Dilated Cardiomyopathy Due to Alimentary Iron Deficiency.

Dilated cardiomyopathy (DCM) is a severe condition, characterised by left ventricular dilation and systolic dysfunction, necessitating heart transplantation when all other treatment options fail. This case report describes a 2-year-old girl initially presenting with oedema, listlessness, and severe iron deficiency anaemia. She was diagnosed with DCM. Extensive diagnostic workup ruled out other causes, leading to the suspicion of DCM due to alimentary iron deficiency. This was confirmed by the parents' report that the girl was fed almost exclusively with low-fat cow's milk. Prompt treatment, including packed red cell transfusion, iron supplementation, and heart failure medications (diuretics, ACE inhibitors, beta blockers, and aldosterone antagonists), resulted in significant improvement in cardiac function within days. This report demonstrates the potential risks of alimentary iron deficiency, the most common cause of microcytic hypochromic anaemia in young children, which might even result in the development of life-threatening cardiac dysfunction in extreme cases.

Myocarditis and sports in the young: data from a nationwide registry on myocarditis-"MYKKE-Sport".

Background: Myocarditis represents one of the most common causes of Sudden Cardiac Death in children. Myocardial involvement during a viral infection is believed to be higher as a consequence of intensive exertion. Recommendations for return to sports are based on cohort and case studies only. This study aims to investigate the relationship between physical activity and myocarditis in the young. Patient: Every patient in the MYKKE registry fulfilling criteria for suspicion of myocarditis was sent a questionnaire regarding the physical activity before, during and after the onset of myocarditis. Method: This study is a subproject within the MYKKE registry, a multicenter registry for children and adolescents with suspected myocarditis. The observation period for this analysis was 93 months (September 2013-June 2021). Anamnestic, cardiac magnetic resonance images, echocardiography, biopsy and laboratory records from every patient were retrieved from the MYKKE registry database. Results: 58 patients (mean age 14.6 years) were enrolled from 10 centers. Most patients participated in curricular physical activity and 36% in competitive sports before the onset of myocarditis. There was no significant difference of heart function at admission between the physically active and inactive subjects (ejection fraction of 51.8 ± 8.6% for the active group vs. 54.4 ± 7.7% for the inactive group). The recommendations regarding the return to sports varied widely and followed current guidelines in 45%. Most patients did not receive an exercise test before returning to sports. Conclusion: Sports before the onset of myocarditis was not associated with a more severe outcome. There is still a discrepancy between current literature and actual recommendations given by health care providers. The fact that most participants did not receive an exercise test before being cleared for sports represents a serious omission.

Non-invasive pediatric cardiac imaging-current status and further perspectives.

BACKGROUND: Non-invasive cardiac imaging has a growing role in diagnosis, differential diagnosis, therapy planning, and follow-up in children and adolescents with congenital and acquired cardiac diseases. This review is based on a systematic analysis of international peer-reviewed articles and additionally presents own clinical experiences. It provides an overview of technical advances, emerging clinical applications, and the aspect of artificial intelligence. MAIN BODY: The main imaging modalities are echocardiography, CT, and MRI. For echocardiography, strain imaging allows a novel non-invasive assessment of tissue integrity, 3D imaging rapid holistic overviews of anatomy. Fast cardiac CT imaging new techniques-especially for coronary assessment as the main clinical indication-have significantly improved spatial and temporal resolution in adjunct with a major reduction in ionizing dose. For cardiac MRI, assessment of tissue integrity even without contrast agent application by mapping sequences is a major technical breakthrough. Fetal cardiac MRI is an emerging technology, which allows structural and functional assessment of fetal hearts including even 4D flow analyses. Last but not least, artificial intelligence will play an important role for improvements of data acquisition and interpretation in the near future. CONCLUSION: Non-invasive cardiac imaging plays an integral part in the workup of children with heart disease. In recent years, its main application congenital heart disease has been widened for acquired cardiac diseases.

Left Ventricular Strain Analysis During Submaximal Semisupine Bicycle Exercise Stress Echocardiography in Childhood Cancer Survivors.

