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Introduction: Fetal magnetocardiography (fMCG) is considered the best technique for diagnosis of fetal arrhythmia. It is superior to more widely used methods such as fetal, fetal electrocardiography, and cardiotocography for evaluation of fetal rhythm. The combination of fMCG and fetal echocardiography can provide a more comprehensive evaluation of fetal cardiac rhythm and function than is currently possible. In this study, we demonstrate a practical fMCG system based on optically pumped magnetometers (OPMs). Methods: Seven pregnant women with uncomplicated pregnancies underwent fMCG at 26–36 weeks' gestation. The recordings were made using an OPM-based fMCG system and a person-sized magnetic shield. The shield is much smaller than a shielded room and provides easy access with a large opening that allows the pregnant woman to lie comfortably in a prone position. Results: The data show no significant loss of quality compared to data acquired in a shielded room. Measurements of standard cardiac time intervals yielded the following results: PR = 104 ± 6 ms, QRS = 52.6 ± 1.5 ms, and QTc = 387 ± 19 ms. These results are compatible with those from prior studies performed using superconducting quantum interference device (SQUID) fMCG systems. Conclusions: To our knowledge, this is the first European fMCG device with OPM technology commissioned for basic research in a pediatric cardiology unit. We demonstrated a patient-friendly, comfortable, and open fMCG system. The data yielded consistent cardiac intervals, measured from time-averaged waveforms, compatible with published SQUID and OPM data. This is an important step toward making the method widely accessible. [ABSTRACT FROM AUTHOR]

Performance of cardiovascular magnetic resonance (CMR) in the planning phase of percutaneous pulmonary valve implantation (PPVI) is needed for the accurate delineation of the right ventricular outflow tract (RVOT), coronary anatomy and the quantification of right ventricular (RV) volume overload in patients with significant pulmonary regurgitation (PR). This helps to find the correct timings for the intervention and prevention of PPVI-related complications such as coronary artery compression, device embolization and stent fractures. A defined CMR study protocol should be set for all PPVI candidates to reduce acquisition times and acquire essential sequences that are determinants for PPVI success. For correct RVOT sizing, contrast-free whole-heart sequences, preferably at end-systole, should be adopted in the pediatric population thanks to their high reproducibility and concordance with invasive angiographic data. When CMR is not feasible or contraindicated, cardiac computed tomography (CCT) may be performed for high-resolution cardiac imaging and eventually the acquisition of complementary functional data. The aim of this review is to underline the role of CMR and advanced multimodality imaging in the context of pre-procedural planning of PPVI concerning its current and potential future applications. [ABSTRACT FROM AUTHOR]

Background Patients with congenital cardiovascular disease involving the right ventricular outflow tract and with prosthetic valves in the heart are at high risk for developing infective endocarditis (IE). Recently, there has been concern about relatively high rates of IE after percutaneous pulmonary valve implantation (PPVI). Although there are factors specific to percutaneous valves that could plausibly contribute to the risk of IE, including procedural steps prior to implantation such as crimping the valved stent or mechanical forces during dilation, little is known about actual risk factors for this complication. Methods and results The purpose of this study was to assess the incidence rate of IE after PPVI in a single centre long-term experience. The cumulative follow-up time comprised 883.4 patient years for 226 transcatheter valves implanted in the pulmonic position. The annualized incidence rate of IE for all patients receiving valved stents in the RVOT was 1.9%. Freedom from IE 8 years after PPVI was estimated at 87%. The probability for valve removal because of IE was estimated after 8 years at 7%. Conclusion In our experience, the incidence rate of IE after PPVI is acceptable and comparable to surgically implanted biological valves. Despite some IE events, freedom from reoperation was high and there was good performance of the valve in long-term follow-up. [ABSTRACT FROM AUTHOR]

Objective Multiple studies have demonstrated the predictive value of arterial stiffness parameters like augmentation index (AIx) for cardiovascular events, the onset of hypertension, and the progression of heart failure. There is evidence that arterial stiffness is increased in some diagnostic subgroups of patients with congenital heart defects (CHD). This study aims to investigate AIx in a large cross-sectional cohort of patients with CHD. Patients and methods We prospectively examined 1125 consecutive patients with various congenital heart defects (27.3 ± 12.1 years, 464 female) referred for routine cardiopulmonary exercise testing (CPET) in our institution, and 322 healthy volunteers (29.4 ± 18.4 years, 165 female). AIx was estimated in supine position using the oscillometric Vicorder device (SMT medical, Würzburg, Germany). Afterward patients performed a CPET. Results In multivariable regression, presence of a CHD emerged as independent risk factor for higher AIx (p < .001). AIx was also higher in older (p < .001), smaller (p < .001) and heavier (p < .001) patients and in females (p = .008). Patients with aortic stenosis (p < .001), Tetralogy of Fallot (p < .001), transposition of the great arteries after atrial switch (p < .001) or Rastelli procedure (p = .013) and after Fontan procedure (p = .002) had higher AIx. Higher peak oxygen uptake (p < .001) and an ACE-inhibitor (p = .088) were associated with a lower AIx. Conclusions AIx is increased in patients with CHD. Several diagnostic subgroups are at risk. A better understanding of pathophysiologic mechanisms, genetic predisposition, the role of surgical aortic scars or implanted conduits/patches and medication is needed to define the value of AIx for further cardiovascular risk assessment in this cohort. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Early circuit separation enhances the long-term success of Fontan haemodynamics. To test this hypothesis, we analysed the postoperative cardiopulmonary capacity in children and adults. Patients: Spiroergometry was performed at least twice in 43 patients with a median age of 14 (range: 7–43) years, with a median time interval of 4.6 (1.1–10.4) years between early and late testing. Twenty-eight patients had been operated on in childhood and 15 as adults. The exercise capacity (W max) and oxygen consumption capacity () were compared between children and adults. Results: The in children early postoperatively was better than in adults (median 27.9 vs 22.9, p =0.032). Both (median 30.1mlmin−1 kg−1 vs 16.9mlmin−1 kg−1, p <0.001), and W max (median 2.2Wkg−1 vs 1.4Wkg−1, p <0.001) were significantly better in children late after surgery. In the patient group as a whole, there was a significant decrease of between early and later testing (median 26.5lmin−1 kg−1 vs 20.7lmin−1 kg−1, p <0.001). Conclusions: Fontan palliation in early childhood results in better cardiopulmonary capacity during long-term follow-up. Regular surveillance of the physical capacity by spiroergometry is indispensable for the supervision of patients with Fontan haemodynamics. [Copyright &y& Elsevier]

