Vascular health of fathers with history of intracytoplasmic sperm injection.

OBJECTIVE: Studies suggest that men who undergo assisted reproductive technologies (ART) may have a higher risk of cardiovascular disease; however, limited data on this matter is available. This observational pilot study aimed to investigate the overall vascular health of fathers with history of intracytoplasmic sperm injection (ICSI) compared to fathers whose partners conceived spontaneously. METHODS: Diet quality, physical activity, sedentary behavior as well as overall vascular function including the assessment of pulse wave analysis, intima-media thickness (cIMT), arterial stiffness of the common carotid artery (CCA) and blood lipids, were evaluated. RESULTS: A total of 34 fathers with history of ICSI and 29 controls (48.49 [46.32 - 57.09] years vs. 47.19 [40.62 - 55.18] years, p = 0.061) were included. After adjusting for age, no significantly increased cardiovascular risk was detected regarding vascular function. CONCLUSIONS: The results suggest an unaltered cardiovascular risk profile in fathers with history of ICSI. In the future, prospective multicenter studies are required to validate these preliminary results.

MRI pulmonary artery flow detects lung vascular pathology in preterms with lung disease.

BACKGROUND: Pulmonary vascular disease (PVD) affects the majority of preterm neonates with bronchopulmonary dysplasia (BPD) and significantly determines long-term mortality through undetected progression into pulmonary hypertension. Our objectives were to associate characteristics of pulmonary artery (PA) flow and cardiac function with BPD-associated PVD near term using advanced magnetic resonance imaging (MRI) for improved risk stratification. METHODS: Preterms <32 weeks postmenstrual age (PMA) with/without BPD were clinically monitored including standard echocardiography and prospectively enrolled for 3 T MRI in spontaneous sleep near term (AIRR (Attention to Infants at Respiratory Risks) study). Semi-manual PA flow quantification (phase-contrast MRI; no BPD n=28, mild BPD n=35 and moderate/severe BPD n=25) was complemented by cardiac function assessment (cine MRI). RESULTS: We identified abnormalities in PA flow and cardiac function, i.e. increased net forward volume right/left ratio, decreased mean relative area change and pathological right end-diastolic volume, to sensitively detect BPD-associated PVD while correcting for PMA (leave-one-out area under the curve 0.88, sensitivity 0.80 and specificity 0.81). We linked these changes to increased right ventricular (RV) afterload (RV-arterial coupling (p=0.02), PA mid-systolic notching (t2; p=0.015) and cardiac index (p=1.67×10-8)) and correlated echocardiographic findings. Identified in moderate/severe BPD, we successfully applied the PA flow model in heterogeneous mild BPD cases, demonstrating strong correlation of PVD probability with indicators of BPD severity, i.e. duration of mechanical ventilation (rs=0.63, p=2.20×10-4) and oxygen supplementation (rs=0.60, p=6.00×10-4). CONCLUSIONS: Abnormalities in MRI PA flow and cardiac function exhibit significant, synergistic potential to detect BPD-associated PVD, advancing the possibilities of risk-adapted monitoring.

Energy drinks: effects on pediatric 24-h ambulatory blood pressure monitoring. A randomized trial.

BACKGROUND: Energy drinks (EDs) are popular beverages among minors. To date, clinical studies investigating ED-induced effects on the pediatric cardiovascular system are sparse. This study aimed to investigate the effects of a single, bodyweight-adjusted ED dosage on 24-h ambulatory blood pressure monitoring (ABPM) in healthy children and adolescents. METHODS: This study was a randomized, single-blind, placebo-controlled, crossover clinical trial. Study participants received a single, bodyweight-adjusted ED amount or a placebo drink on 2 consecutive days at similar morning hours. Twenty-four-hour ABPM was assessed via an automated oscillometric blood pressure device after beverage consumption on both study days. RESULTS: A total of 17 healthy children and teenagers (13.90 (12.29-17.89) years) were included in the final analysis. The ED consumption led, compared to the placebo intake, to a significantly higher 24-h systolic (115.90 (110.22-118.04) vs. 110.64 (108.09-115.45) mmHg, p = 0.013) and diastolic blood pressure (66.08 (64.20-68.32) vs. 62.63 (61.40-66.46) mmHg, p = 0.005). CONCLUSIONS: The single, bodyweight-adjusted ED consumption is linked with a significantly higher systolic as well as diastolic 24-h blood pressure in healthy children and adolescents. Minors, particularly those with an increased cardiovascular morbidity, should be discouraged from drinking EDs. IMPACT: Energy drinks (EDs) are consumed by many children and teenagers. While adverse cardiovascular events after ED consumption were reported in the literature, the effects of these beverages on the pediatric 24-h blood pressure profile have not been systematically evaluated yet. In our manuscript, we demonstrate for the first time that acute ED consumption is associated with a significantly higher 24-h systolic blood pressure and diastolic blood pressure in healthy minors.

