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.

Long-term follow-up of percutaneous secundum-type atrial septal defect closure using Amplatzer Septal Occluder since 1995: a single-centre study.

INTRODUCTION: The goal of this single-centre study was to evaluate long-term results of percutaneous closure of secundum-type atrial septal defect using Amplatzer Septal Occluder with the follow-up to 25 years. METHODS: A retrospective analysis of patients who underwent percutaneous closure of secundum-type atrial septal defect between September 1995 and October 2012 in our institution was performed. All procedures were performed after fulfilling strict indication criteria. More than 5 years follow-up was reached in 651/803 patients (81%) with median follow-up time of 12 (5-25) years. RESULTS: The mean stretched defect diameter was 14,0 ± 5,2 mm. Early reintervention due to moderate or severe residual atrial septal defect had to be performed in 3/803 patients (0,03%). The incidence of long-term moderate or severe residual atrial septal defect was 0,0%. The complete closure rate at 10-year follow-up was 98,5%, as trivial residual shunts persisted in 8/508 patients (1,5%). A significant rate of the echocardiographic right ventricular end-diastolic diameter post-procedural normalisation (p < 0,05) was encountered. The rate of major complications was 0,5%. One device embolisation, one thrombus formation at the occluder surface, and one cardiac erosion in periprocedural or short post-procedural course were experienced. Only one late complication of infective endocarditis at the region of implanted occluder and the aortic valve was detected. The survival rate of all followed patients was 100%. CONCLUSIONS: Percutaneous closure of secundum-type atrial septal defect using Amplatzer Septal Occluder is a safe and effective procedure accounting for a very low incidence of major complications in the long-term follow-up.

Adult congenital heart disease training in Europe: current status, disparities and potential solutions.

OBJECTIVES: This study aimed to determine the status of training of adult congenital heart disease (ACHD) cardiologists in Europe. METHODS: A questionnaire was sent to ACHD cardiologists from 34 European countries. RESULTS: Representatives from 31 of 34 countries (91%) responded. ACHD cardiology was recognised by the respective ministry of Health in two countries (7%) as a subspecialty. Two countries (7%) have formally recognised ACHD training programmes, 15 (48%) have informal (neither accredited nor certified) training and 14 (45%) have very limited or no programme. Twenty-five countries (81%) described training ACHD doctors 'on the job'. The median number of ACHD centres per country was 4 (range 0-28), median number of ACHD surgical centres was 3 (0-26) and the median number of ACHD training centres was 2 (range 0-28). An established exit examination in ACHD was conducted in only one country (3%) and formal certification provided by two countries (7%). ACHD cardiologist number versus gross domestic product Pearson correlation coefficient=0.789 (p<0.001). CONCLUSION: Formal or accredited training in ACHD is rare among European countries. Many countries have very limited or no training and resort to 'train people on the job'. Few countries provide either an exit examination or certification. Efforts to harmonise training and establish standards in exit examination and certification may improve training and consequently promote the alignment of high-quality patient care.

Advanced glycation end products predict long-term outcome of catheter ablation in paroxysmal atrial fibrillation.

PURPOSE: Oxidative stress is an important contributor to the etiology of atrial fibrillation (AF). Our aim was to study oxidative stress biomarkers in patients undergoing pulmonary vein isolation (PVI) for paroxysmal AF with radiofrequency catheter ablation and to assess its prognostic value in predicting long-term PVI outcome. METHODS: In this prospective cohort study, we included 62 patients (mean age 55±8 years, 12 females and 50 males) with paroxysmal AF and implanted ECG loop recorders who underwent PVI. Plasmatic concentrations of advanced glycation end-products (AGEs), fructosamine, advanced oxidation protein products, and thiobarbituric-acid reacting substances were measured before PVI. AF burden (percentage of time spent in AF) was continually assessed during the follow-up period (1063±271 days). RESULTS: Nineteen patients (31%) were defined as optimal responders (oR) with AF burden < 0.5% after PVI. Remaining 43 patients (69%) were defined as sub-optimal responders. Concentration of AGEs was significantly lower in oR by 3.7 g/g (CI: -6.5 to -1.7; P=0.0003). After adjustment for age, sex, BMI, left atrial size, arterial hypertension, and AF burden before PVI, only low concentration of AGEs remained significantly associated with oR (odds ratio: 1.3; P=0.04). AGEs concentration achieved area under the curve of 0.78 for predicting optimal long-term PVI response. CONCLUSIONS: AGEs concentration before PVI was associated with long-term PVI outcome in patients with paroxysmal AF. Further research will show if this biomarker could contribute to optimal patient selection for catheter ablation.

Percutaneous treatment of a huge patent ductus venosus and severe portal vein hypoplasia using a Figulla Flex II atrial septal defect occluder in a 2-year-old infant: a case report.