Intravascular or intracardiac stenoses occur in many forms of congenital heart disease (CHD). Therefore, the implantation of stents has become an accepted interventional procedure for stenotic lesions in pediatric cardiology. Furthermore, stents are know to be used to exclude vessel aneurysm or to ensure patency of existing or newly created intracardiac communications. With the further refinement of the first generation of devices, a variety of "modern" stents with different design characteristics have evolved. Despite the tremendous technical improvement over the last 20 years, the "ideal stent" has not yet been developed. Therefore, the pediatric interventionalist has to decide which stent is suitable for each lesion. On this basis, currently available stents are discussed in regard to their advantages and disadvantages for common application in CHD. New concepts and designs developed to overcome some of the existing problems, like the failure of adaptation to somatic growth, are presented. Thus, in the future, biodegradable or growth stents might replace the currently used generation of stents. This might truly lead to widening indications for the use of stents in the treatment of CHD. [ABSTRACT FROM AUTHOR]

Background: Protein S-100 is found in extracerebral sources. The aim of our study was to examine the content of protein S-100 in native pericardial fluid as well as in postoperative extracerebral fluids after cardiac surgery in children. Methods: We conducted a prospective study in 90 children measuring protein S-100 concentration in pericardial fluid directly after opening the pericardial sac before starting with cardiac surgery. Postoperatively, we examined pleural, peritoneal and pericardial fluid. Results: Pericardial fluid sampled directly after opening the pericardial sac has a protein S-100 content of 3.2 (1.3- 5.4) μg/L. Postoperatively, protein S-100 content was 0.89 (0.56-2.6) μg/L in pleural effusion, 0.14 (0.1-1.1) μg/L in peritoneal fluid and 2.75 (2.2-24.4) μg/L in pericardial fluid. The protein S-100 concentration in pericardial fluid before and after cardiac operation did not differ significantly. Pericardial protein S-100 concentrations were significantly higher than pleural and peritoneal protein S-100 concentrations. Conclusions: Protein S-100 is present in extracerebral fluids before and after cardiac surgery in children. The time point of fluid withdrawal after the operation did not influence the protein S-100 concentration. [ABSTRACT FROM AUTHOR]

Objective: Low pulmonary artery pressure (PAP) and unobstructed flow in the pulmonary arteries (PA) and the systemic veins are the most important factors for optimal long-term Fontan hemodynamics. We retrospectively analyzed the impact of transcatheter interventions for treatment of stenoses in the Fontan pathway to optimize the circulation, with special attention to chronic ascites. Patients and Design: Sixty-three of a total of 112 patients underwent heart catheterization 4.6 (1.0–11.4) years after Fontan operation. The median age of the patients at the end of follow-up was 11 (3.1–45) years. Patients were divided into two groups, one with chronic ascites and one without. The impact of elevated PAP and of morphologic stenoses in the Fontan pathway for the development of chronic ascites was analyzed. Results: Ascites was observed in 11 patients; 5 of them had stenosis in the Fontan pathway (P = 0.010). High PAP of 15 mmHg or more (n = 13) was correlated positively with the development of ascites (5 of 13, P = 0.040). Twenty interventions for removal of stenosis in the Fontan pathway were performed in 14 patients. We observed temporary relief of ascites in all and complete resolution of ascites after alleviation of the stenosis in 2 patients with PAP <12 mmHg. Conclusion: Transcatheter treatment of stenosis can effectively improve the hemodynamics in Fontan circulation, even in patients without measurable pressure gradient. In those with low PAP, it may lead to the disappearance of chronic ascites. [ABSTRACT FROM AUTHOR]