Tricuspid Annular Plane Systolic Excursion (TAPSE) correlates with mean pulmonary artery pressure especially 10 years after pediatric heart transplantation.

Tricuspid annular plane systolic excursion (TAPSE) is important in the noninvasive echocardiographic assessment of right heart function. This retrospective observational study shows correlations of TAPSE with invasive right heart catheterization parameters after pediatric heart transplantation (HTx). The study included patients after pediatric HTx with cardiac catheterizations in 2018/2019 and measurement of TAPSE (n = 52 patients with 57 examinations; 50.9% adults, 52.6% female, median age: 18.54 years). TAPSE was compared with normal values. Stepwise, linear and multiple regression were used to show influencing variables on TAPSE. Mean TAPSE z-score was -3.48 (SD: 2.25) and 68.4% of HTx-recipients showed abnormally reduced TAPSE (z-score ←2) compared to normal values. Multiple regression (p-value <0.001; corrected R2 = 0.338) showed significant correlations of time since HTx (p-value <0.001) and mPAP (p-value: 0.008) with TAPSE z-scores. Divided into subgroups (time since HTx <10 and ≥10 years), TAPSE and mPAP correlated only ≥10 years after HTx (p-value = 0.002). This study provides data of TAPSE even ≥10 years after pediatric HTx. Most patients showed a decreased TAPSE early after HTx, which improved over time. TAPSE z-scores correlated significantly with time since HTx and mPAP, especially ≥10 years post-HTx. Therefore, TAPSE must be used carefully in the early follow-up.

Kawasaki disease and increased cardiovascular risk: Is there a link to circulating glycocalyx biomarkers?

AIMS: Kawasaki disease (KD) is an acute systemic vasculitis with possible long-term impact of general cardio-vascular health. An endothelial glycocalyx disorder during the disease's acute phase might predispose to long-term vascular anomalies leading to endothelial dysfunction and atherosclerosis. To investigate any association between increased cardiovascular risk and endothelial glycocalyx, we assessed circulating glycocalyx components in patients with a KD history, and analysed their association with acute-phase clinical features and more importantly, with patients' current cardiovascular risk factors. METHODS: This prospective observational cohort study included 51 subjects: 31 patients with a history of KD, and 20 healthy subjects matched for age and sex. We analysed serum syndecan-1 and hyaluronan via ELISA. We assessed features reported during the acute phase of KD such as blood counts, C-reactive protein (CRP) levels and coronary artery aneurysms (CAA), and their current blood pressure and lipid markers in relation to measured glycocalyx components. RESULTS: Our multivariate analysis revealed that hyaluronan and syndecan-1 levels were not associated with KD. However, the latter exhibited a significant association with acute-phase blood count alterations in patients with KD. Furthermore, significant interactions of hyaluronan and syndecan-1 with certain cardiovascular risk factors like blood lipids and blood pressure were only present in KD patients. CONCLUSION: Vasculitis during KD's acute phase might predispose to a long-term endothelial glycocalyx alteration, influenced by other factors having a vascular impact such as blood pressure and circulating lipids. CLINICAL TRIAL REGISTRATION: German Clinical Trials Register on 25th February 2016, DRKS00010071 https://www.drks.de/drks_web/.

Vascular Health in Adults Born After Using Assisted Reproductive Technologies.

