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The recommendations of the Association for European Paediatric and Congenital Cardiology for basic training in paediatric and congenital cardiology required to be recognised as a paediatric cardiologist by the Association for European Paediatric and Congenital Cardiology are described below. Those wishing to achieve more advanced training in particular areas of paediatric cardiology should consult the training recommendations of the different Association for European Paediatric and Congenital Cardiology Working Groups available on the Association for European Paediatric and Congenital Cardiology website (www.aepc.org) and the respective publications 1–6. The development of training requirements is the responsibility of the Educational Committee and the Association for European Paediatric and Congenital Cardiology Council in collaboration with the Working Groups of the Association for European Paediatric and Congenital Cardiology. Trainees should be exposed to all aspects of general paediatric and congenital cardiology from fetal life to adolescence and adulthood. Centres performing generalised and specialised work in paediatric and congenital cardiology should be committed to deliver postgraduate training. At each training institute, trainers should be appointed to supervise and act as mentors to the trainees. Association for European Paediatric and Congenital Cardiology will provide basic teaching courses to supplement the training process.

Percutaneous treatment of aortic coarctation is based on angioplasty and/or stenting of the isthmus. We report a case of a 28-year-old girl suffering from aortic coarctation syndrome (coarctation + ventricular septal defect + bicuspid aorta). She underwent coarctectomy with subclavian flap and pulmonary bandage followed by ventricular septal defect closure and bandage removal in her first year of life. When she was 27 years old, a follow-up echocardiography detected an isthmic pressure gradient and a demodulated Doppler in abdominal aorta. A cardiac catheterisation confirmed the diagnosis of aortic re-coarctation. An AndraStent® XL 48 mm was implanted with a resolution of the isthmic gradient. One year later, because of the reappearance of demodulated Doppler in abdominal aorta, a chest X-ray was performed, which showed a stent third-grade fracture. The fracture was corrected by positioning a covered stent cheatham platinum 45 mm through the fragments. The rarest complication after stenting procedures is the fracture of the device with an incidence between 0.01% and 0.08%. Pressure overload beyond the elastic threshold of the material and the pulsatile tension exerted by the blood flow on the walls of the stent are the main mechanisms at the base of the fracture, together with the compliance of the tissue. A vessel that underwent multiple surgical rearrangements could interfere with and complicate the physiopathology at the basis of the fracture. In conclusion, stenting is a safe technique to treat aortic coarctation; stent fracture is a rare event, and different anatomical and haemodynamic factors are related to this complication.

Background:Limited data exist on training of European paediatric and adult congenital cardiologists.Methods:A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.Results:Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87–9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63–10.72 million), and one training centre per 4.29 million population (range 1.63–10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1–17), and duration of training was 3 years (range 2–5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41).Conclusion:Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.

The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting. The aim of this paper is to present the AEPC position on frequently asked questions based on the most recent scientific data, as well as to frame a discussion on how to take care of our patients during this unprecedented crisis. As the times are changing quickly and information regarding COVID-19 is very dynamic, continuous collection of evidence will help guide constructive decision-making.

AIM In complex congenital heart diseases (CHD), patients may remain affected by significant morbidity and mortality after surgery. We analysed the end-of-life (EoL) care in children with severe CHD who died in our institution and investigated perspectives of parents and health-care professionals (HCPs). METHODS Medical records of all children (age

Percutaneous treatment of aortic coarctation is based on angioplasty and/or stenting of the isthmus. We report a case of a 28-year-old girl suffering from aortic coarctation syndrome (coarctation + ventricular septal defect + bicuspid aorta). She underwent coarctectomy with subclavian flap and pulmonary bandage followed by ventricular septal defect closure and bandage removal in her first year of life. When she was 27 years old, a follow-up echocardiography detected an isthmic pressure gradient and a demodulated Doppler in abdominal aorta. A cardiac catheterisation confirmed the diagnosis of aortic re-coarctation. An AndraStent® XL 48 mm was implanted with a resolution of the isthmic gradient. One year later, because of the reappearance of demodulated Doppler in abdominal aorta, a chest X-ray was performed, which showed a stent third-grade fracture. The fracture was corrected by positioning a covered stent cheatham platinum 45 mm through the fragments. The rarest complication after stenting procedures is the fracture of the device with an incidence between 0.01% and 0.08%. Pressure overload beyond the elastic threshold of the material and the pulsatile tension exerted by the blood flow on the walls of the stent are the main mechanisms at the base of the fracture, together with the compliance of the tissue. A vessel that underwent multiple surgical rearrangements could interfere with and complicate the physiopathology at the basis of the fracture. In conclusion, stenting is a safe technique to treat aortic coarctation; stent fracture is a rare event, and different anatomical and haemodynamic factors are related to this complication.

Purpose To define weight-stratified Diagnostic Reference Levels (DRL) typical values for pediatric interventional cardiology (IC) procedures adopting standardized methodologies proposed by ICRP135 and RP185. Methods Procedures performed at the pediatric catheterization room of the University-Hospital of Padua were analysed. Patients were stratified into body weight (BW) classes and DRL quantities were analysed for the most performed procedures. Typical values are defined as median PKA and Ka,r. For database consistency, sampling and exclusion methods were precisely defined. The DRL-curve methodology by means of quantile regression median curves was investigated to assess the relationship between PKA and weight. A like-to-like comparison with literature was made. Results 385 procedures were analysed. A large PKA variability was observed in each weight group. PKA differences across BW groups were not always statistically significant. When stratifying by procedure, PKA variability decreased while correlations of PKA and PKA/FT with weight increased. The established typical values are generally lower than DRLs published data, whatever stratification method adopted. The highest PKA median values were observed for Angioplasty (4.9 and 11.6 Gycm2 for 5- Conclusions Typical values for pediatric IC DRL quantities were determined according to ICRP135 and RP185 methodologies. Stratification by BW classification does not reduce the variability of the PKA values, unlike what happens when stratifying by procedure type. Results seem to corroborate that variability and exposure are more affected by procedure type and complexity than by patient weight. DRL-curve is a feasible approach.