Background Childhood cancer survivors (CCSs) show relevant cardiac morbidity and mortality throughout life. Early detection is key for optimal support of patients at risk. The aim of this study was to evaluate 2-dimensional speckle-tracking echocardiography strain analysis during semisupine exercise stress in CCSs for detection of subclinical left ventricular dysfunction after cancer treatment. Methods and Results Seventy-seven CCSs ≥1-year postchemotherapy were prospectively examined at rest, low, and submaximal stress level and compared with a cohort of healthy adolescents and young adults (n=50). Global longitudinal strain (GLS), short axis circumferential strain, and corresponding strain rates were analyzed using vendor-independent software. CCSs at median 7.8 years postchemotherapy showed comparable left ventricular GLS, circumferential strain, and strain rate values at all stress stages to healthy controls. Yet, prevalence of abnormal GLS (defined as <2 SD of controls reference) in CCSs was 1.3% at rest, 2.7% at low, and 8.6% at submaximal stress. In CCSs, relative change of circumferential strain from rest to submaximal stress was lower than in healthy controls, median 16.9 (interquartile range [IQR], 3.4; 28.8) % versus 23.3 (IQR, 11.3; 33.3) %, P=0.03, most apparent in the subgroups of CCSs after high-dose anthracycline treatment and cancer diagnosis before the age of 5 years. Conclusions In this prospective 2-dimensional speckle tracking echocardiography strain study, prevalence of abnormal left ventricular GLS increased with stress level reflecting impaired cardiac adaptation to exercise stress in some CCSs. However, relatively early after last chemotherapy, this did not result in significant differences of mean GLS-, circumferential strain-, and strain rate values between CCSs and controls at any stress level.

Clinical characteristics and outcome of biopsy-proven myocarditis in children - Results of the German prospective multicentre registry "MYKKE".

BACKGROUND: Heart failure (HF) due to myocarditis might not respond in the same way to standard therapy as HF due to other aetiologies. The aim of this study was to investigate the value of endomyocardial biopsies (EMB) for clinical decision-making and its relation to the outcome of paediatric patients with myocarditis. METHODS: Clinical and EMB data of children with myocarditis collected for the MYKKE-registry between 2013 and 2020 from 23 centres were analysed. EMB studies included histology, immunohistology, and molecular pathology. The occurrence of major adverse cardiac events (MACE) including mechanical circulatory support (MCS), heart transplantation, and/or death was defined as a combined endpoint. RESULTS: Myocarditis was diagnosed in 209/260 patients: 64% healing/chronic lymphocytic myocarditis, 23% acute lymphocytic myocarditis (AM), 14% healed myocarditis, no giant cell myocarditis. The median age was 12.8 (1.4-15.9) years. Time from symptom-onset to EMB was 11.0 (4.0-29.0) days. Children with AM and high amounts of mononuclear cell infiltrates were significantly younger with signs of HF compared to those with healing/chronic or healed myocarditis. Myocardial viral DNA/RNA detection had no significant effect on outcome. The worst event-free survival was seen in patients with healing/chronic myocarditis (24%), followed by acute (31%) and healed myocarditis (58%, p = 0.294). A weaning rate of 64% from MCS was found in AM. CONCLUSIONS: EMB provides important information on the type and stage of myocardial inflammation and supports further decision-making. Children with fulminant clinical presentation, high amounts of mononuclear cell infiltrates or healing/chronic inflammation and young age have the highest risk for MACE.

Moyamoya disease associated with pediatric pulmonary hypertension-a case report.