An increasing number of children are conceived by assisted reproductive technologies (ART). Several studies indicated vascular alterations in ART children. However, limited data is available within the adult ART population. Therefore, this study investigated the overall vascular health of young ART adults in comparison to spontaneously conceived peers. In total, 16 ART subjects and 22 spontaneously conceived peers (22.06 ± 2.21 years vs. 22.00 ± 2.14 years, p = 0.194) were enrolled for the assessment of endothelial function, brachial blood pressure, central blood pressure, pulse wave velocity, carotid intima-media thickness, and blood lipids. No significant differences in vascular function were detected between the in vitro fertilization subgroup (n = 9), the intracytoplasmic sperm injection subgroup (n = 7) and spontaneously conceived peers. This pilot study suggests an unimpaired vascular function in young ART adults. In the future, multi-centric studies with a greater sample size are required to confirm the results of the current study and enable precise cardiovascular risk stratification of the adult ART population.

Energy Drinks Decrease Left Ventricular Efficiency in Healthy Children and Teenagers: A Randomized Trial.

Background: Minors are considered the main consumer group of energy drinks (EDs). The aim of this study was to investigate the acute effects of ED consumption on left ventricular (LV) hemodynamics and efficiency in healthy children and teenagers. Methods: This study was a randomized, single-blind, placebo-controlled, crossover clinical trial. Study participants consumed a weight-adjusted amount of an ED or a placebo on two consecutive days. LV hemodynamics and efficiency parameters were evaluated non-invasively by generating LV pressure−volume loops (PVLs) through simultaneous echocardiography and blood pressure measurement. Results: A total of 24 children and teenagers (14.90 ± 2.27 years, 13 male) were included in the present study. Conventional echocardiographic parameters of LV function did not show significant differences between both beverage groups. The non-invasive generation of LV PVLs revealed a significantly lower cardiac efficiency 240 min after the ED consumption compared to the placebo intake (140.72 (133.21−149.73) mmHg vs. 135.60 (124.78−140.33) mmHg, p < 0.01). Conclusions: Acute ED consumption is associated with a significantly lower cardiac efficiency in healthy minors. The generation of non-invasive LV PVLs might be beneficial in the assessment of subtle changes in LV efficiency. Further studies need to investigate the influence of chronic ED consumption on LV function and morphology.

Conversion to everolimus in pediatric heart transplant recipients is a safe treatment option with an impact on cardiac allograft vasculopathy and renal function.

BACKGROUND: Cardiac allograft vasculopathy (CAV) and nephrotoxicity affect long-term survival after heart transplantation (HTX). Studies, mostly conducted in adults, showed a positive effect of everolimus (EVL) on these problems. We describe the effects of conversion of the immunosuppressive therapy to an everolimus including regime on CAV, renal function, and safety in heart transplanted children/adolescents. METHODS: This retrospective single-center study included 36 participants (mean time after HTX 6.3 ± 4.7 years). Descriptive pre/post-comparisons were performed with an observation period partially up to 4 years. Impact on CAV was assessed based on intravascular imaging and Stanford grading. Safety analysis included cytomegalovirus (CMV)-infection and acute rejection. RESULTS: In terms of CAV (9 out of 36 patients) four showed no progression, three an improvement, one a worsening; one new diagnosis. The average CrCl showed a significant improvement 6, 12, and 24 months after conversion regarding all patients (n = 29). There was no acute rejection or CMV-infection. CONCLUSION: Conversion to an EVL-based therapy after pediatric HTX is a safe immunosuppressive regime without increasing risk of acute rejection or CMV-infection. There was some evidence of reduction in progression of CAV and a significant improvement of the renal function.

Quality of life and patient satisfaction with outpatient care after heart transplantation in adult and pediatric patients - room for improvement?

Reduced adherence after heart transplantation increases the risk for acute rejection. Therefore, the aim of this study was to evaluate the patient's satisfaction with outpatient care and quality of life (QOL) after pediatric and adult heart transplantation. Observational study after pediatric (n = 22) and adult (n = 65) heart transplantation and the parents of the pediatric patients (n = 22) to evaluate the patients' satisfaction with outpatient care and QOL. Established standardized questionnaires were used for patient satisfaction (ZAP survey) and QOL (SF36); the latter was compared with the cohort of the BGS98 survey (BGS98 cohort). ZAP score: excellent results with almost all values >80. QOL: pediatric cohort showed significantly higher values in physical functioning (P = 0.041) and role physical (P = 0.003) but significantly lower values in the sub-scale general health (P = 0.02) compared to adult cohort. In comparison with BGS98 cohort, children showed almost similar results, whereas adult cohort showed worse values in physical and emotional functioning, but higher values regarding general health. The QOL of patients after pediatric heart transplantation is comparable to a standardized reference population in Germany, whereas adult patients show reduced physical and emotional functioning, but better values regarding general health. The patients' satisfaction with the outpatient care is very high.