Background: Despite recent technical advances, interventional cardiac catheterization is still challenging in neonatal age and no specific data concerning early outcome are so far published in literature. Methods: Neonatal trans-catheter cardiac interventions performed in high-volume Italian referral centers were retrospectively analyzed. Primary outcomes were procedural major adverse events, in-hospital mortality and procedural failure. Secondary outcomes were minor adverse events and need for blood transfusion. Results: From January 2000 to December 2017, 1423 newborns (mean weight 3.0 ± 0.6 kg, range 1.0–5.8; median age 2.0 days) underwent interventional cardiac catheterization. Overall, global procedure adverse event rate and in-hospital mortality were 10.2% and 5.2%, respectively. At multi-variable analysis, primary composite outcome was significantly related to low-weight (

Background: The aim of the study was to document cardiovascular clinical findings, cardiac imaging and laboratory markers in children presenting with the novel multisystemic inflammatory syndrome associated with COVID-19. Methods: A real-time internet based survey was sent via the member mailing database for Association for European Paediatric and Congenital Cardiologists (AEPC) working groups for Cardiac Imaging and Cardiovascular Intensive Care member. Inclusion criteria was children 0-18 years admitted to hospital between March 1 and June 6, 2020 with diagnosis of an inflammatory syndrome and acute cardiovascular complications. Findings: A total of 286 children from 55 centres from 17 European countries were included. The median age was 8·4 years (IQR 3·8-12·4 years) and 67% were males. Most common cardiovascular complications were shock (40%), cardiac arrhythmias (35%), pericardial effusion (28%) and coronary artery dilatation (24%). Reduced left ventricular ejection fraction was present in 52% of patients and 93% had raised cardiac troponin (cTnT). The biochemical markers of inflammation were raised in majority of patients on admission: elevated CRP (99%), ferritin (79%), procalcitonin (96%), NT-proBNP (93%), IL-6 level (88%) and D-dimers (90%). There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and need of intensive care support (p

Background RASopathies are a set of relatively common autosomal dominant clinically and genetically heterogeneous disorders. Cardiac outcomes in terms of mortality and morbidity for common heart defects (such as pulmonary valve stenosis and hypertrophic cardiomyopathy) have been reported. Nevertheless, also Atypical Cardiac Defects (ACDs) are described. The aim of the present study was to report both prevalence and cardiac outcome of ACDs in patients with RASopathies. Methods A retrospective, multicentric observational study (CArdiac Rasopathy NETwork-CARNET study) was carried out. Clinical, surgical, and genetic data of the patients who were followed until December 2019 were collected. Results Forty-five patients out of 440 followed in CARNET centers had ACDs. Noonan Syndrome (NS), NS Multiple Lentigines (NSML) and CardioFacioCutaneous Syndrome (CFCS) were present in 36, 5 and 4 patients, respectively. Median age at last follow-up was 20.1 years (range 6.9-47 years). Different ACDs were reported, including mitral and aortic valve dysfunction, ascending and descending aortic arch anomalies, coronary arteries dilation, enlargement of left atrial appendage and isolated pulmonary branches diseases. Five patients (11%) underwent cardiac surgery and one of them underwent a second intervention for mitral valve replacement and severe pericardial effusion. No patients died in our cohort until December 2019. Conclusions Patients with RASopathies present a distinct CHD spectrum. Present data suggest that also ACDs must be carefully investigated for their possible impact on the clinical outcome. A careful longitudinal follow up until the individuals reach an adult age is recommended.

Background and aims Preterm newborns undergo hemodynamic challenges in the postnatal period. The aim of this study was to investigate myocardial mechanics changes in the postnatal period in preterm infants using speckle tracking echocardiography (STE). Material and methods Thirty-nine preterm infants ≤34 weeks' gestation underwent cardiac ultrasound evaluation during the first 96 hours of life. A repeated echocardiogram at 3 weeks of age was performed. Echocardiographic assessment involved left ventricular ejection fraction, mitral E/A ratio, S' and E' velocities, E/E' ratio, tricuspid annular plane systolic excursion (TAPSE), left atrium-to-aorta ratio, ductal diameter and ductal shunt pattern. Left ventricular longitudinal, circumferential and radial strain, apex-basal rotation and twist were measured from the apical 4-chamber and short-axis views using STE. Results The mean gestational age was 30 ± 2.7 weeks with a mean birth weight of 1318 ± 485 g. Apical segments demonstrated higher longitudinal strain than basal and mid-ones. In all gestational ages, endocardial longitudinal strain was higher than the epicardial. Epicardial longitudinal strain significantly increased during the first 3 weeks, resulting in the change in basal rotation from counterclockwise to clockwise and thus in the acquisition of twist. Deformation parameters were higher in infants with a hemodynamic significant patents ductus arteriosus. Conclusions Echocardiographic assessment of myocardial deformation parameters is feasible in preterm infants. Our data suggest that the maturational process of the myocardium is due to the development of the epicardial layer after birth, which allows the acquisition of the twist.

A comprehensive description of morbidity and mortality in patients affected by mutations in genes encoding for signal transducers of the RAS-MAPK cascade (RASopathies) was performed in our study recently published in the International Journal of Cardiology. Seven European cardiac centres participating to the CArdiac Rasopathy NETwork (CARNET), collaborated in this multicentric, observational, retrospective data analysis and collection. In this study, clinical records of 371 patients with confirmed molecular diagnosis of RASopathy were reviewed. Cardiac defects, crude mortality, survival rate of patients with 1) hypertrophic cardiomyopathy (HCM) and age