Background: We compared two different near-infrared spectrophotometers: cerebral tissue oxygenation index (TOI) measured by NIRO 200 and regional cerebral oxygenation index (rSO2) measured by INVOS 5100 with venous oxygen saturation in the jugular bulb (SjO2) and central SvO2 from the superior caval vein (SVC) during elective cardiac catheterization in children. Methods: A prospective observational clinical study in 31 children with congenital heart defects in a catheterization laboratory was undertaken. TOI was compared with SjO2 in the left jugular bulb and with SvO2. rSO2 was compared with SjO2 from the right jugular bulb and SvO2. Linear regression analysis and Pearson’s correlation coefficient were calculated and Bland–Altman analyses were performed. Results: Cerebral TOI and SjO2 were significantly correlated ( r = 0.56, P < 0.0001), as well as TOI and SvO2 with r = 0.74 ( P < 0.0001). Bland–Altman plots showed a mean bias of −4.3% with limits of agreement of 15.7% and −24.3% for TOI and SjO2 and a mean bias of −4.9% with limits of agreement of 10.3% and −20.1% for TOI and SvO2. Cerebral rSO2 and SjO2 showed a significant correlation ( r = 0.83, P < 0.0001) and rSO2 and SvO2 showed excellent correlation with r = 0.93 ( P < 0.0001). Bland–Altman plots showed a mean bias of −5.2% with limits of agreement of between 8.4% and −18.8% for rSO2 and SjO2 and a mean bias of 5.6% with limits of agreement of 13.4% and −2.2% for rSO2 and SvO2. Conclusions: Both near-infrared spectroscopy devices demonstrate a significant correlation with SjO2 and SvO2 values; nevertheless both devices demonstrate a substantial bias of the measurements to both SjO2 and SvO2. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Biventricular repair of complete atrioventricular septal defect (CAVSD) with small left ventricle aims to restore the normal loading conditions of the left ventricle. This report retrospectively evaluates the outcome of biventricular repair in 19 children with CAVSD and a small left ventricle. Methods: Our computer database was searched for all patients with CAVSD operated on between January 1988 and December 2005. Patients who underwent biventricular repair of CAVSD were considered for investigation if they had a preoperative left ventricle-to-right ventricle long axis ratio (LAR) of <1.1 as determined by cardiac catheterization. Results: There were 259 patients who underwent surgical correction of complete atrioventricular septal defect. Nineteen (10.3%) of 184 patients who underwent biventricular repair had small left ventricle based on LAR measurements. These children had no other associated congenital heart anomalies and had no previous surgery. Nine of these children had associated Trisomy 21. There were 10 (52.6%) males and 9 (47.4%) females, with age of 1–210 (122±67) days and weight of 2.5–9.0 (5.26±1.7) kg at surgery. Mean LAR was 0.76±0.14. Two patients (10.5%) died on the 8th and 11th postoperative day, respectively. Both had very small left ventricle (LAR of 0.45 and 0.60, respectively) and received ECMO support for postoperative low output syndrome and intractable pulmonary hypertension. A patient with an LAR of 0.62 who had ECMO support for postoperative myocardial failure underwent successful heart transplantation on the 21st postoperative day. The long-term survivors (89.5%) with LAR>0.65 had an uncomplicated postoperative course, had undergone regular follow-up (65±36 months) with echocardiographic assessment of the left ventricle, and had good left ventricular function. There was no early reoperation for residual ventricular septal defect, left AV valve regurgitation, or left ventricular outflow tract obstruction. Late reoperation was performed in three patients (17.4%) who underwent mitral valve repair for significant regurgitation in the 18th, 59th, and 87th month postoperatively. Conclusions: Biventricular repair of CAVSD with small left ventricle in infants and children whose LAR is >0.65, although not without risks, improve patients’ functional and clinical status even in long-term follow-up. Particular caution should be taken in patients with LAR of <0.65, since these are patients who may not be amenable to biventricular repair, but for whom univentricular palliation may be more suitable. [Copyright &y& Elsevier]

Objectives: This study sought to evaluate the use of covered Cheatham-platinum (CP) stents in the treatment of aortic coarctation (CoA). Background: Aortic aneurysms and stent fractures have been encountered after surgical and transcatheter treatment for CoA. Covered stents have previously been used in the treatment of abdominal and thoracic aneurysms in adults. We implanted covered CP stents as a rescue treatment in patients with CoA aneurysms or previous stent-related complications and in patients at risk of developing complications because of complex CoA anatomy or advanced age. Methods: Thirty-three covered CP stents were implanted in 30 patients; 16 patients had had previous procedures. The remaining patients had complex or near-atretic CoA. Results: The mean patient age and weight were 28 (±17.5) years (range 8 to 65 years), and 62 (±13) kg (range 28 to 86 kg), respectively. The systolic gradient across the CoA decreased from a mean (±SD) of 36 ± 20 mm Hg before to a mean of 4 ± 4 mm Hg after the procedure (p < 0.0001), and the diameter of the CoA increased from 6.4 ± 3.8 mm to 17.1 ± 3.1 mm (p < 0.0001). The follow-up period was up to 40 months (mean, 11 months). All stents were patent and in good position on computed tomography or magnetic resonance imaging performed three to six months later. In 43% of the patients antihypertensive medication was either decreased or stopped. Conclusions: Covered CP stents may be used as the therapy of choice in patients with complications after CoA repairs, whereas they provide a safe alternative to conventional stenting in patients with severe and complex CoA lesions or advanced age. [Copyright &y& Elsevier]