Motor outcome, executive functioning, and health-related quality of life of children, adolescents, and young adults after ventricular assist device and heart transplantation.

OBJECTIVE: The aim of the current study is to measure long-term executive function, motor outcome, and QoL in children, adolescents, and young adults after VAD and Htx. METHODS: Patients were examined during routine follow-up. Investigation tools were used as follows: Examination for MND of motor outcomes, Epitrack® for attention and executive functioning, and Kidscreen-52 and EQ-5D-5L questionnaires for QoL. Additional data were retrospectively obtained by an analysis of patient medical records. RESULTS: Out of 145 heart transplant recipients at the department of pediatric cardiology of the University Hospital Munich, 39 were implanted with a VAD between 1992 and 2016. Seventeen (43.6%) patients died before or after Htx; 22 (56.4%) patients were included in our study. Mean age at transplant was 9.52 years (range: 0.58-24.39 years, median 9), and the mean follow-up time after Htx was 6.18 years (range: 0.05-14.60 years, median 5.82). MND examination could be performed in 13 patients (normal MND: n = 11, simple MND: n = 1, complex MND: n = 1). Executive functioning was tested in 15 patients. Two (13.3%) patients had good results, six (40%) average results, three (20%) borderline results, and four (26.7%) impaired results. QoL (Kidscreen n = 7, EQ-5D-5L n = 8) was similar to a healthy German population. CONCLUSION: Motor outcome, executive functioning and QoL in survivors of VAD bridging therapy and Htx can be good, though underlying diseases and therapies are associated with a high risk of cerebral ischemic or hemorrhagic complications.

Lateral Atrial Tunnel Fontan Operation Predisposes to the Junctional Rhythm.

The goal of the study was to compare the early postoperative course after fenestrated lateral atrial tunnel (LT) and non-fenestrated extracardiac conduit (EC) Fontan operation (FO) in a single center where both techniques were parallelly used. Between 2004 and 2012, 56(32.7%) children underwent LT and 113(66.1%) EC FO. The mean age was 3.7 ± 2.9 years and mean weight was 14.6 ± 7.3 kg. The most common malformation was hypoplastic left heart syndrome (50.9%). The general approach was to perform LT in children after hemi-Fontan operation and EC in patients after Glenn anastomosis. Medical records were retrospectively reviewed. The hospital survival was 100%. In EC group, cardiopulmonary bypass time (CPB) was shorter (p = 0.004) and less patients needed aortic cross-clamping (p < 0.001). Children after EC stayed longer in the hospital (p = 0.016) and manifested more often prolonged effusions (p = 0.038). The incidence of all forms of junctional rhythm was higher in the LT group, early postoperatively (p < 0.001), during hospitalization (p = 0.004) and at discharge (p < 0.001). Children after LT required more often temporary pacemaker stimulation (p < 0.001). Patients without postoperative normofrequent sinus rhythm had longer CPB time (p = 0.008) and were more often operated on with aortic cross-clamping (p = 0.028). Lateral atrial tunnel Fontan operation with fenestration facilitates early adaptation to the total passive pulmonary flow, but predisposes the patients to the loss of sinus rhythm. The crucial role in the preservation of sinus rhythm plays the last step of the multistage surgery of the single ventricle malformations, probably not only the surgical technique but also factors associated with the cardiopulmonary bypass.

Cardiovascular pre-participation screening in young athletes: Recommendations of the Association of European Paediatric Cardiology.

Sudden death in young competitive athletes can be avoided by implementation of pre-participation screening programmes. A screening programme should be performed only by trained physicians and should include the athlete's personal and family history, physical examination results, and the readings from a 12-lead-electrocardiogram. The athlete should undergo this screening programme every second year to detect progressive diseases. In addition, the programme should include detailed instructions to the athletes to pause training during infections in order to prevent sudden death due to myocarditis.

Reduced arterial stiffness in very fit boys and girls.