Accurate cardiovascular imaging is essential for the successful management of patients with congenital heart disease (CHD). Echocardiography and angiography have been for long time the most important imaging modalities in pediatric cardiology, but nuclear medicine has contributed in many situations to the comprehension of physiological consequences of CHD, quantifying pulmonary blood flow symmetry or right-to-left shunting. In recent times, remarkable improvements in imaging equipments, particularly in multidetector computed tomography and magnetic resonance imaging, have led to the progressive integration of high resolution modalities in the clinical workup of children affected by CHD, reducing the role of diagnostic angiography. Technology has seen a parallel evolution in the field of nuclear medicine, with the advent of hybrid machines, as SPECT/CT and PET/CT scanners. Improved detectors, hugely increased computing power, and new reconstruction algorithms allow for a significant reduction of the injected dose, with a parallel relevant decrease in radiation exposure. Nuclear medicine retains its distinctive capability of exploring at the tissue level many functional aspects of CHD in a safe and reproducible way. The lack of invasiveness, the limited need for sedation, the low radiation burden, and the insensitivity to body habitus variations make nuclear medicine an ideal complement of echocardiography. This is particularly true during the follow-up of patients with CHD, whose increasing survival represent a great medical success and a challenge for the health system in the next decades. Metabolic imaging using 18FDG PET/CT has expanded its role in the management of infection and inflammation in adult patients, particularly in cardiology. The same expansion is observed in pediatric cardiology, with an increasing rate of studies on the use of FDG PET for the evaluation of children with vasculitis, suspected valvular infection or infected prosthetic devices. The introduction in the clinical practice of the first integrated PET/MR scanners and the development of new radiopharmaceuticals, as fluorinated compounds for the study of myocardial perfusion, open new perspectives in the use of nuclear medicine techniques in pediatric cardiology, offering the potential of a detailed noninvasive morphofunctional characterization in many types of CHD.

Congenital heart block (CHB) is an autoantibody mediated disorder presumably caused by placental transmission of maternal autoantibodies to Ro/SSA 52 kd, p200, Ro/SSA 60 kd, La/SSB ribonucleoproteins. This study investigated the clinical significance of isolated anti-Ro/SSA 52 kd, anti-p200, anti-Ro/SSA 60 kd, and anti-La/SSB antibodies in positive pregnant patients. One hundred sixty-three pregnant women positive to anti-Ro/SSA 52 kd and/or anti-Ro/SSA 60 kd and/or anti-La/SSB antibodies were prospectively enrolled in the study. Anti-Ro52, anti-Ro60, anti-p200, and anti-La antibodies were assayed using home-made ELISA assays. Isolated antibody positivity was found in 25 women (15.3%), while multiple antibody positivity in 138 (84.7%). Twenty-four developed CHB, and the 139 had a favorable pregnancy outcome. The prevalence of isolated anti-Ro/SSA 60 kd antibodies was significantly higher (p

Background Mitral valve resistance (MVR) is a hemodynamic consequence of mitral stenosis but it has no clear threshold and it has a shortage of data to be reliable. Purpose is to investigate match and mismatch between opening area and resistance especially in patients with moderate and mild mitral stenosis. Materials This cross section case control study comprised 88 patients with moderate and mild rheumatic mitral stenosis. Transthorathic echocardiographic study estimated: mitral valve area (MVA) both by planimerty (2D) and pressure half time (PHT), mitral valve score (MVS), mean transmitral pressure gradient (MPG), diastolic filling time(DFT), left ventricular out flow tract diameter (LVOTd) and velocity time integral (LVOT vti) , the MVR was calculated as: MPG/aortic flow ratio [(LVOTd) (LVOTvti)/ DFT] in dynes.sec.cm5, NYHA function class of all patients was estimated. We classified our patients into 2 groups, group 1 (51 patients) with matched MVR and group 2 (37 patients) with unmatched MVR (unexpected high MVR in relation to valve area). Methods Patients with moderate mitral stenosis have MVR less than 105 dynes.sec/cm5, while patient with mild mitral stenosis have MVR less than 76 dynes.sec/cm5 this is in the matched group, but there are patients with unmatched higher MVR. Group 2 compared to group 1; had higher NYHA function class (1.4±0.6 vs. 1.2±0.4, P < 0.05), MVS (8.1±1.8 vs 7±0.9, P < 0.05), MPG (11,3±3.7 vs.7.8±2.5 mmHg, P < 0.01) and higher MVR (122.37±29.87 vs. 67.18±20.12 dynes.sec/cm5 , P < 0.01), MVR showed positive correlation with MVS (r=0.5, P < 0.05), Step wise logistic regression analysis showed that MVS is the only independent predictor of the MVR severity in the mismatched (unexpected high) group , so the higher the MVS the higher the expected MVR whatever the MVA is ; (B±SE=6.997±2.826, t=2.476, 95% CI 1.241±12.752 with an odds ratio=0.412, P < 0.05). Conclusion It would make much more sense to investigate match and mismatch between opening area and resistance in rheumatic mitral stenosis, the only independent predictor of mismatch is the mitral valve score.