Moyamoya disease (MD) is a rare vaso-occlusive disorder that primarily affects intracranial cerebral arteries. The involvement of extracranial vessels is unusual. However, there are previous reports suggesting MD to be a systemic disorder, causing disease manifestations in vessels of other parts of the body. We report the case of a female patient with MD and multiple episodes of ischemic strokes followed by bypass surgery of cerebral arteries during infancy. Due to corresponding ischemic lesions the girl showed global retardation of psychomotor development and central right sided movement disorder. At the age of 10 years the girl was admitted to our hospital with recurrent syncope. While cranial MRI excluded any newly added ischemic lesions, electrocardiography revealed evidence of right ventricular hypertrophy, and subsequent echocardiography then indicated pulmonary hypertension, which was confirmed by cardiac catheterization. Despite an upfront combination pulmonary vasodilating therapy, the pulmonary vascular disease appeared to be progressive. Genetic analysis showed heterozygous c.12341C>T mutation in the RNF213 gene. This case presentation demonstrates that pulmonary arterial hypertension is a rare comorbidity in patients with MD, especially in patients with genetic predictors such as the RNF213 mutation. Thus, regular echocardiographic screening for early signs of pulmonary arterial hypertension in patients with MD should be part of regular clinical work-up. Early detection and treatment of pulmonary arterial hypertension in MD might help to improve the long-term outcome in the individual patient.

Cardiac catheterization in pediatric pulmonary hypertension: a systematic and practical approach.

Significant progress in the understanding of the etiology, epidemiology, pathobiology and prognosis of pulmonary hypertension (PH) has been made over the last years. Especially in the pediatric patient population the etiology of PH is very heterogeneous. Nevertheless, the most recent change of the definition of PH to a mean pulmonary artery pressure (mPAP) >20 mmHg has been accepted by pediatricians for uniformity and concordance with adult physicians. Based on the diverse underlying medical conditions leading to PH, a comprehensive and systematic approach for diagnosis and treatment is mandatory. Cardiac catheterization remains the gold standard for invasive assessment and acute vasoreactivity testing (AVT) additionally providing detailed information about nature of PH. In most patients repeat cardiac catheterization may be helpful for evaluation of response to targeted PH treatment, risk stratification and indication for lung transplantation. However, the information and results taken from cardiac catheterization should be interpreted by experienced investigators only who are familiar with confounding factors that may influence the results. Here we provide an overview of current recommendations for invasive hemodynamic evaluation in pediatric PH. We point out different patient scenarios and provide a structured approach for AVT and response interpretation.

Left Ventricular Strain and Strain Rate during Submaximal Semisupine Bicycle Exercise Stress Echocardiography in Healthy Adolescents and Young Adults: Systematic Protocol and Reference Values.

OBJECTIVE: Combining stress echocardiography with strain analysis is a promising approach for early detection of subclinical cardiac dysfunction not apparent at rest. Data on normal myocardial strain and strain rate (SR) response to exercise in adolescents and young adults are contradictory and limited. The aim of this study was to propose a standardized protocol for semisupine bicycle stress echocardiography and to provide corresponding reference values of left ventricular (LV) two-dimensional speckle-tracking echocardiography (2D STE) strain and SR in adolescents and young adults. METHODS: Fifty healthy adolescents and young adults (mean age, 17.8 ± 3.2 years, 44% female) were prospectively assessed. Images were acquired at rest, low stress, submaximal stress, and during recovery. Optimal image quality for offline strain analysis was pursued, and image quality was rated. Global longitudinal strain and SR from apical four-/two-/three-chamber views and short-axis circumferential strain and SR were analyzed using vendor-independent software. Interobserver variability was assessed. RESULTS: Strain and SR increased during progressive exercise stress. Mean LV global longitudinal strain was -20.4% ± 1.3%, SR -1.1 ± 0.15/sec at rest (heart rate, 79.4 ± 12.0 beats/minute), increasing to -22.6% ± 1.6% and -1.5 ± 0.16/sec at low stress level (heart rate, 117.1 ± 8.7 beats/minute) and -23.7% ± 1.1% and -1.9 ± 0.29/sec at submaximal stress level (heart rate, 154.2 ± 7.0 beats/minute), respectively, returning to -20.6% ± 1.4% and -1.2 ± 0.16/sec postexercise (heart rate, 90.1 ± 9.4 beats/minute). Restriction on submaximal stress level ensured adequate image quality for 2D STE strain analysis. Interobserver variability for strain was acceptable even during submaximal stress. CONCLUSIONS: This study provides a systematic, standardized protocol and corresponding reference data for 2D LV STE-derived strain and SR during semisupine bicycle exercise testing in adolescents and young adults. According to our results, global longitudinal strain and SR appear to be the most comprehensible parameters for cross-sectional studies.