Abstract: Objective: We set out to examine whether the extracardiac Fontan operation (ECFO) in young children is beneficial for the early postoperative course and whether it has a negative impact on the mid-term hemodynamics and growth of the children due to absent growth potential of the prosthetic conduit. Therefore we compared our medium-term experience with ECFO in children under 4 years of age to that in older children regarding the incidence of postoperative complications, somatomotoric development and conduit status. Methods and results: Between 11/95 and 12/02 ECFO was performed in 30 children under 4 years of age and 21 older children aged 4–13 years. There were no deaths in children under 4 years of age and two older children died. No prolonged support (>72h) of suprarenin was required in small children compared to 4 older children. In twenty-seven children, who underwent postoperative heart catheterization no pulmonary artery or systemic vein distortion occurred. One re-operation and one transcatheter intervention were performed because of the partial conduit stenosis. During the median follow-up of 4.8 years a manifestly accelerated postoperative weight gain was observed in children operated on under 4 years of age, compared to that in older children (up to the 50 vs. 10th percentile, P<0.05). Conclusions: The ECFO could be performed in young children with low morbidity and mortality rates. In the majority of patients, implanted prosthetic grafts maintain stable form without the development of stenosis. Accelerated somatic development, especially in small children, is to be observed after completion of the Fontan circulation. [Copyright &y& Elsevier]

Objective We aimed to analyze somatic growth of patients after total cavopulmonary connection (TCPC) as well as to identify factors influencing postoperative catch-up growth. Methods A total of 309 patients undergoing TCPC at 4 years old or less between 1994 and 2021 were included. Weight for age z-score (WAZ) and height for age-z-score (HAZ) at TCPC and at postoperative time between 1 and 3 years were calculated. Factors influencing somatic growth were analyzed. Results Most frequent diagnosis and initial palliation were hypoplastic left heart syndrome (HLHS) (34%) and the Norwood procedure (51%), respectively. Median age and weight at TCPC were 2.0 (IQR: 1.7–2.5) years and 11.3 (10.5–12.7) kg, respectively. Median 519 days after TCPC, a significant increase in WAZ (−0.4 to −0.2, p < 0.001) was observed, but not in HAZ (−0.6 to −0.6, p = 0.38). Older age at TCPC (p < 0.001, odds ratio [OR]: 2.6) and HLHS (p = 0.007, OR: 2.2) were risks for low WAZ after TCPC. Older age at TCPC (p = 0.009, OR: 1.9) and previous Norwood procedure (p = 0.021, OR: 2.0) were risks for low HAZ after TCPC. Previous bidirectional cavopulmonary shunt (BCPS) was a protective factor for both WAZ (p = 0.012, OR: 0.06) and HAZ (p = 0.028, OR: 0.30) at TCPC. Conclusion In patients undergoing TCPC at the age of 4 years or less, a significant catch-up growth was observed in WAZ after TCPC, but not in HAZ. Previous BCPS resulted to be a protective factor for a better somatic development at TCPC. HLHSs undergoing Norwood were considered as risks for somatic development after TCPC. [ABSTRACT FROM AUTHOR]

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19–58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning–Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning–Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES To evaluate longitudinal systemic ventricular function and atrioventricular valve regurgitation in patients after the neonatal Norwood procedure. METHODS Serial postoperative echocardiographic images before Fontan completion were assessed in neonates who underwent the Norwood procedure between 2001 and 2020. Ventricular function and atrioventricular valve regurgitation were compared between patients with modified Blalock–Taussig shunt and right ventricle to pulmonary artery conduit. RESULTS A total of 335 patients were identified including 273 hypoplastic left heart syndrome and 62 of its variants. Median age at Norwood was 8 (7–12) days. Modified Blalock–Taussig shunt was performed in 171 patients and the right ventricle to pulmonary artery conduit in 164 patients. Longitudinal ventricular function and atrioventricular valve regurgitation were evaluated using a total of 4352 echocardiograms. After the Norwood procedure, ventricular function was initially worse (1–30 days) but thereafter better (30 days to stage II) in the right ventricle to pulmonary artery conduit group (P  < 0.001). After stage II, the ventricular function was inferior in the right ventricle to the pulmonary artery conduit group (P  < 0.001). Atrioventricular valve regurgitation between the Norwood procedure and stage II was more frequent in the modified Blalock–Taussig shunt group (P  < 0.001). After stage II, there was no significant difference in atrioventricular valve regurgitation between the groups (P  = 0.171). CONCLUSIONS The effect of shunt type on haemodynamics after the Norwood procedure seems to vary according to the stage of palliation. After the Norwood, the modified Blalock–Taussig shunt is associated with poorer ventricular function and worse atrioventricular valve regurgitation compared to right ventricle to pulmonary artery conduit. Whereas, after stage II, modified Blalock–Taussig shunt is associated with better ventricular function and comparable atrioventricular valve regurgitation, compared to the right ventricle to pulmonary artery conduit. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES In this study, we aimed to compare infants with univentricular hearts who underwent an initial ductus stenting to those receiving a surgical systemic-to-pulmonary shunt (SPS). METHODS All infants with univentricular heart and ductal-dependent pulmonary blood flow who underwent initial palliation with either a ductus stenting or a surgical SPS between 2009 and 2022 were reviewed. Outcomes were compared after ductus stenting or SPS including survival, probability of re-interventions and the probability to reach stage II palliations. RESULTS A total of 130 patients were evaluated, including 49 ductus stenting and 81 SPSs. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19 patients. There was comparable hospital mortality (2.0% stent vs 3.7% surgery, P  = 0.91) between the groups, but shorter intensive care unit stay (median 1 vs 7 days, P  < 0.01) and shorter hospital stay (median 7 vs 17 days, P  < 0.01) were observed in patients with initial ductus stenting, compared to those with SPS. However, acute procedure-related complications were more frequently observed in patients with ductus stenting, compared with those with SPS (20.4 vs 6.2%, P  = 0.01), and 10 patients needed a shunt procedure after the initial ductus stent. The cumulative incidence of reaching stage II was similar between ductus stenting and SPS (88.0 vs 90.6% at 12 months, P  = 0.735). Pulmonary artery (PA) index (median 194 vs 219 mm2/m2, P  = 0.93) at stage II was similar between patients with ductus stenting and SPS. However, the ratio of the left to the right PA index [0.69 (0.45–0.95) vs 0.86 (0.51–0.84), P  = 0.015] was higher in patients who reached stage II with surgical shunt physiology, compared with patients with ductus stent physiology. CONCLUSIONS After initial ductus stenting in infants with univentricular heart, survival is comparable and post-procedural recovery shorter, but more acute stent dysfunctions and lower development of left PA are observed, compared to acute shunt dysfunctions. The less invasive procedure and shorter hospital stay are at the expense of more stent reinterventions. [ABSTRACT FROM AUTHOR]