Low cardiorespiratory fitness is associated with higher cardiovascular risk, whereas high levels of cardiorespiratory fitness protect the cardiovascular system. Carotid intima-media thickness and arterial distensibility are well-established parameters to identify subclinical cardiovascular disease. Therefore, this study investigated the influence of cardiorespiratory fitness and muscular strength on carotid intima-media thickness and arterial distensibility in 697 children and adolescents (376 girls), aged 7-17 years. Cardiorespiratory fitness and strength were measured with the test battery FITNESSGRAM; carotid intima-media thickness, arterial compliance, elastic modulus, stiffness index ß, and pulse wave velocity ß were assessed by B- and M-mode ultrasound at the common carotid artery. In bivariate correlation, cardiorespiratory fitness was significantly associated with all cardiovascular parameters and was an independent predictor in multivariate regression analysis. No significant associations were obtained for muscular strength. In a one-way variance analysis, very fit boys and girls (58 boys and 74 girls>80th percentile for cardiorespiratory fitness) had significantly decreased stiffness parameters (expressed in standard deviation scores) compared with low fit subjects (71 boys and 77 girls<20th percentile for cardiorespiratory fitness): elastic modulus -0.16±1.02 versus 0.19±1.17, p=0.009; stiffness index ß -0.15±1.08 versus 0.16±1.1, p=0.03; and pulse wave velocity ß -0.19±1.02 versus 0.19±1.14, p=0.005. Cardiorespiratory fitness was associated with healthier arteries in children and adolescents. Comparison of very fit with unfit subjects revealed better distensibility parameters in very fit boys and girls.

Preventing cardiac diseases in childhood.

OBJECTIVE: The burden of cardiac disease in childhood is unknown. It will be a sum of 1% of living births in the general population, suffering from Congenital Heart Disease (CHD) + approximately 2.5% of the general population suffering from bicuspid aortic valve diseases + an unknown higher prevalence of acquired diseases. Cardiomyopathies, arrhythmias - sudden cardiac death (SCD), rheumatic heard disease, hypertension and accelerating atherosclerosis are among the most frequent. Adding on, genetic syndromes including cardiac defects, endocarditis and myocarditis we can address a large pediatric population worldwide, suffering from heart disease. Diagnosis and treatment of these diseases are not afforded in many countries worldwide due to luck of human and material resources. The aim of this paper is to describe how some of the above mentioned diseases can be either early detected or prevented. The working Group "Cardiovascular Prevention" of the Association of European Pediatric and Congenital Cardiology (AEPC) focused on some forms of them since its formation in 2011. These areas are: 1) some forms of critical CHD, 2) sudden cardiac death linked to sport activities and 3) detecting- preventing cardio vascular diseases CVD in the young. Methods-Populations: Measurements of pre and post ductal saturation of oxygen using pulse oximeters, after the first day from birth, can early and cheaply detect critical Ductal Arteriosus dependent pulmonary or systemic and cyanotic CHD, saving lives and decreasing significantly the cost of medical care. This screening test can be applied to all neonates as late as possible after their birth and before released to their homes. A combination of detailed medical history, physical examination and 12 lead ECG, during a pre-participation in sport activities medical screening test can prevent SCD, related to a variety of nosology. This combined screening test can be applied to all children before they are exposed to school or leisure sport activities. Screening to early detect and treating existent risk factors (RF) for CVD as well as preventing obesity and hypertension, contributes in lowering the burden of CVD. Specific screening tests as laboratory measurements of lipids, fasting glucose or regular measurements of Blood Pressure and waist to hip ratio in children with a family history of CVD or other co-morbidity that provokes accelerating atherosclerosis must be done on a regular basis. RESULTS: Since 2010, four European studies reporting the test accuracy of routine pulse oximetry screening, in over 150.000 babies, have delivered new data. A systematic review and meta-analysis of 230.000 screened babies, reported high specificity, moderate sensitivity and a low false-positive rate. Routine screening for critical CHD using pulse oximetry is being increasingly supported and was added to the recommended uniform screening panel in the USA in 2011. Evaluating children with CHD before their involvement in sport activities, so a clear view in what they can and what they can't to is vital for their safety. For children involved in competitive or leisure sport activities, an initial evaluation and a yearly F/U is vital. In cases of near SCD events an additional thorough investigation and appropriate management is required. Investigating the severity of the existing RF and cooperating with Pediatricians in their treatment (e.g. heredity forms of hyperlipidemias, existing hypertension) of them is essential. Furthermore preventing acceleration of atherosclerosis in patients with: Diabetes Mellitus I, II, chronic renal disease, post Kawasaki disease, post heart transplantation patients, Cardio-Metabolic Syndrome patients, by eradicating RF primordially or by alternating them by opposing a healthy life style or by medicine treatment, has sown in many studies to post pone clinical events in adulthood. DISCUSSION: As many studies have proved the role of preventive measures that can alternate the outcome of cardiac diseases in childhood. AEPC/Preventive Cardiology working group is in the process to publish in the near future guidelines on this topic.