Background: Juvenile Systemic Sclerosis (JSSc) is a rare multisystemic connective tissue disease, with onset before the age of 16. Cardiac involvement, recognized in 8-24% of the patients [1], begins in early stages of JSSc and has a poor prognosis. The traditional cardiac US imaging, including the left ventricular ejection fraction (EF), evaluates the global function of the heart, thus being inappropriate to assess the subclinical course of the disease. A new echocardiographic technique, the speckle tracking echocardiography (STE), has been shown to be able to identify regional ventricular dysfunctions also in early stages of adult-onset SSc[2,3]. Objectives: Aim of our study was to assess the longitudinal strain of right and left ventricle in JSSc patients, in order to identify ventricular dysfunctions earlier and more effectively than with traditional echocardiography. Furthermore, we investigated the evolution of cardiac involvement during the follow-up and possible correlations with the overall disease severity, measured by the Juvenile Systemic Sclerosis Severity Score (J4S)[4]. Methods: Consecutive patients with JSSc underwent clinical and cardiologic evaluation. This included traditional echocardiography (such as M-Mode, EF, Pulsed- and Tissue-Doppler), 3D-Echocardiography and STE, measuring the global longitudinal strain of left ventricle (GLS) and the longitudinal strain of right ventricle free-wall (RVLS). Each patient was assessed several times by pediatric rheumatologists for J4S and by cardiologists with STE and standard echo. Results: 18 JSSc patients (12 F, 6 M), mean age 12.3 years, disease duration 4.5 years, entered the study. At baseline evaluation, EF was abnormal in 1 patient, whereas GLS and RVLS were abnormal in 5. The diagnostic sensitivity of cardiac involvement of STE increased, with a prevalence rising from 22.2% to 38.8%. During the follow-up, lasted mean 30 months (range 17–43), the mean GLS values gradually worsened (-21.2; -20.1; -19.4%) while there was no significant variations of EF. The strong correlation between GLS and J4S, found at baseline, vanished during the follow-up. Conclusion: Speckle tracking echocardiography is a useful technique to evaluate the cardiac involvement in patients with JSSc. In comparison with traditional EKG or echocardiography, it allows to increase the diagnostic sensitivity of cardiac involvement. Over time, we observed a gradual worsening of GLS, sign of a progressive left ventricular dysfunction, that was not identified by EF. It is possible that the coronary microvascular damage compromises the subendocardial fibers function which are more sensitive to ischemia and whose contractility is well assessed by GLS [5]. Finally, the initial correlation between strain and J4S disappeared during the follow-up, maybe because of the pharmacological therapy, which was effective on several aspects of the disease but had low impact on the ventricular function. References: [1] Martini G et al. Arthritis Rheum 2006; 54:3971–3978. [2] Spethmann S et al. Eur. Heart J Cardiovascular Imaging 2012; 13:863–870. [3] Dedeoglu R et al. Pediatric Cardiol 2017; 38:1686–1695. [4] La Torre F et al. Arthritis Rheum 2012; 64:4143–4150. [5] Geyer H et al. J Am Soc Echocardiog 2010; 23:351– 369. Disclosure of Interests: None declared

Background Stent implantation is the treatment of choice for adolescents and adults with aortic coarctation (CoAo). Despite excellent short-term results, 20%–40% of the patients develop arterial hypertension later in life, which was attributed to inappropriate response of the aortic baroreceptors to increased stiffness of the ascending aorta (ASAO), either congenital or induced by CoAo repair. In particular, it has been hypothesized that stent itself may cause or sustain hypertension. Therefore, we aimed to study the hemodynamic and structural impact following stent implantation in the normal aorta of a growing animal. Methods Eight female sheep completed the study and a stent was implanted in four. Every 3 mo we measured blood pressure of the anterior and posterior limbs and left ventricular function by echocardiography. Twelve months later invasive pressure was measured under baseline and simulated stress conditions. Expression of genes indicating oxidative stress (OS), endothelial dysfunction (ED) and stiffness, as well as pathological examination were performed in ascending (ASAO) and descending aorta (DSAO). Results SOD1 and MMP9 gene expression were higher in ASAO of the stented animals, compared to DSAO and controls, while NOS3 was decreased. No differences were found in blood pressure and echocardiographic parameters. No histological differences were found in the aorta of the two groups of animals. Conclusions Stent does not affect central and peripheral hemodynamics, cardiac structure and function even in the long term. However, the finding of markers of OS and increased stiffness of ASAO, proximal to the stent, points to molecular mechanisms for increased cardiovascular risk of patients with stented CoAo.

Background and aim of the study Congenital mitral valve (MV) dysplasia is a relatively rare and highly complex cardiac disease. We present our results and illustrate the techniques used to repair these valves. Methods Between 1972 and 2014, 100 consecutive patients underwent surgical repair of congenital MV dysplasia at our institution. Predominant MV regurgitation was present in 53 patients (53%) whereas mitral stenosis was prevalent in 47 (47%). Results There were five early (5%) and eight late deaths (9%). Actuarial survival was 95%, 94%, and 93% at 5, 10, and 20 years, respectively. Sixteen patients (18%) required reintervention due to subsequent MV dysfunction. Actuarial freedom from reintervention for MV dysfunction was 95%, 92%, and 89% at 5, 10, and 20 years, respectively. Conclusions The mechanism underlying the valve dysfunction in congenital mitral valve dysplasia is multifactorial and requires the application of a variety of surgical techniques for repair. doi: 10.1111/jocs.12743 (J Card Surg 2016;31:352-356).

Background The TOPP Registry has been designed to provide epidemiologic, diagnostic, clinical, and outcome data on children with pulmonary hypertension (PH) confirmed by heart catheterisation (HC). This study aims to identify important characteristics of the haemodynamic profile at diagnosis and HC complications of paediatric patients presenting with PH. Methods and results HC data sets underwent a blinded review for confirmation of PH (defined as mean pulmonary arterial pressure≥25mmHg, pulmonary capillary wedge pressure≤12mmHg and pulmonary vascular resistance index [PVRI] of >3WU×m 2 ). Of 568 patients enrolled, 472 who fulfilled the inclusion criteria and had sufficient data from HC were analysed. A total of 908 diagnostic and follow-up HCs were performed and complications occurred in 5.9% of all HCs including five (0.6%) deaths. General anaesthesia (GA) was used in 53%, and conscious sedation in 47%. Complications at diagnosis were more likely to occur if GA was used (p=0.04) and with higher functional class (p=0.02). Mean cardiac index (CI) was within normal limits at diagnosis when analysed for the entire group (3.7L/min/m 2 ; 95% confidence interval 3.4–4.1), as was right atrial pressure despite a severely increased PVRI (16.6WU×m 2, 95% confidence interval 15.6–17.76). However, 24% of the patients had a CI of 2 at diagnosis. A progressive increase in PVRI and decrease in CI was observed with age (p Conclusion In TOPP, haemodynamic assessment was remarkable for preserved CI in the majority of patients despite severely elevated PVRI. HC-related complication incidence was 5.9%, and was associated with GA and higher functional class.

During the last decade interest in preserving pulmonary valve (PV) function has stimulated a few surgeons to apply valve-sparing techniques in repairing tetralogy of Fallot (TOF), with the aim of preserving long-term right ventricular function. Since June 2007, we embarked upon a program for preserving PV function in selected patients during TOF repair. More recently, the introduction of more complex PV plasty techniques allowed us to further extend the applicability of PV preservation techniques. We believe that preservation of PV function during early repair of TOF, by combining different intraoperative surgical maneuvers, can be extended to almost all patients with classic TOF.