We present a 7-year-old boy with tuberous sclerosis and congenital segmental lymphedema (CSL) of the left leg, as well as two aortic aneurysms. He was treated with everolimus (EVE) since the age of 14 months. His CSL regressed under treatment with EVE. His first aneurysms required operative intervention at age of 17 months. Four months afterward a new aortic aneurysm had been detected above the Dracon graft, but this one remained stable since that time. The patient didn't experience severe side effects. EVE has been well tolerated without disturbance of somatic growth or serious adverse effect. [ABSTRACT FROM AUTHOR]

Children with congenital heart disease (CHD) undergoing cardiac surgery on cardiopulmonary bypass (CPB) are at risk for systemic inflammation leading to endothelial dysfunction associated with increased morbidity. Bioactive adrenomedullin (bio-ADM) is a peptide regulating vascular tone and endothelial permeability. The aim of this study was to evaluate the dynamics of plasma bio-ADM in this patient cohort and its role in capillary leak. Plasma samples from 73 pediatric CHD patients were collected for bio-ADM measurement at five different timepoints (TP) in the pre-, intra-, and post-operative period. The primary endpoint was a net increase in bio-ADM levels after surgery on CPB. Secondary endpoints included association of bio-ADM levels with clinical signs for endothelial dysfunction. Bio-ADM levels increased after surgery on CPB from pre-operative median of 12 pg/mL (IQR [interquartile range] 12.0–14.8 pg/mL) to a maximum post-operative median of 48.8 pg/mL (IQR 34.5–69.6 pg/mL, p<0.001). Bio-ADM concentrations correlated positively with post-operative volume balance, (r=0.341; p=0.005), increased demand for vasoactive medication (duration: r=0.415; p<0.001; quantity: TP3: r=0.415, p<0.001; TP4: r=0.414, p<0.001), and hydrocortisone treatment for vasoplegia (bio-ADM median [IQR]:129.1 [55.4–139.2] pg/mL vs. 37.9 [25.2–64.6] pg/mL; p=0.034). Patients who required pleural effusion drainage revealed higher bio-ADM levels compared to those who did not (median [IQR]: 66.4 [55.4–90.9] pg/mL vs. 40.2 [28.2–57.0] pg/mL; p<0.001). Bio-ADM is elevated in children after cardiac surgery and higher levels correlate with clinical signs of capillary leakage. The peptide should be considered as biomarker for endothelial dysfunction and as potential therapeutic target in this indication. [ABSTRACT FROM AUTHOR]