Familiar Disposition of May-Thurner Syndrome-A Case Series.

May-Thurner syndrome is a venous compression syndrome of the pelvic vessels that represents a relevant risk factor for thrombus formation. The standard procedure to secure a diagnosis is venography, followed by endovascular therapy as the preferred treatment choice if the patient is symptomatic. In our case series, there are three related patients with May-Thurner syndrome. A 16-year-old female was admitted with pulmonary embolism, dyspnoea and hip pain. The compression syndrome was diagnosed with interventional venography, and the patient received venous stent implantation. Due to her family history, we also suspected her mother to be affected by the syndrome and elucidated the diagnosis shortly afterwards by invasive venography. Subsequently, we examined the patient's 19-year-old brother, and magnetic resonance imaging confirmed May-Thurner syndrome. A similar case series has not been published before. In this case, the family relation indicates a possible hereditary aspect of May-Thurner syndrome. This hypothesis should be the subject of further research. In conclusion, it is essential to assess family history thoroughly when treating patients with May-Thurner syndrome.

Vascular Health of Females with History of Assisted Reproductive Technology.

The use of assisted reproductive technologies (ART) for the treatment of infertility is gaining popularity. Limited data on the overall vascular health of females with history of ART are available. This pilot study aimed to investigate the overall vascular health of females with history of ART compared to individuals who conceived spontaneously. The assessment of overall vascular health included the measurement of brachial blood pressure, central blood pressure, and pulse wave velocity, as well as the evaluation of the arterial stiffness and carotid intima-media thickness (cIMT) of the common carotid arteries. Conventional blood lipids including lipoprotein a (Lp(a)) were also determined. In total, 45 females with history of ART and 52 females who conceived spontaneously were included (mean age: 47.72 ± 5.96 years vs. 46.84 ± 7.43 years, p = 0.525). An initial comparison revealed a significantly higher prevalence of elevated Lp(a) in ART females (p = 0.011). However, after multiple comparison correction, the significant result disappeared (p = 0.132). Within the cohort of ART females, no significantly higher cardiovascular risk was detected regarding vascular function. The potentially higher prevalence of elevated Lp(a) in ART females must be further investigated in future studies, as it might contribute to the impaired reproductive process in this cohort.

Energy Drinks and Adverse Health Events in Children and Adolescents: A Literature Review.

This review aims to investigate and summarize adverse health events in children and adolescents associated with energy drink (ED) consumption, while also exploring the impact of simultaneous trigger factors and/or preexisting health conditions. We searched the database of PubMed, Cochrane library, and Web of Science for cases associated with ED consumption in minors up to 9 May 2023. The literature written in English met inclusion criteria if patients were <18 years of age and the ED consumption was confirmed. Records, relevant articles, and reports that met all inclusion criteria were fully read by two researchers. In total, 18 cases reporting adverse health events were included. Of those, 45% affected the cardiovascular system, 33% the neuropsychological system, and 22% other organ systems. In 33% of cases, additional triggers were reported. In 44% preexisting health conditions were present. This literature review suggests that ED intake may well be associated with adverse health events in minors. The cardiovascular and the neuropsychiatric systems seem to be predisposed. ED consumption in combination with potential trigger factors or in the presence of preexisting health conditions appears to be critical. To prevent adverse health events in the future, children and adolescents should be informed about risk factors and responsible consumption behaviors.

Routine follow-up transjugular liver biopsy in Fontan patients: technical considerations and safety of an initial case series and literature review.