BACKGROUND: Intra- or extrahepatic porto-caval shunts (PCSs) can account for multiorgan dysfunction with pulmonary arterial hypertension and portosystemic encephalopathy as the most serious consequences of bypass of the hepatic circulation. The ductus venosus (DV) represents a rare foetal PCS and might be persistently patent in newborns after birth. Treatment strategies include surgical ligation and percutaneous device closure. The degree of portal vein hypoplasia limits therapy making liver transplantation the only option in some of them. CASE SUMMARY: In a newborn female patient a huge persistently patent DV, known already prenatally, resulted in severe secondary portal vein hypoplasia. She presented with hyperammonaemia, elevated liver enzymes, and pulmonary hypertension. With only diminutive portal venous branches and exceedingly high portal venous pressures during test-occlusion of the DV, shunt closure was not possible. At the age of 2 years more favourable portal venous pressures allowed transcatheter device closure with a nitinol atrial septal defect occlusion device. Pulmonary artery pressures and ammonia levels normalized after the procedure without any signs of portal hypertension. DISCUSSION: The case highlights the importance of meticulous imaging using balloon occlusion angiography of PCSs like the DV, to search for intrahepatic portal veins. Moreover, portal vein pressure during test-occlusion can identify patients amenable for surgical or endovascular shunt closure. Occlusion devices licensed for other indications like atrial septal defect closure can be used safely in huge PCS vessels in a one-step or staged procedure. Optimal timing of the intervention should be tailored to the patient's needs.

Association Between Apelin and Atrial Fibrillation in Patients With High Risk of Ischemic Stroke.

Background: Atrial fibrillation (AF) is associated with high risk of stroke preventable by timely initiation of anticoagulation. Currently available screening tools based on ECG are not optimal due to inconvenience and high costs. Aim of this study was to study the diagnostic value of apelin for AF in patients with high risk of stroke. Methods: We designed a multicenter, matched-cohort study. The population consisted of three study groups: a healthy control group (34 patients) and two matched groups of 60 patients with high risk of stroke (AF and non-AF group). Apelin levels were examined from peripheral blood. Results: Apelin was significantly lower in AF group compared to non-AF group (0.694 ± 0.148 vs. 0.975 ± 0.458 ng/ml, p = 0.001) and control group (0.982 ± 0.060 ng/ml, p < 0.001), respectively. Receiver operating characteristic (ROC) analysis of apelin as a predictor of AF scored area under the curve (AUC) of 0.658. Apelin's concentration of 0.969 [ng/ml] had sensitivity = 0.966 and specificity = 0.467. Logistic regression based on manual feature selection showed that only apelin and NT-proBNP were independent predictors of AF. Logistic regression based on selection from bivariate analysis showed that only apelin was an independent predictor of AF. A logistic regression model using repeated stratified K-Fold cross-validation strategy scored an AUC of 0.725 ± 0.131. Conclusions: Our results suggest that apelin might be used to rule out AF in patients with high risk of stroke.

Supraventricular tachyarrhythmias during the intrauterine, neonatal, and infant period: A 10-year population-based study.

BACKGROUND: We aimed to evaluate the incidence, type, and management of supraventricular tachyarrhythmias (SVT) during the first year of life in a retrospective, population-based, single-center study during a 10-year period. METHODS: The analyzed patient cohort is based on data from the only specialized center managing all cases of neonatal and infant SVTs between 2009 and 2018 in the Slovak Republic (5.5 million population). A total of 116 consecutive patients <366 days old were included in the study. RESULTS: Calculated SVT incidence ratio was 1:4500 in the first year of life. AV reentry tachycardia was the leading arrhythmia (49%). SVT in this specific population was frequently a transient problem with spontaneous resolution in 87% of patients during a median 3-year follow up. Congenital heart disease was common (16%). Intrauterine treatment by drugs administered to mother was safe and effective in preventing unnecessary cesarean deliveries. In arrhythmia termination, amiodarone and propafenone were equally safe and effective, with possible more favorable pharmacodynamics of the former. For prophylactic treatment, sotalol and propafenone were equally safe and effective and became the preferred basis of long-term drug therapy in our center. However, this therapy requires intensive monitoring during its initiation. CONCLUSION: The prognosis of SVT in the first year of life is good: with optimized pharmacological treatment, the need for early catheter ablation and mortality rate are low (<1%) and there is a high rate of spontaneous arrhythmia resolution. Heart failure is a possible predictor of arrhythmia persistence with need for ablation in later life.

Right aortic arch with isolated left subclavian artery: a rare association with coarctation of the left pulmonary artery.

We present a case report of rare association of right aortic arch with isolated left subclavian artery with coarctation of the left pulmonary artery.

Utilisation of three-dimensional printed heart models for operative planning of complex congenital heart defects.

BACKGROUND AND AIM: To evaluate the accuracy of the three-dimensional (3D) printing of cardiovascular structures. To explore whether utilisation of 3D printed heart replicas can improve surgical and catheter interventional planning in patients with complex congenital heart defects. METHODS: Between December 2014 and November 2015 we fabricated eight cardiovascular models based on computed tomography data in patients with complex spatial anatomical relationships of cardiovascular structures. A Bland-Altman analysis was used to assess the accuracy of 3D printing by comparing dimension measurements at analogous anatomical locations between the printed models and digital imagery data, as well as between printed models and in vivo surgical findings. The contribution of 3D printed heart models for perioperative planning improvement was evaluated in the four most representative patients. RESULTS: Bland-Altman analysis confirmed the high accuracy of 3D cardiovascular printing. Each printed model offered an improved spatial anatomical orientation of cardiovascular structures. CONCLUSIONS: Current 3D printers can produce authentic copies of patients` cardiovascular systems from computed tomography data. The use of 3D printed models can facilitate surgical or catheter interventional procedures in patients with complex congenital heart defects due to better preoperative planning and intraoperative orientation.