Aims: High-sensitive troponin T (hs-TnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and C-reactive protein (CRP) are established prognostic biomarkers for cardiovascular (CV) morbidity and mortality and frequently used in symptomatic and/or hospitalized adults with congenital heart disease (ACHD). Their prognostic value in clinically stable ACHD has not yet been well established. This study investigates the predictive value of hs-TnT, NT-proBNP, and CRP for survival and CV events in stable ACHD. Methods and results: In this prospective cohort study, 495 outpatient ACHD (43.9 ± 10.0 years, 49.1% female) underwent venous blood sampling including hs-TnT, NT-proBNP, and CRP. Patients were followed up for survival status and the occurrence of CV events. Survival analyses were performed with Cox proportional hazards regression analysis and Kaplan–Meier curves. During a mean follow-up of 2.8 ± 1.0 years, 53 patients (10.7%) died or reached a cardiac-related endpoint including sustained ventricular tachycardia, hospitalization with cardiac decompensation, ablation, interventional catheterization, pacer implantation, or cardiac surgery. Multivariable Cox regression revealed hs-TnT (P = 0.005) and NT-proBNP (P = 0.018) as independent predictors of death or cardiac-related events in stable ACHD, whilst the prognostic value of CRP vanished after multivariable adjustment (P = 0.057). Receiver-operator characteristic curve analysis identified cut-off values for event-free survival of hs-TnT ≤9 ng/L and NT-proBNP ≤200 ng/L. Patients with both increased biomarkers had a 7.7-fold (confidence interval 3.57–16.40, P < 0.001) higher risk for death and cardiac-related events compared with patients without elevated blood values. Conclusion: Subclinical values of hs-TnT and NT-proBNP are a useful, simple, and independent prognostic tool for adverse cardiac events and survival in stable outpatient ACHD. Registration: German Clinical Trial Registry DRKS00015248. Graphical Abstract [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Effects of aortopulmonary collaterals (APCs) on outcomes after the total cavopulmonary connection (TCPC) are unclear. This study evaluated the incidence of APCs before and after TCPC and analysed the impacts of APCs on adverse outcomes. METHODS A total of 585 patients, who underwent TCPC from 1994 to 2020 and whose preoperative angiographies were available, were included. Pre-TCPC angiograms in all patients were used for the detection of APCs, and post-TCPC angiograms were evaluated in selected patients. Late adverse events included late death, protein-losing enteropathy (PLE) and plastic bronchitis (PB). RESULTS The median age at TCPC was 2.3 (1.8–3.4) years with a body weight of 12 (11–14) kg. APCs were found in 210 patients (36%) before TCPC and in 81 (14%) after TCPC. The closure of APCs was performed in 59 patients (10%) before TCPC, in 25 (4.2%) at TCPC and in 59 (10%) after TCPC. The occurrences of APCs before and after TCPC were not associated with short-term or mid-term mortality. The APCs before TCPC were associated with chylothorax (P  = 0.025), prolonged chest tube duration (P  = 0.021) and PB (P  = 0.008). The APCs after TCPC were associated with PLE (P  < 0.001) and PB (P  < 0.001). With APCs following TCPC, freedom from PLE and PB was lower than without (P  < 0.001, P  < 0.001). CONCLUSIONS APCs before TCPC were associated with chylothorax, prolonged chest tube duration and PB. APCs after TCPC were associated with both PLE and PB. The presence of APCs might affect the lymph drainage system and increase the incidence of chylothorax, PLE and PB. [ABSTRACT FROM AUTHOR]

Background: Pressure measurement in total cavopulmonary connection (TCPC) patients is a domain of cardiac catheterization. 4D velocity encoded cardiovascular magnetic resonance (4D-flow MRI) offers an alternative for assessment of even minor pressure differences. The scope of this study was to measure even minor pressure differences in the anastomosis of TCPC patients, who are clinically uncompromised. Methods: Twenty-four patients (median 15 years [8;34]) with TCPC were studied prospectively by 4D-flow MRI. Pressure differences between superior vena cava (SVC) and extracardiac conduit (C) to both right pulmonary artery (RPA) and left pulmonary artery (LPA) were assessed. Small fluid obstructions as vortices within the anastomosis were detected by flow pathlines from 4D-flow MRI. In two patients pressure differences were calculated also by computational flow dynamics (CFD) as a plausibility check for the order of magnitude. Results: Median values of pressure differences in the anastomosis between SVC and RPA were 0.63 (0.21-2.1) mmHg, between C and RPA 0.67 (0.3-2.2) mmHg, between SVC and LPA 0.8 (0.3-2.4) mmHg and between C and LPA 0.7 (0.2-1.9) mmHg. Patients with potential flow obstruction (stents, occluder, vortices) had significantly higher gradients at the anastomosis (p < 0.05) than patients without potential obstructions, although the absolute values were small. CFD- and measurement-based pressure difference showed good agreement. Conclusion: 4D-flow MRI is able to detect minor pressure differences within the Fontan circuit even in patients with apparently satisfactory TCPC. Slightly higher pressure differences are due to the presence of small flow obstruction. [ABSTRACT FROM AUTHOR]

Systemic-to-pulmonary shunt malfunction contributes to morbidity in children with complex congenital heart disease after palliative procedure. Neointimal hyperplasia might play a role in the pathogenesis increasing risk for shunt obstruction. The aim was to evaluate the role of epidermal growth factor receptor (EGFR) and matrix-metalloproteinase 9 (MMP-9) in the formation of neointimal within shunts. Immunohistochemistry was performed with anti-EGFR and anti-MMP-9 on shunts removed at follow-up palliative or corrective procedure. Whole-genome single-nucleotide polymorphisms genotyping was performed on DNA extracted from patients´ blood samples and allele frequencies were compared between the group of patients with shunts displaying severe stenosis (≥ 40% of lumen) and the remaining group. Immunohistochemistry detected EGFR and MMP-9 in 24 of 31 shunts, located mainly in the luminal area. Cross-sectional area of EGFR and MMP-9 measured in median 0.19 mm2 (IQR 0.1–0.3 mm2) and 0.04 mm2 (IQR 0.03–0.09 mm2), respectively, and correlated positively with the area of neointimal measured on histology (r = 0.729, p < 0.001 and r = 0.0479, p = 0.018, respectively). There was a trend of inverse correlation between the dose of acetylsalicylic acid and the degree of EGFR, but not MMP-9, expression within neointima. Certain alleles in epidermal growth factor (EGF) and tissue inhibitor of metalloproteinases 1 (TIMP-1) were associated with increased stenosis and neointimal hyperplasia within shunts. EGFR and MMP-9 contribute to neointimal proliferation in SP shunts of children with complex cyanotic heart disease. SP shunts from patients carrying certain risk alleles in the genes encoding for EGF and TIMP-1 displayed increased neointima. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES This study aimed to prove if pulmonary artery (PA) size influences survival and if an additional aortopulmonary shunt (APS) promotes left PA growth after bidirectional cavopulmonary shunt (BCPS) in patients with hypoplastic left heart syndrome. METHODS The medical records of patients with hypoplastic left heart syndrome who underwent Norwood procedure and BCPS between 2007 and 2020 were reviewed. Right, left and total (right + left) PA indices were calculated according to Nakata and colleagues. RESULTS A total of 158 patients were included in this study. The median age at Norwood and BCPS was 8 (7–11) days and 3.6 (3.1–4.6) months, respectively. There were 7 hospital deaths and 12 late deaths. Survival after BCPS was 90.3% at 1 year and 86.2% at 2 years. Total, right and left PA indices were 238 (195–316), 136 (101–185) and 102 (75–130) mm2/m2 at the time of BCPS, and they were 237 (198–284), 151 (123–186) and 86 (69–108) mm2/m2 at the time of Fontan. Left PA index decreased significantly between the time of BCPS and Fontan (P  < 0.01). Nine patients needed partial takedown and additional APS due to failing BCPS, but the additional APS did not promote the PA growth significantly. CONCLUSIONS Preoperative PA index did not affect the mortality after BCPS. The partial takedown and additional APS for failing BCPS were unable to improve left PA size. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). METHODS In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. RESULTS The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8–3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P  = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P  = 0.007, 20 years: P  = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P  < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P  = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P  < 0.001). CONCLUSIONS There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV. [ABSTRACT FROM AUTHOR]