Heart Rate Response During Treadmill Exercise Test in Children and Adolescents With Congenital Heart Disease.

Background: Impaired exercise capacity is a common feature of congenital heart disease (CHD). In adults with CHD, it has been shown that impaired heart rate response during exercise may contribute to exercise limitation. Systematic data in children and adolescents on this topic is limited. We therefore purposed to assess heart rate response during treadmill exercise testing in children and adolescents with CHD compared to healthy controls. Methods: One hundred and sixty three children and adolescents (103 with CHD, median age 15 years and 60 age-matched controls) performed cardiopulmonary exercise testing and were included in this study. Beyond peak oxygen consumption, increase in heart rate from resting level to peak exercise (heart rate reserve) and decrease of heart rate after peak exercise (heart rate recovery) were measured. Chronotropic index was defined as percentage of age predicted maximal heart rate reserve. According to data from adults on bicycle exercise, chronotropic incompetence was assumed for chronotropic index below 0.8. Results: While resting heart rate was similar between both groups, peak heart rate, heart rate reserve as well as chronotropic index were lower in the CHD group than in controls. Chronotropic index was lowest in patients with single ventricle hemodynamics and correlated with peak oxygen consumption. Heart rate recovery was impaired in the CHD group 1 and 2 min after peak exercise compared to controls and correlated with peak oxygen consumption. Chronotropic index below 0.8 was a relatively frequent finding even in the control group suggesting that the threshold of 0.8 appears inadequate for the identification of chronotropic incompetence using treadmill exercise testing in children. After normalizing to the 2.5th chronotropic index percentile of the control group we obtained a chronotropic incompetence threshold of 0.69. Conclusion: As an adjunct to measurement of peak oxygen consumption, heart rate response to exercise appears to be a physiologically important diagnostic parameter in children and adolescents with CHD. However, interpretation of heart rate response needs to consider specific age characteristics and the mode of exercise test. Our data may help future studies on chronotropic incompetence using treadmill ergometer protocols in children and adolescents.

Impact of the Right Ventricular Sokolow-Lyon Index in Children with Idiopathic Pulmonary Arterial Hypertension.

Right ventricular (RV) hypertrophy is regarded as the adaptation on chronic RV pressure load in pulmonary hypertension. As the RV Sokolow-Lyon index (RVSLI) is an electrocardiographic marker of RV hypertrophy, we hypothesized that RVSLI might be able to reflect RV pressure load. Therefore, the purpose of this study was to characterize the diagnostic impact of the RVSLI in children with idiopathic pulmonary arterial hypertension (IPAH) in order to assess disease severity and to evaluate its value for the prediction of worse outcome. Forty-two children (aged 3-17 years) with IPAH were included in this retrospective study. The follow-up after baseline examination was 59 ± 17 months. Receiver-operating characteristic (ROC) curves and Kaplan-Meier analysis were used to discriminate a cut-off value of RVSLI and to assess its predictive value regarding morbidity and mortality. In 12/42 patients (29%) severe cardiovascular events (defined as death, lung transplantation, or Potts shunt) were observed (time to event 20 ± 22 months). Patients with an event showed higher RVSLI values (3.6 ± 1.2 mV vs. 2.6 ± 1.6 mV; p < 0.05). ROC analysis discriminated an RVSLI of 2.1 as the best cut-off value (area under the ROC curve: 0.79, sensitivity: 0.91, specificity: 0.70, p < 0.05) to detect patients with high-risk PAH (mPAP/mSAP ratio > 0.75). Relative risk for a severe event with an index > 2.1 mV was 1.76 (95% CI 1.21-3.20). Relative risk for death with RVSLI > 2.1 mV was 2.01 (95% CI 1.61-4.80). Our study demonstrates a strong relationship between RVSLI and disease severity in children with IPAH. An RVSLI > 2.1 mV at the time of first diagnosis is a predictor for patients at risk for cardiac events. As an adjunct to the usual diagnostic assessment this parameter may therefore contribute to the initial prognostic estimation.

