, available balloon-expandable valves have limitations and cannot be used by most patients. Pulsta® transcatheter heart valve is a new self-expandable valve system and offers a new solution to be used in patients with different types of native right ventricular outflow tract geometry. PATIENTS AND METHODS: Ten patients with severe regurgitation after tetralogy of fallot repair with a transannular patch have been enrolled in the study according to echocardiographic examination. MRI was used in asymptomatic patients to delineate the indication and the right ventricular outflow tract geometry. Pulsta® transcatheter heart valve implantation was performed in ten patients, and preprocedural, procedure, and 6 months follow-up findings of the patients were evaluated. RESULTS: Pulsta® pulmonary valve implantation was performed in ten patients successfully without any severe complications. Valve functions were perfect in six of ten patients, while the others had insignificant regurgitation by echocardiographic examination at the end of 6 months follow-up. CONCLUSIONS: This study showed that Pulsta® transcatheter heart valve is a feasible, effective, and safe method in the treatment of severe pulmonary regurgitation due to transannular patch repair in patients with tetralogy of fallot.

Early and mid-term outcomes of transcatheter closure of perimembranous ventricular septal defects using the Lifetech™ Konar-MF Occluder device (MFO).

BACKGROUND: Transcatheter closure of perimembranous ventricular septal defects is challenging. Double disk-designed devices have high adverse event rates; therefore, research into new devices persists. One such devise is the LifetechTM Konar-MF Occluder device (MFO), which is increasingly used. The purpose of this study is to present mid-term results of MFO for transcatheter closure of patients with perimembraneous ventricular septal defect. PATIENTS AND METHODS: Records of the 52 patients with perimembraneous ventricular septal defect who had undergone transcatheter closure with MFO were reviewed. Demographic, echocardiographic, and procedure details were investigated. Three years follow-up results were recorded. RESULTS: We closed the perimembraneous ventricular septal defect in 51 of 52 patients (98%). The femoral venous approach was used in 27 patients (53 %) whilst no arteriovenous loop was established in the remaining patients. No significant procedure-related complication occurred. On day 1 echocardiography, the residual ventricular septal defect rate was 31%. Mean±SD (range) follow-up duration was 36 ± 7,9 (18-54) months. In the follow-up, at 6th months, only four patients had hemodynamically insignificant residual defects. No severe dysrhythmia was detected including complete heart block. A right bundle branch block pattern was seen in one patient. CONCLUSION: This study showed that MFO is a safe and effective device in the transcatheter treatment of perimembraneous ventricular septal defect's with mid-term follow-up.

Sequential percutaneous tricuspid and pulmonary valve implantation in a young child operated previously for Ebstein Anomaly.

We report a successful percutaneous tricuspid valve implantation followed by a percutaneous pulmonary valve implantation in a young child with Ebstein's anomaly of tricuspid valve and pulmonary stenosis who was previously treated surgically at 1 year of age with tricuspid ring annuloplasty and a transannular outflow patch. This article shows the feasibility of sequential implantation of two valves in young patients with severe tricuspid and pulmonary valve insufficiency.

Early postnatal metabolic profile in neonates with critical CHDs.

BACKGROUND: Cyanotic CHD is a life-threatening condition that presents with low oxygen saturation in the newborn period. Hypoxemia might cause alterations in the metabolic pathways. In the present study, we aimed to evaluate the early postnatal amino acid and carnitine/acylcarnitine profiles of newborn infants with cyanotic CHD. METHODS: A single centre case-control study was conducted. Twenty-seven patients with cyanotic CHD and 54 healthy newborn controls were enrolled. As part of the neonatal screening programme, results of amino acid and carnitine/acylcarnitine were recorded and compared between groups. RESULTS: Twenty-seven neonates with cyanotic CHD and 54 healthy newborns as controls were enrolled in the study. Cyanotic CHD neonates had higher levels of alanine, phenylalanine, leucine/isoleucine, citrulline, ornithine, C5, C5-OH; but lower levels of C3, C10, C12, C14, C14:1, C16, C16.1, C18, C5-DC, C6-DC, C16-OH, C16:1-OH when compared with the healthy controls. CONCLUSION: This study showed that there are differences between patients with cyanotic CHD and healthy controls in terms of postnatal amino acid and carnitine/acylcarnitine profiles.

Transcatheter management of life-threatening pulmonary arteriovenous fistula with extracorporeal membrane oxygenation support in an infant.