Background: About 5–10% of adults with congenital heart disease (ACHD) will develop pulmonary arterial hypertension (PAH), which is associated with significant mortality. Studies on risk factors for poor outcome in a contemporary cohort of these patients with PAH associated with CHD (PAH-CHD) are rare. Methods: In this retrospective, single-center study, adult patients with the diagnosis PAH-CHD who had at least one contact as an outpatient or inpatient at the German Heart Centre Munich during the period January 2010–September 2019 were included. Patients with PAH without a CHD were excluded. The primary endpoint was all-cause mortality. Results: Altogether, 158 patients (mean age 39.9 ± 15.4 years, female 64.6%) were included in the study. A pre-tricuspid shunt was present in 17.7%, other shunts in 51.3%, PAH associated with complex CHD in 22.8%, and segmental PAH in 8.2%. An NT-proBNP measurement at baseline was available in 95 patients (60.1%). During a median follow-up of 5.37 years [IQR 1.76–8.63], the primary endpoint occurred in 10 patients (6.7%). On univariate analysis, CRP (log) (HR 3.35, 95% CI (1.07–10.48), p = 0.037), NT-proBNP (log) (HR: 7.10, 95% CI: 1.57–32.23, p = 0.011), and uric acid (HR: 1.37, 95% CI: 1.05–1.79, p = 0.020) were predictors of the primary endpoint. On multivariate analysis, only NT-proBNP (log) (HR: 6.91, 95% CI: 1.36–35.02, p = 0.0196) remained as an independent predictor. Conclusion: NT-proBNP is an independent predictor of all-cause mortality in a contemporary cohort of PAH-CHD patients. The role of CRP and uric acid should be further assessed in future studies. [ABSTRACT FROM AUTHOR]