Glutaraldehyde Treatment of Allografts and Aortic Outcomes Post-Norwood: Challenging Surgical Decision.

BACKGROUND: Glutaraldehyde (GA) treatment of allografts used for arch reconstruction prevents the immunologic sensitization that occurs with untreated allografts, but its use may cause tissue changes that predispose to recurrent obstruction. The objective was to determine whether GA treatment of allografts used in Norwood procedures increases the risk of recurrent aortic obstruction. METHODS: All infants who underwent a Norwood procedure between 2000 and 2015 were included. Cryopreserved pulmonary allografts were used for all arch reconstructions; starting in 2005 all were treated with GA before use. Complete follow-up was obtained, including survival, transplantation, and all repeat procedures. Competing risks analyses were used to assess for differences in aortic reintervention over time. RESULTS: Two hundred six infants (132 male) were included. There were 60 deaths and 14 transplantations; 5-year transplantation-free survival was 71.9%. GA treatment of patches (n = 142, 68.9%) was not predictive of death (hazard ratio [HR] 1.38, 95% confidence interval [CI]: 0.61 to 3.08). Fifty-five patients had at least one aortic reintervention and 31 patients (15.0%) required surgical aortic reintervention. At 1-year, freedom from all aortic reintervention was similar between patients with and without treated patches, but freedom from surgical aortic reintervention was lower in the treated group (87.6% versus 95.3%, p = 0.0256). GA treatment was not associated with the combined end point of catheter-based or surgical reintervention but was associated with specific need for surgical reintervention (HR 4.05, 95% CI: 1.19 to 13.77). CONCLUSIONS: GA treatment is associated with increased late surgical aortic reintervention. The advantages of decreased sensitization with GA treatment need to be balanced against the risk of aortic reobstruction.

Quantitative, Phenotypical, and Functional Characterization of Cellular Immunity in Children and Adolescents With Down Syndrome.

Background: Infections and autoimmune disorders are more frequent in Down syndrome, suggesting abnormality of adaptive immunity. Although the role of B cells and antibodies is well characterized, knowledge regarding T cells is limited. Methods: Lymphocyte subpopulations of 40 children and adolescents with Down syndrome and 51 controls were quantified, and phenotype and functionality of antigen-specific effector T cells were analyzed with flow cytometry after polyclonal and pathogen-specific stimulation (with varicella-zoster virus [VZV] and cytomegalovirus [CMV]). Results were correlated with immunoglobulin (Ig) G responses. Results: Apart from general alterations in the percentage of lymphocytes, regulatory T cells, and T-helper 1 and 17 cells, all major T-cell subpopulations showed higher expression of the inhibitory receptor PD-1. Polyclonally stimulated effector CD4+ T-cell frequencies were significantly higher in subjects with Down syndrome, whereas their inhibitory receptor expression (programmed cell death 1 [PD-1] and cytotoxic T-lymphocyte antigen 4 [CTLA-4]) was similar to that of controls and cytokine expression profiles were only marginally altered. Pathogen-specific immunity showed age-appropriate levels of endemic infection, with correlation of CMV-specific cellular and humoral immunity in all subjects. Among VZV IgG-positive individuals, a higher percentage of VZV-specific T-cell-positive subjects was seen in those with Down syndrome. Conclusions: Despite alterations in lymphocyte subpopulations, individuals with Down syndrome can mount effector T-cell responses with similar phenotype and functionality as controls but may require higher effector T-cell frequencies to ensure pathogen control.

Toward evidence-based diagnosis of myocarditis in children and adolescents: Rationale, design, and first baseline data of MYKKE, a multicenter registry and study platform.