Introduction: Patients with Fontan palliation are susceptible to congestive hepatopathy and Fontan-associated liver disease (FALD) because of hemodynamic changes. The staging of liver fibrosis involves various methods, including invasive biopsy. Transjugular liver biopsy (TJLB) offers a less invasive alternative, enhancing liver disease surveillance in routine cardiac catheterization. We detail the technical aspects, share initial outcomes, and discuss existing literature. Methods/results: During routine follow-up cardiac catheterization indicated by hemodynamic or clinical alterations, four patients aged between 16 and 26 years with univentricular Fontan circulation and three patients with biventricular circulation underwent TJLB during routine surveillance catheterization. The examinations were performed under conscious sedation and local anesthesia without general anesthesia. Jugular access was obtained at the site of liver localization, and a 5 F multipurpose catheter was inserted into the liver veins. After hand angiography to delineate the local hepatic venous anatomy, an exchange wire was used to place the bioptome, and three consecutive biopsies were performed. There were no complications, especially perforation or bleeding. The technical success rate was 100%, with all obtained samples appropriate for histopathological diagnostics. The total additional procedure time was less than 20 min. Conclusion: TJLB is an attractive alternative method for obtaining liver specimens in the scope of FALD care. We believe that it should be performed during routine hemodynamic evaluations in Fontan patients and can be performed safely with very low additional time expenditure. As the biopsy site is intravascular, the risk of external bleeding or hematoma is significantly reduced despite the high intrahepatic pressures and the usually impaired coagulation profile in these patients. Based on our initial experience and the lower complication rates compared with other techniques, TJLB should be considered a standard approach in these patients and used more often during the long-term follow-up of Fontan patients. It can be performed in the same setting whenever a hemodynamic assessment of patients with congenital heart defects is required.

Case report: Beneficial long-term effect of the atrial-flow-regulator device in a pediatric patient with idiopathic pulmonary arterial hypertension and recurring syncope.

We report the long-term effect after successfully implanting an 8 mm Atrial-flow-regulator (AFR) device in a 7-year-old girl with idiopathic pulmonary hypertension with persistent syncope under triple therapy with significant improvement after implantation and absence of any further syncope. Early Implantation of the AFR device (Occlutech, Germany) can be efficient and safe interventional therapy option for pulmonary arterial hypertension with a history of syncope.

Evaluation of the diagnostic and prognostic potential of optical coherence tomography (OCT) of the pulmonary arteries during standardised right heart catheterisation in patients with pulmonary hypertension: a cross-sectional single-centre experience.

Background: Pulmonary hypertension (PH) is diagnosed based on an invasive evaluation of the mean pulmonary artery (PA) pressure. The morphological assessment of the pulmonary arteries was only recently not feasible. With the advent of optical coherence tomography (OCT)-imaging, an accessible tool allows to study PA morphology longitudinally. The primary hypothesis was that OCT distincts the PA structure of PH patients from control subjects. The secondary hypothesis was that PA wall thickness (WT) correlates with the progression of PH. Methods: This is a retrospective monocentric study of 28 paediatric patients with (PH group) and without PH (control group) who had undergone cardiac catheterisation including OCT imaging of the PA branches. OCT parameters analysed were WT and the quotient of WT and diameter (WT/DM) and those were compared between the PH group and the control group. In addition, the OCT parameters were aligned with the haemodynamic parameters to evaluate the potential of OCT as a risk factor for patients with PH. Results: WT and WT/DM in the PH group were significantly higher compared to the control group {WT: 0.150 [0.230, range (R): 0.100-0.330] vs. 0.100 [0.050, R: 0.080-0.130] mm, P<0.001; WT/DM: 0.06 [0.05] vs. 0.03 [0.01], P=0.006}. There were highly significant correlations between WT and WT/DM with the haemodynamic parameters mean pulmonary arterial pressure (mPAP) [Spearman correlation coefficient (rs) =0.702, P<0.001; rs=0.621, P<0.001], systolic pulmonary arterial pressure (sPAP) (rs=0.668, P<0.001; rs=0.658, P<0.001) and WT and pulmonary vascular resistance (PVR) (rs=0.590, P=0.02). Also, there was a significant correlation between WT and WT/DM and the risk factors quotient of mPAP and mean systemic arterial pressure (mSAP) (mPAP/mSAP) (rs=0.686, P<0.001; rs=0.644, P<0.001) and pulmonary vascular resistance index (PVRI) (rs=0.758, P=0.002; rs=0.594, P=0.02). Conclusions: OCT can detect significant differences in WT of the PA in patients with PH. Furthermore, the OCT parameters correlate significantly with haemodynamic parameters and risk factors for patients with PH. More investigations are required to evaluate to what extent the impact of OCT can contribute to the clinical care of children with PH.