The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy. Catheter ablation in patients with CHD has paralleled the advances of this technology in pediatric and adult patients with structurally normal hearts. Growing experience and introduction of new techniques like the 3D mapping systems into clinical practice have been particularly beneficial for this growing population of patients with abnormal cardiac anatomy and physiology. Finally, device therapies allowing maintanence of chronotropic competence and AV conduction, improving haemodynamics by cardiac resynchronization, and preventing sudden death are increasingly used. For pharmacological therapy, ablation procedures, and device therapy decision making requires a deep understanding of the individual pathological anatomy and physiology as well as detailed knowledge on natural history and long-term prognosis of our patients. Composing expert opinions from cardiology and paediatric cardiology as well as from non-invasive and invasive electrophysiology this position paper was designed to state the art in management of young individuals with congenital heart defects and arrhythmias.

AIM: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and quality of life is unknown and objective of this study. METHODS: This multicenter retrospective/cross-sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connection). Resting spirometry, cardiopulmonary exercise tests, and quality-of-life assessment (SF-36 questionnaire) were performed between 2003 and 2015. RESULTS: Overall, mean forced expiratory volume in one second (FEV1 ) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV1 was 80%, suggestive of a restrictive ventilatory pattern. Reduced FEV1 was associated with a reduced peakVO2 of 67.0 ± 17.6%pred. (r = 0.43, P < .0001), even if analyzed together with possible confounding factors (sex, BMI, age, years after palliation, number of interventions, scoliosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV1 was associated to quality of life in terms of physical component summary (r = 0.30, P = .002), physical functioning (r = 0.25, P = .008), bodily pain (r = 0.22, P = .02), and general health (r = 0.16, P = .024). Lower FEV1 was associated with diaphragmatic paralysis (P = .001), scoliosis (P = .001), higher number of interventions (P = .002), and lower BMI (P = .01). No correlation was found to ventricular morphology, type of surgeries, or other perioperative/long-term complications. CONCLUSIONS: This study shows that the common restrictive ventilatory pattern in Fontan patients is associated with lower exercise capacity and quality of life. Risk factors are diaphragmatic paralysis, scoliosis, a high total number of interventions and low BMI.

Objectives The objective of this investigation is to evaluate the safety, the impact of endomyocardial biopsy (EMB) results in myocarditis management and the incidence of different etiologies of myocarditis in a pediatric population. Background Although EMB is an established diagnostic tool to evaluate suspected myocarditis, there is lack of clear diagnostic and management guidelines for myocarditis in pediatric patients, particularly in infants. Methods We performed a retrospective database review and subsequent outcomes analysis from five Italian pediatric cardiology centers to identify patients aged 0–18 years who underwent EMB for suspected myocarditis or inflammatory cardiomyopathy (ICMP) between 2009 and 2011. Results EMB was performed in 41 children, of which 16 were male. The population ranged between 16 days of age to 17 years (mean age at EMB = 5.2 ± 4.9 years). The overall incidence of EMB-related complications was 15.5% (31.2% in infants, and 6.8% in children > 1 year of age; P = 0.079) while the incidence of EMB-driven treatment changes was 29.2%. Histological examination together with PCR on heart biopsy specimens allowed an etiological diagnosis in 26/41 patients (63%). Among the 15 patients (36.5%) with diagnosis of dilated cardiomyopathy (DCM) 11 had idiopathic DCM. Finally, we found an overall incidence of death/cardiac transplantation of 24%. Conclusions In a pediatric population with suspected myocarditis/ICMP, EMB was useful in confirming the diagnosis only in 41% of cases but showed an overall diagnostic power of 63%. As complications of EBM are not negligible, particularly in infants, the risk/benefit ratio should be taken into account in each patient. © 2015 Wiley Periodicals, Inc.

Aims During the last decade the cooperation between surgeons and cardiologists has further expanded by combining surgical and interventional techniques (CCBSI) performed in the operating room, without the use of fluoroscopy. We sought to evaluate the results of our experience with CCBSI. Methods All children with congenital heart disease (CHD) who underwent a CCBSI in the operating room between June 2007 and January 2014 were enrolled. Results Sixty-eight patients were included. Median age at CCBSI was five months (range 1–48 months). The three main diagnoses leading to surgery included: (1) tetralogy of Fallot (TOF) (n = 40), (2) muscular ventricular septal defects (VSD) (n = 12), (3) single ventricle with pulmonary artery branch stenosis (n = 4). There were 72 catheter-based procedures associated with surgical maneuvres, including: (1) transatrial balloon dilation (BD) of the pulmonary valve (n = 45), (2) transinfundibular BD of the main pulmonary artery trunk (n = 12), (3) perventricular VSD closure with septal occluder (n = 8), (4) BD of pulmonary artery branches (n = 5), and other less common procedures (n = 2). There were no procedure-related complications and no hospital deaths. Median follow-up time was four years (range 0.95–7.9 years). There was one late death for respiratory distress after transapical balloon dilation of the aortic valve. One patient required BD and stenting of the left pulmonary artery branch 3.6 years after intraoperative BD for residual stenosis. Conclusions The CCBSI represents a safe and effective treatment for selected patients with complex CHD. It will be helpful in minimizing patients’ surgical trauma and in shortening or avoiding the use of cardiopulmonary bypass. doi: 10.1111/jocs.12595 (J Card Surg 2015;30:719–723)