The aim of this registry is to provide data on age-related clinical features of suspected myocarditis and to create a study platform allowing for deriving diagnostic criteria and, at a later stage, testing therapeutic interventions in patients with myocarditis. STUDY DESIGN AND RESULTS: After an initial 6-month pilot phase, MYKKE was opened in June 2014 as a prospective multicenter registry for patients from pediatric heart centers, university hospitals, and community hospitals with pediatric cardiology wards in Germany. Inclusion criteria consisted of age<18 years and hospitalization for suspected myocarditis as leading diagnosis at the discretion of the treating physician. By December 31, 2015, fifteen centers across Germany were actively participating and had enrolled 149 patients. Baseline data reveal 2 age peaks (<2 years, >12 years), show higher proportions of males, and document a high prevalence of severe disease courses in pediatric patients with suspected myocarditis. Severe clinical courses and early adverse events were more prevalent in younger patients and were related to severely impaired leftventricular ejection fraction at initial presentation. SUMMARY: MYKKE represents a multicenter registry and research platform for children and adolescents with suspected myocarditis that achieve steady recruitment and generate a wide range of real-world data on clinical course, diagnostic workup, and treatment of this group of patients. The baseline data reveal the presence of 2 age peaks and provide important insights into the severity of disease in children with suspected myocarditis. In the future, MYKKE might facilitate interventional substudies by providing an established collaborating network using common diagnostic approaches.

Home Exercise Training in Children and Adolescents with Pulmonary Arterial Hypertension: A Pilot Study.

Pulmonary arterial hypertension (PAH) is often associated with impaired exercise capacity. It has been shown that supervised training can improve exercise capacity in adult patients with PAH. The objective of this prospective study was to assess the feasibility of a home exercise training program in children with PAH. Nine children and adolescents (mean age 15.2 ± 3.8 years) with low-risk PAH (defined as mean pulmonary to systemic arterial pressure ratio <0.75; WHO functional class I and II) performed home-based exercise training for 16 weeks. Cardiopulmonary exercise testing and health-related quality of life was evaluated before and after 16 weeks of training. The amount of training at home and patients' well-being was supervised by periodical phone calls and online-questionnaires. Home exercise training was well tolerated in all patients, and no adverse events occurred. After 16 weeks of training, patients significantly improved their exercise capacity [treadmill running distance increased from 589.5 ± 153.9 to 747.9 ± 209.2 m (p = 0.036)]. Oxygen consumption at the anaerobic threshold increased from 1307.8 (±417) to 1406.4 (±418) ml (p = 0.028). Chronotropic index improved from 0.77 ± 0.12 to 0.82 ± 0.11 (p = 0.004) and was slightly related to the increase in running distance (r = 0.62; p = 0.07). Home exercise training is feasible in children and adolescents with low-risk PAH, and the preliminary results of this pilot study indicate beneficial effects. The observed increase in exercise capacity was accompanied by an improved chronotropic competence and increased oxygen consumption at the anaerobic threshold. Future research is needed to investigate the safety and efficacy of home exercise training in a larger population of children with PAH including also patients in WHO functional class III or IV.

Pulmonary hypertension in the intensive care unit. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

Acute pulmonary hypertension (PH) complicates the course of several cardiovascular, pulmonary and other systemic diseases in children. An acute rise of RV afterload, either as exacerbating chronic PH of different aetiologies (eg, idiopathic pulmonary arterial hypertension (PAH), chronic lung or congenital heart disease), or pulmonary hypertensive crisis after corrective surgery for congenital heart disease, may lead to severe circulatory compromise. Only few clinical studies provide evidence on how to best treat children with acute severe PH and decompensated RV function, that is, acute RV failure. The specific treatment in the intensive care unit should be based on the underlying pathophysiology and not only be focused on so-called 'specific' or 'tailored' drug therapy to lower RV afterload. In addition therapeutic efforts should aim to optimise RV preload, and to achieve adequate myocardial perfusion, and cardiac output. Early recognition of patients at high risk and timely initiation of appropriate therapeutic measures may prevent the development of severe cardiac dysfunction and low cardiac output. In patients not responding adequately to pharmacotherapy, (1) novel surgical and interventional techniques, temporary mechanical circulatory support with extracorporeal membrane oxygenation, (2) pumpless lung assist devices (3) and/or lung or heart-lung transplantation should be timely considered. The invasive therapeutic measures can be applied in a bridge-to-recovery or bridge-to-lung transplant strategy. This consensus statement focuses on the management of acute severe PH in the paediatric intensive care unit and provides an according treatment algorithm for clinical practice.

Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

UNLABELLED: : The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific forms such as pulmonary arterial hypertension (PAH)-congenital heart disease, pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, persistent PH of the newborn, and related cardiac dysfunction. METHODS: The writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2015) and held five face-to-face meetings with votings. Clinical trials, guidelines, and reviews limited to paediatric data were searched using the terms 'pulmonary hypertension' and 5-10 other keywords, as outlined in the other nine articles of this special issue. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on paediatric data only, or on adult studies that included >10% children. RESULTS: A total of 9 original consensus articles with graded recommendations (COR/LOE) were developed, and are summarised here. The topics included diagnosis/monitoring, genetics/biomarker, cardiac catheterisation, echocardiography, cardiac magnetic resonance/chest CT, associated forms of PH, intensive care unit/ventricular assist device/lung transplantation, and treatment of paediatric PAH. CONCLUSIONS: The multipaper expert consensus statement of the European Paediatric PVD Network provides a specific, comprehensive, detailed but practical framework for the optimal clinical care of children with PH.

First-pass and steady-state MR angiography of thoracic vasculature in children and adolescents.

Magnetic resonance angiography (MRA) is an established noninvasive imaging modality for detection and evaluation of vascular pathologies in children with congenital heart disease. Standard first-pass (FP)-MRA uses a 3-dimensional MRA sequence with an extracellular contrast agent, in which spatial resolution is limited by breath-hold duration, and image quality (IQ) is limited by motion artifacts. The purpose of this study was to compare the diagnostic confidence, IQ, and image artifacts of standard FP-MRA to a high-resolution, motion compensated steady-state (SS)-MRA of the thoracic vasculature in children and adolescents with congenital heart disease using a blood-pool contrast agent (gadofosveset trisodium). SS-MRA of the thoracic vasculature (technically successful in 90% of patients) offers superior diagnostic confidence and IQ compared with FP-MRA and shows fewer motion-related image artifacts. In addition, SS-MRA revealed findings missed by FP-MRA. Therefore, SS-MRA may prove specifically beneficial for imaging of thoracic vessels that are small and/or subject to motion.

Implantation of stents as an alternative to reoperation in neonates and infants with acute complications after surgical creation of a systemic-to-pulmonary arterial shunt.

Creation of a systemic-to-pulmonary shunt is still the firstline treatment in neonates with duct-dependent pulmonary circulation, or in patients with hypoplastic left heart syndrome as a part of the first stage of the Norwood sequence. Acute complications after such surgery, such as stenosis, thrombosis, or kinking, are potentially lifethreatening. These complications require immediate revision or exchange of the shunt. In this report, we discuss interventional treatment as an alternative to surgery in 5 patients with acute stenosis or complete occlusion of a shunt. The age of the patients ranged from 12 to 62 days, with a median of 30 days, and their weights ranged between 2.2 and 4.6 kilogrammes, with a median of 3.2 kilogrammes. In 3 patients, the shunts were central or of modified Blalock-Taussig type, while the 2 patients with hypoplastic left heart syndrome had shunts from the right ventricle to the pulmonary arteries. We implanted 6 coronary arterial and 2 peripheral stent systems. The diameter of the balloon used for implantation had a ratio to the shunt of 0.9. to 1. All shunts were successfully reopened by stenting. During follow-up, 3 patients underwent further procedures on an elective basis. We have one patient on the waiting list for further surgical intervention, but one patient died of septicaemia unrelated to the interventional procedure one month after implantation. In our limited experience, implantation of stents is an effective and long-lasting treatment for complications of shunts in an emergency situation.