Pulmonary arteriovenous malformation is a rare disease leading to cyanosis, where there is a direct relation between the pulmonary artery and pulmonary vein without a capillary structure. Arteriovenous fistulae may be single or multiple. Clinical signs emerge depending on the size of the fistulae and amount of shunt. Due to the advancements in transcatheter devices and increased experience render enable the fistula embolisation procedure as an alternative to surgical treatment. Extracorporeal membrane oxygenation is used to support the patient haemodynamically and respirationally in cases of treatment-resistant, severe and revocable cardiac or pulmonary sufficiency. This paper presents an infant patient with pulmonary arteriovenous malformation, who had haemodynamic instability due to severe hypoxia and received successful transcatheter fistula embolisation via extracorporeal membrane oxygenation under emergency conditions.

Optimizing percutaneous pulmonary valve implantation with patient-specific 3D-printed pulmonary artery models and hemodynamic assessment.

Background: Percutaneous pulmonary valve implantation (PPVI) has emerged as a less invasive alternative for treating severe pulmonary regurgitation after tetralogy of Fallot (TOF) repair in patients with a native right ventricular outflow tract (RVOT). However, the success of PPVI depends on precise patient-specific valve sizing, the avoidance of oversizing complications, and optimal valve performance. In recent years, innovative adaptations of commercially available cardiovascular mock loops have been used to test conduits in the pulmonary position. These models are instrumental in facilitating accurate pulmonic valve sizing, mitigating the risk of oversizing, and providing insight into the valve performance before implantation. This study explored the utilization of custom-modified mock loops to implant patient-specific 3D-printed pulmonary artery geometries, thereby advancing PPVI planning and execution. Material and Methods: Patient-specific 3D-printed pulmonary artery geometries of five patients who underwent PPVI using Pulsta transcatheter heart valve (THV) ® were tested in a modified ViVitro pulse duplicator system®. Various valve sizes were subjected to 10 cycles of testing at different cardiac output levels. The transpulmonary systolic and regurgitation fractions of the valves were also recorded and compared. Results: A total of 39 experiments were conducted using five different patient geometries and several different valve sizes (26, 28, 30, and 32 mm) at 3, 4, and 5 L/min cardiac output at heart rates of 70 beats per minute (bpm) and 60/40 systolic/diastolic ratios. The pressure gradients and regurgitation fractions of the tested valve sizes in the models were found to be similar to the pressure gradients and regurgitation fractions of valves used in real procedures. However, in two patients, different valve sizes showed better hemodynamic values than the actual implanted valves. Discussion: The use of 3D printing technology, electromagnetic flow meters, and the custom-modified ViVitro pulse duplicator system® in conjunction with patient-specific pulmonary artery models has enabled a comprehensive assessment of percutaneous pulmonic valve implantation performance. This approach allows for accurate valve sizing, minimization of oversizing risks, and valuable insights into hemodynamic behavior before implantation. The data obtained from this experimental setup will contribute to advancing PPVI procedures and offer potential benefits in improving patient outcomes and safety.

Treatment of 12-year-old girl with midaortic syndrome by using Cook Formula stent.

Middle aortic syndrome is a severe form of aortic coarctation and mostly involves the thoracic and abdominal part of the aorta. Traditional surgical approach has some potential risks and repetitive operations. Percutaneous stent implantation is a feasible and safe method for the palliation of middle aortic syndrome in childhood. Herein, a child with middle aortic syndrome was presented who has been treated by transcatheter stent implantation.

Does a prenatal diagnosis affect mortality and morbidity for neonatal arterial switch operation.

BACKGROUND: The effect of prenatal diagnosis on prognosis in patients with transposition of the great arteries is not clear. In this study, we compared the outcomes after arterial switch operation. METHODS: Outcome of 112 patients who had arterial switch operation in the neonatal period were analysed. The patients were divided into two groups: those who had prenatal diagnosis (Group 1; n = 34) and those who did not (Group 2; n = 78). The patients were also classified based on their diagnosis: simple transposition, transposition with ventricular septal defect and/or aortic arch hypoplasia, and Taussig-Bing anomaly. RESULTS: In Group 1, the C-section delivery rate was higher (82% vs. 44%; p = 0.004), and it was observed that patients in Group 1 were more often intubated upon admission to the neonatal ICU (38% vs. 9%; p = 0.005). No differences were found between the two groups in terms of operation time, cardiopulmonary bypass time, post-operative invasive respiratory support duration, or extracorporeal membrane oxygenation support. It was observed that those who had Taussig-Bing anomaly had a higher mortality. CONCLUSIONS: Timely treatment have a positive effect on neonatal mortality and morbidity. That's why all families with prenatal diagnosis of critical CHD should be recommended to have the delivery in a tertiary care hospital. Although it could not be demonstrated in this study, prenatal diagnosis has a potential to improve surgical results especially in countries or cities, which does not have enough resources for transfer and surgical units. Further efforts are needed to improve prenatal screening programmes.