Conflicting data were reported about normalization of sizes of right chambers, systolic and diastolic function and prevalence of arrhythmias after ostium secundum atrial septal defect closure. We sought to compare surgical and percutaneous approaches in terms of arrhythmias, right chamber volumes, and function at long-term follow-up.In all, 107 patients were enrolled, all corrected at pediatric age. Forty-four of them were treated surgically with a right thoracotomy approach and 63 were treated percutaneously. All patients underwent a standard echocardiogram and electrocardiographic Holter examinations.No difference was detected between the two groups regarding right atrial or ventricular volumes. The global right ventricular function assessed by fractional area change was similar between the two groups. However, the longitudinal function and the diastolic function were significantly impaired in the surgical group (tricuspid annulus peak systolic excursion 23.7 ± 4.5 mm versus 18.7 ± 3.5 mm, p0.001; S' wave 13.7 ± 3.1 cm/s versus 9.8 ± 2.4 cm/s, p0.001; E/E' 4.7 ± 1.7 versus 7.1 ± 2.9, p0.001). There was a low incidence of supraventricular couples or runs, but slightly higher in the surgical group (6.8% versus 1.6%), although not statistically significant. No echocardiographic variable related to ventricular or supraventricular arrhythmic events.Either surgical or percutaneous closure of atrial septal defect have a similar efficacy on the volume normalization of the right chamber. Modern surgical techniques have a limited impact on the systolic and diastolic function as well as on the arrhythmic risk; however, the right ventricular longitudinal and diastolic function seems to be better preserved in the percutaneous group.

Background Clinical utility of remote monitoring of implantable cardiac devices has been previously demonstrated in several trials in the adult population. The aim of this study was to assess the clinical utility of remote monitoring in a pediatric population undergoing pacemakers implantation. Methods The study population included 73 consecutive pediatric patients who received an implantable pacemaker. The remote device check was programmed for every 3 months and all patients had a yearly out-patient visit. Data on device-related events, hospitalization, and other clinical information were collected during remote checks and out-patient visits. Results During a mean follow-up of 18 ± 10 months, 470 remote transmissions were collected and analyzed. Two deaths were reported. Eight transmissions (1.7%) triggered an urgent out-patient visit. Twenty percent of transmissions reported evidence of significant clinical or technical events. All young patients and their families were very satisfied when using remote monitoring to replace out-patient visits. Conclusions The ease in use, together with satisfaction and acceptance of remote monitoring in pediatric patients, brought very good results. The remote management of our pediatric population was safe and remote monitoring adequately replaced the periodic out-patient device checks without compromising patient safety.

We report the case of an 11-year-old boy with Loeys-Dietz syndrome, with both aortic and pulmonary aneurysms requiring cardiac operation because of progressive valve incompetence resulting from loss of coaptation of the cusps. Arterial medial changes, consisting of disarray of elastic fibers and increased collagen deposition, were observed in surgical specimens from both the aorta and the pulmonary artery of our patient, and the strong pSmad2 nuclear staining of smooth muscle cells of both aortic and pulmonary tunica media are the best evidence of transforming growth factor-β pathway activation in Loeys-Dietz syndrome.

Scimitar syndrome is treated either by surgery or by clinical monitoring depending on a series of defined patient characteristics. Treatment decisions are often challenging and it is a dilemma how to treat borderline cases, that is, patients with isolated scimitar syndrome (who often are asymptomatic and might be diagnosed serendipitously). The natural history of patients with scimitar syndrome is strongly influenced by associated comorbidities. Patients with isolated forms of scimitar syndrome may be asymptomatic for a long time, whilst those forms with associated comorbidities may be highly symptomatic in infancy and may die in the first years of age, despite optimal care. Medical therapy is indicated to treat heart failure, to allow growth before surgical repair, to reduce lung congestion and pulmonary resistances and it is used for poor surgical candidates.

Background RASopathies are developmental disease caused by mutations in genes encoding for signal transducers of the RAS-MAPK cascade. The aim of the present study was to provide a comprehensive description of morbidity and mortality in patients with molecularly confirmed RASopathy. Methods A multicentric, observational, retrospective study was conducted in seven European cardiac centres participating to the CArdiac Rasopathy NETwork (CARNET). Clinical records of 371 patients with confirmed molecular diagnosis of RASopathy were reviewed. Mortality was described as crude mortality, cumulative survival and restricted estimated mean survival. Multivariable regression analysis was used to assess the impact of mutated genes on number of interventions and overall prognosis. Results Cardiac defects occurred in 80.3% of cases, almost half of them underwent at least one intervention. Overall, crude mortality was 0.29/100 patients-year. Cumulative survival was 98.8%, 98.2%, 97.7%, 94.3%, at 1, 5, 10, and 20 years, respectively. Restricted estimated mean survival at 20 years follow-up was 19.6 years. Ten patients died (2.7% of the entire cohort; 3.4% of patients with cardiac defect). Patients with hypertrophic cardiomyopathy (HCM) and age < 2 years or young adults, as well as subjects with biventricular obstruction and PTPN11 mutations had a higher risk of cardiac death. Conclusions The risk of intervention was higher in individuals with Noonan syndrome and pulmonary stenosis carrying PTPN11 mutations. Overall, mortality was relatively low, even though the specific association between HCM, biventricular outflow tract obstructions and PTPN11 mutations appeared to be associated with early mortality, including immediate post-operative events and sudden death.

Stent implantation is the treatment of choice for adolescents and adults with aortic coarctation (CoAo). Despite excellent short-term results, 20%-40% of the patients develop arterial hypertension later in life, which was attributed to inappropriate response of the aortic baroreceptors to increased stiffness of the ascending aorta (ASAO), either congenital or induced by CoAo repair. In particular, it has been hypothesized that stent itself may cause or sustain hypertension. Therefore, we aimed to study the hemodynamic and structural impact following stent implantation in the normal aorta of a growing animal.Eight female sheep completed the study and a stent was implanted in four. Every 3 mo we measured blood pressure of the anterior and posterior limbs and left ventricular function by echocardiography. Twelve months later invasive pressure was measured under baseline and simulated stress conditions. Expression of genes indicating oxidative stress (OS), endothelial dysfunction (ED) and stiffness, as well as pathological examination were performed in ascending (ASAO) and descending aorta (DSAO).SOD1 and MMP9 gene expression were higher in ASAO of the stented animals, compared to DSAO and controls, while NOS3 was decreased. No differences were found in blood pressure and echocardiographic parameters. No histological differences were found in the aorta of the two groups of animals.Stent does not affect central and peripheral hemodynamics, cardiac structure and function even in the long term. However, the finding of markers of OS and increased stiffness of ASAO, proximal to the stent, points to molecular mechanisms for increased cardiovascular risk of patients with stented CoAo.