First experiences with Myval Transcatheter Heart Valve System in the treatment of severe pulmonary regurgitation in native right ventricular outflow tract and conduit dysfunction.

The rate of morbidity and mortality related to pulmonary regurgitation and pulmonary stenosis are big concerns after the surgery for CHD. Percutaneous pulmonary valve implantation has been established as a less invasive technique compared to surgery with promising results according to long-term follow-up of the patients. There are only two approved valve options for percutaneous pulmonary valve implantation until now, which are Melody (Medtronic, Minneapolis, Minn, USA) and Sapien (Edwards Lifesciences, Irvine, Ca, USA). Both valves have limitations and do not cover entire patient population. Therefore, the cardiologists need more options to improve outcomes with fewer complications in a such promising area. Herein, we present a case series applying for pulmonary position in conduits and native right ventricular outflow tract of a new transcatheter valve system Myval ® which is designed for transcatheter aortic valve implantation procedures. This is the first patient series in which the use of Myvalv in dysfunctional right ventricular outflow tracts is described, after surgical repair of CHD.

Management of Interrupted Aortic Arch With Associated Anomalies: A Single-Center Experience.

OBJECTIVES: Interrupted aortic arch (IAA) includes a broad spectrum of associated anomalies. In this study, we present our surgical management and patient-specific decisions regarding IAA anomalies with early- and mid-term outcomes. METHODS: The medical records of 25 patients undergoing IAA repair between 2014 and 2019 were retrospectively reviewed. Sixteen patients had type B (64%) interruptions, 7 had type A (28%) interruptions, and 2 had type C (8%) interruptions. Fourteen patients had an isolated ventricular septal defect, and 3 of them had associated left ventricular outflow tract obstruction. Other associated anomalies were functional single ventricle (n = 5), Taussig-Bing anomaly (n = 3), aortopulmonary window (n = 1), multiple ventricular septal defects (n = 1), and truncus arteriosus with dextrocardia (n = 1). The initial operation age was 17.2 ± 14 (range: 1 - 60) days. RESULTS: Single-stage total repair was performed for 15 patients. Six patients underwent aortic arch repair and pulmonary artery banding. Four patients with left ventricular outflow tract obstruction or who were premature underwent the hybrid procedure. The aortic arch repair was performed in 16 cases (64%) by the anterior patch augmentation technique, in 3 cases (12%) by the reverse left subclavian artery flap technique, and in 3 cases (12%) by direct end-to-end anastomosis. Postoperative early mortality occurred in 4 (16%) patients, and sternal closure was delayed in 13 (52%) patients. Three patients who underwent a hybrid procedure due to left ventricular outflow tract obstruction underwent biventricular repair 8 to 13 months later. Eight patients (38%) required reintervention due to arch restenosis during the follow-up period. The mean follow-up was 37.1 ± 21.7 months. CONCLUSION: Planning surgical treatment according to the characteristics of the patients and accompanying anomalies may improve the results.

Evaluation of neonatal acute kidney injury after critical congenital heart disease surgery.

BACKGROUND: Acute kidney injury (AKI) is a common complication of congenital heart diseases (CHDs) after cardiac surgery. This study aimed to define the frequency and critical course, risk factors and short-term outcomes of AKI in postoperative CHD neonates. METHODS: Postoperatively followed term CHD newborn infants were enrolled in the study. Infants with congenital anomalies of the urinary tract and other major congenital anomalies were excluded. Neonatal modified KDIGO criteria were used to assess AKI. RESULTS: A total of 199 postoperatively followed newborn infants were included in the study. Acute kidney injury was detected in 71 (35.6%) patients. Of these patients, 24 (33.8%) were in stage 1, 14 (19.7%) in stage 2, and 33 (46.5%) in stage 3. Acute kidney injury occurred within the first week (median 1 day [IQR 1-2 days]) of cardiac surgery in 93% of the patients. The duration of invasive respiratory support and extracorporeal membrane oxygenation (ECMO) and mortality were significantly higher in stage 3 patients. Higher vasoactive-inotropic score (OR, 1.02; 95% CI, 1.0-1.04; p = 0.008) and receiving ECMO (OR, 7.9; 95% CI, 2.6-24.4; p = 0.001) were associated with risk for the development of AKI. The mortality rate was 52.1% in the AKI (+) patients, and having AKI (OR 7.1; 95% CI, 3.5-14.18) was significantly associated with mortality. CONCLUSION: Acute kidney injury, a common early complication after critical neonatal CHD cardiac surgery, is associated with increased morbidity and mortality. Stage 3 AKI is associated with significantly higher mortality rates.