Background Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD), whose surgical repair is associated with high mortality and reoperation rates. We sought to identify predictors of early and late outcomes. Methods Data from medical records of patients who underwent surgical repair for TAPVC from 1989 to 2012 were included. The patients were divided in two groups, according to absence or presence of associated major CHDs. Results Forty-six patients were included (M/F: 26/20, median age 26 days, interquartile range 15 to 59, median weight 3.350 kg, interquartile range 1800 to 4470). Anatomic types of TAPVC were: supracardiac in 48%, intracardiac in 20%, infracardiac in 20%, and mixed in 12%; TAPVC was obstructive in 33%; TAPVC was isolated in 63%, complex in 37%. Single ventricle physiology was present in 11 patients, heterotaxy in eight patients. Overall operative mortality was 19.6% (9/46): 6.9% in isolated TAPVC, 41.2% in complex type (p-value: 0.002). It was associated with low weight at intervention ( 60 minutes (p = 0.097). At a median follow-up of 2.97 years (range 43 days to 22 years, 91% complete), there were nine late deaths (24.3%); 15 patients (40.5%) had major events (including late death). Multivariate analysis of event-free survival showed worse outcome in the complex group (p = 0.001). Conclusion Surgical mortality and morbidity remain consistent in patients with TAPVC and associated major CHD, while the isolated type shows excellent outcomes. Pulmonary vein obstruction is a rare but highly lethal complication. doi: 10.1111/jocs.12399 (J Card Surg 2014;29:678–685)

Background The aim of the study was to evaluate our results with pulmonary valve (PV) preservation in selected patients with tetralogy of Fallot (TOF). Methods From January 2007, 69 patients who underwent early transatrial TOF repair were enrolled in the study. The patients were divided into 2 groups: PV preservation by PV annulus balloon dilation (group 1) and PV cusp reconstruction after annular incision (group 2). Results Thirty-four patients underwent a successful PV annular preservation (49%). Median age at surgery was 113 days (range, 36-521 days) (group-1 vs group-2, P = not significant). Median preoperative PV Z score was −3.15 (range, −0.95 to −5.62) (group-1 vs group-2, P = .03). Median intensive care unit and hospital stays were 3 and 10 days, respectively (group 1 vs group 2, P = not significant). Median follow-up time was 580 days (range, 189-1940 days) (group 1 vs group 2, P = .08). Two patients were reoperated for residual right ventricular outflow tract (RVOT) obstruction (1 in group 1 and 1 in group 2). The remaining patients are alive and well. Median peak RVOT gradient was 25 mm Hg (range, 8-60 mm Hg) (group 1 vs group 2, P = not significant). The degree of PV regurgitation in group 1 was none/mild in 24 patients (80%) and moderate in 6 (20%) and was none/mild in 8 patients (25%), moderate in 11 (34.4%), and severe in 13 (28.6%) in group 2 ( P = .001). Median right ventricular fractional area change was 55% (range, 42%-70%) in group 1 and 50% (range, 40%-63%) in group 2 ( P = .003). Conclusions The integrity and function of the PV can be preserved in selected patients during early repair of TOF by concomitant balloon dilation, leading to a better mid-term right ventricular function.

Resumen Introduccion y objetivos Analizar el estado clinico de los pacientes con sindrome de la cimitarra «no corregido» en un estudio multicentrico italiano. Metodos Se analizo la evolucion natural del sindrome de la cimitarra en 44 individuos afectados de nueve centros de Italia. Resultados La mediana de edad de diagnostico fue 1,05 anos (intervalo, 1 dia-41 anos). En total, 33 pacientes (75%) presentaban una forma aislada; en 11 (25%) habia cardiopatias congenitas asociadas; 22 pacientes (50%) tenian sintomas en el momento del diagnostico, que consistian en sintomas respiratorios (n = 20) e insuficiencia cardiaca congestiva (n = 6). Los pacientes con cardiopatias congenitas asociadas presentaban prevalencias de insuficiencia cardiaca congestiva (4 de 11 [36,4%] frente a 2 de 33 [6,1%]; p = 0,027) e hipertension arterial pulmonar (7 de 11 [63,6%] frente a 2 de 33 [6,1%]; p = 0,027) superiores que los pacientes con formas aisladas del trastorno. A 10 pacientes (22,7%), se les practico una correccion de los defectos cardiacos asociados, con lo que se dejo intacto el drenaje venoso pulmonar. La mediana de duracion del seguimiento despues del diagnostico fue de 6,4 (0,2-27,5) anos. Fallecieron 2 pacientes, ambos con defectos cardiacos asociados e hipertension arterial pulmonar grave. De los 42 supervivientes, 39 (92,8%) estaban asintomaticos en la ultima visita de seguimiento; 3 pacientes continuaban refiriendo sintomas respiratorios. No se observaron diferencias entre las formas aisladas y las formas asociadas de la enfermedad. Conclusiones En la mayoria de los pacientes, el sindrome de la cimitarra se manifesto en forma de una lesion aislada con evolucion benigna. No obstante, cuando se asociaba a otros defectos cardiacos y a hipertension arterial pulmonar, habia aumento del riesgo de insuficiencia cardiaca congestiva y muerte. La correccion de los defectos cardiacos asociados (transformacion de una forma «asociada» a una forma «aislada»), junto con la oclusion terapeutica de la irrigacion arterial anomala del pulmon, proporciono una evolucion benigna comparable a la de las formas aisladas primarias.

Objective We sought to evaluate our recent experience with surgical treatment of branch pulmonary artery (PA) stenosis both for native and acquired lesions. Materials and Methods The postoperative course of patients who underwent surgical PA plasty augmentation between January 2004 and January 2012 were reviewed. Primary outcomes included the need for further surgical procedures or interventional maneuvers on the branch PAs for residual stenosis. Results Thirty-four patients were included. Median age at PA plasty was eight months (range 8 days to 3.4 years). There were 12 native and 22 acquired PA stenoses, which were mainly located at the PA branch origin (n = 25, 73%). The PA plasty was defined as simple (n = 16, 47%) and as complex (n = 18, 53%), which included multiple maneuvers on the PA branches. Median follow-up time after surgical treatment was 4.7 years (range 0.9 to 8.7 years). One patient died 3 days after complex PA plasty for low output syndrome and another died 22 months later for congestive heart failure. Twenty-one (63.6%) underwent 40 catheter intervention procedures on the PA branches for residual stenosis. The majority of them (n = 10, 57.1%) were operated before the age of six months and the majority had an acquired PA stenosis (14, 66.7%). Three patients underwent additional surgical maneuvers on the PA branches. Conclusions PA branch stenosis represents a life-threatening condition often necessitating further surgical or interventional treatment. A combined collaborative surgical followed by transcatheter approach is important, particularly in cases with an acquired PA stenosis who require complex surgical repair. doi: 10.1111/jocs.12121 (J Card Surg 2013;28:439–445)

Surgical re-implantation of the left coronary artery (LCA) is the treatment of choice in anomalous origin of left coronary artery from pulmonary artery (ALCAPA). Despite normalization of left ventricular function after surgery, residual coronary lesions or myocardial fibrosis may be found. The aim of this study was to detect regional left ventricular dysfunction predictive of coronary lesions or residual myocardial fibrosis using speckle tracking. We enrolled ten patients treated with surgical re-implantation of LCA for ALCAPA. All patients were asymptomatic, and ejection fraction (EF) was normal. Using S-SR imaging, we studied longitudinal, radial, and circumferential function. A cardiac MRI was performed to assess myocardial fibrosis and the anatomy of the coronaries. In case of suspected coronary stenosis, a coronary angiography was performed. Finally, 20 normal subjects were enrolled as control group. Median age at surgery was 188 days, and mean follow-up was 8.7 ± 4.7 years. Longitudinal and circumferential functions were reduced in LCA territory compared to RCA territory and normal. MRI showed LCA stenosis in three of ten patients, confirmed by coronary angiography: these patients had the lowest longitudinal strain values in LCA territories (-11.7, -14.7 and -14.8%). Radial strain was preserved (Normal 45.6 ± 12.1, ALCAPA 43.5 ± 10.7%, p = ns), while circumferential strain was mildly depressed (-23.5 ± 3.8 vs. -20.3 ± 2.0%, p 0.05). After LCA re-implantation, ALCAPA patients showed a residual sub-endocardial damage in LCA territories. Despite a normal systolic and diastolic function, the prevalence of residual coronary lesions was high. A mean longitudinal strain-15% in LCA territories was able to identify those patients.

In August 2010, the Nit-Occlud

Surgery for congenital valve anomalies in children is a challenging topic. We aim to assess early and late functional outcomes of CorMatrix scaffold after repair of aortic and pulmonary valves (PV) in congenital heart disease in a prospective nonrandomized clinical study on children with congenital aortic (Group 1) or PV (Group 2) disease. Primary endpoints were reoperation or reintervention on semilunar valves and echocardiographic evidence of regurgitation or stenosis greater than mild. Results of PV repair in tetralogy of Fallot were compared with a control group of patients who underwent PV repair with polytetrafluoroethylene. A total of 22 consecutive selected patients with complex congenital heart disease were included: PV repair in 18 and aortic valve repair in 4. At discharge bidimensional echocardiography, semilunar valve regurgitation was mild in 50% of all patients. At a median follow-up of 23 months (4.3-51.3), reoperation for aortic valve replacement was necessary in 2; right ventricular outflow tract obstruction occurred in 3, requiring interventional treatment in 2. Pulmonary regurgitation degree worsened in most patients of Group 2 at follow up. When comparing patients with tetralogy of Fallot in Group 2 with patients who underwent PV repair with polytetrafluoroethylene, there were no significant differences in PV function at follow up. We conclude that CorMatrix scaffold for semilunar valve reconstruction does not present with significant advantages to traditional materials at mid term follow up. In addition, when used for pulmonary valve reconstruction, CorMatrix seems to show gradual functional deterioration in the mid term.

Background Over the past 20 years our policy has been to electively repair common atrioventricular canal defects (CAVCD) in patients between 8 and 12 weeks of age. We sought to evaluate the results of our past 20-year experience. Methods From January 1992 to April 2014, 159 consecutive patients underwent CAVCD repair (133 patients had complete CAVCD and 26 patients had a transitional form of CAVCD). Results Surgical repair was accomplished with a double-patch (n = 137 [86%]) or a modified single patch (n = 22 [14%]) technique. Median age at operation was 96 days (interquartile range [IQR], 73–128 days); 90 patients were younger than 3 months of age. There were 3 operative (1.9%) and 12 late (7.7%) deaths. Median follow-up time after repair was 8.2 years (IQR, 3.6–15 years). Twenty patients (13%) required reoperation—16 (10%) for left atrioventricular valve (LAVV) regurgitation. Reoperation on the LAVV was more frequent in patients with a dysplastic LAVV preoperatively ( p = 0.01; odds ratio [OR], 4.2; 95% confidence interval [CI], 1.33–13.5) and in patients who underwent closure for an absent/incomplete cleft at the time of repair ( p = 0.01; OR, 5.4; 95% CI, 1.4–21). Late LAVV performance (regurgitation greater than or equal to moderate or the need for reoperation), including late deaths and patients who underwent reoperation, was significantly worse in patients older than 3 months at repair (10 of 83 patients [12%] versus 20 of 73 patients [27%]; hazard ratio [HR], 2.71; 95% CI, 1.19–6.19) and in patients with LAVV dysplasia (19 of 68 patients [28%] versus 11 of 88 patients [12%]; HR, 3; 95% CI, 1.53–8.51). Conclusions Individualized early repair of CAVCD is safe and beneficial, with good early and long-term results.