The progress in fetal cardiology allows for the early diagnosis of congenital heart defects, but there is still a lack of data on the psychological situation of parents expecting a child with a congenital heart defect. In this cross-sectional study, 77 parents (45 women and 32 men) expecting a child with a heart defect were interviewed with different questionnaires. The standardized Hospital Anxiety and Depression Scale (HADS) questionnaire was used to assess the psychological state of the parents. Various statistical procedures were performed to determine the prevalence, risk factors, and predictors of anxiety and depression. The prevalence for prenatal anxiety was 11.8% and for depressed mood 6.6%, whereas the postnatal prevalence was 25% for anxiety and 16.7% for depressed mood. The mother is influential in protecting against depression as a contact person (p = 0.035). Women were more affected by anxiety and depression than men (p = 0.036). A significant and positive correlation was observed between anxiety and depression before birth (ρ = 0.649, p < 0.001) and after birth (ρ = 0.808, p < 0.001). The level of education correlated negatively with depression (p = 0.016) and anxiety (p = 0.017) before birth. Significantly higher anxiety and depression scores were not observed among health and social workers (p = 0.084), first-time mothers (p = 0.190), and parents whose pregnancies were due to medical assistance (p = 0.051). Close collaboration between maternal-fetal care units, pediatric cardiologists and psychiatric/psychosomatic disciplines is a possible strategy to reduce stress in parents. Therefore, an expert team of professionals, educating with understandable terms and sufficient knowledge about fetal heart disease in parenting counseling, is required. The support of affected parents can positively impact the treatment of the child and should be integrated into the daily routine of the clinic. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Studies focused on infants with univentricular heart undergoing stage I palliation other than the Norwood procedure remain a topic of great interest. This study evaluated the outcome of infants who underwent systemic to pulmonary shunt (SPS) or pulmonary artery banding (PAB). METHODS Infants who underwent SPS or PAB as stage I palliation between 1994 and 2019 were included. Survival and late systemic ventricular function were evaluated. RESULTS Out of 242 patients, 188 underwent SPS (77.7%) and 54 PAB (22.3%). Main diagnosis included tricuspid atresia, unbalanced atrioventricular septal defects, double inlet left ventricles and single ventricles with other morphology. Thirty-eight patients died before stage II palliation (15.7%). Stage II palliation was performed in 182 patients (75.2%), and mortality between stages II and III was 11 (5.6%). Stage III palliation was performed in 160 (66.1%) patients. Survival at 1, 5 and 15 years after stage I procedure was 81.9, 77.1 and 76.2%, respectively, and similar between both procedures (P  = 0.97). Premature birth [ P  = 0.03, hazard ratio (HR) = 2.1], heterotaxy (P  = 0.006, HR = 2.4) and dominant right ventricle (P  = 0.015, HR = 2.2) were factors associated to mortality. Unbalanced atrioventricular septal defect (P  = 0.005, HR = 4.6) was a factor associated to systemic ventricular dysfunction. CONCLUSIONS In patients with univentricular heart who underwent SPS and PAB as stage I palliation, survival at 15 years was 76%, regardless of th chosen approach. Premature birth, heterotaxy and dominant right ventricle were associated to mortality. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Neointimal hyperplasia might affect systemic-to-pulmonary shunt failure in infants with complex cyanotic congenital heart disease. The aim of this study was to elucidate histopathologic changes in polytetrafluoroethylene shunts and to determine whether increased neointimal formation is associated with early interventions comprising balloon dilatation, stent implantation and shunt revision. Furthermore, we intended to identify clinical factors associated with increased neointimal proliferation. METHODS Removed shunts were processed for histopathological analysis. Slides were stained with hematoxylin/eosin and Richardson. Immunohistochemistry was performed with anti-alpha-smooth muscle actin and anti-CD68. Non-parametric analysis and univariable regressions were performed to identify clinical factors associated with neointimal hyperplasia and shunt stenosis. RESULTS Fifty-seven shunts (39 modified Blalock–Taussig anastomosis, 8 right ventricle-to-pulmonary artery anastomosis, 10 central shunts) were analysed. Area of neointimal proliferation within the shunt was in median 0.75 mm2 (interquartile range, 0.3–1.57 mm2) and relative shunt stenosis in median 16.7% (interquartile range, 6.7–30.8%). Neointimal hyperplasia and shunt stenosis correlated with each other and were significantly greater in the group that required early interventions and shunt revision. Univariable linear regression identified smaller shunt size and lower acetylsalicylic acid dosage as factors to be associated with greater neointimal proliferation and shunt stenosis. CONCLUSIONS In infants with complex cyanotic congenital heart disease, neointimal hyperplasia in systemic-to-pulmonary shunts is associated with early interventions comprising balloon dilatation, stent implantation and shunt revision. Smaller shunt size and lower aspirin dosage are associated with increased neointimal proliferation. [ABSTRACT FROM AUTHOR]

Background: Due to various reasons explored in previous studies, adults with congenital heart disease (ACHD) are at risk of developing post-traumatic stress symptoms (PTSS). The aim of this study is to explore multiple potential psychosocial protective factors in ACHD and to understand their role in different complexities of congenital heart disease (CHD) and PTSS.This study was part of the “ABS-AHF” study, where 234 ACHD were recruited from November 2021 to August 2022 at a tertiary CHD care center. Data were collected on PTSS (PDS), resilience (RS-13), sense of coherence (SOC-L9), and social support (F-SozU K-14).The mean scores were 70.55 + / − 12.31 [21–91] for resilience, 35.83 + / − 4.81 [15–60] for sense of coherence (SOC), and 4.30 + / − 0.79 [0.93–5] for social support. SOC (OR, .91; p = .024 [.84; 9.98]) and social support (OR, .48; p = .001 [.29; 7.96]) were shown to reduce the likelihood of PTSS. Low resilience (OR, 2.40, p = .0248 [1.18; 5.18]) seems to increase this likelihood.Integrating parents and relatives early on seems to be an important protective resource. Parental support in childhood affects the development of SOC which is in line with social support related to lower PTSS. With regard to resilience and SOC, a brief and manageable screening option for personal resources is available to refer potentially vulnerable patients to specialized psychosocial care. Care offers should address coping styles and life with CHD. Offering multidisciplinary care, integrating the patient’s social network, and education for patients to increase resilience and SOC might provide a way to enhance psychosocial outcomes, quality of life, and adherence in ACHD.Method: Due to various reasons explored in previous studies, adults with congenital heart disease (ACHD) are at risk of developing post-traumatic stress symptoms (PTSS). The aim of this study is to explore multiple potential psychosocial protective factors in ACHD and to understand their role in different complexities of congenital heart disease (CHD) and PTSS.This study was part of the “ABS-AHF” study, where 234 ACHD were recruited from November 2021 to August 2022 at a tertiary CHD care center. Data were collected on PTSS (PDS), resilience (RS-13), sense of coherence (SOC-L9), and social support (F-SozU K-14).The mean scores were 70.55 + / − 12.31 [21–91] for resilience, 35.83 + / − 4.81 [15–60] for sense of coherence (SOC), and 4.30 + / − 0.79 [0.93–5] for social support. SOC (OR, .91; p = .024 [.84; 9.98]) and social support (OR, .48; p = .001 [.29; 7.96]) were shown to reduce the likelihood of PTSS. Low resilience (OR, 2.40, p = .0248 [1.18; 5.18]) seems to increase this likelihood.Integrating parents and relatives early on seems to be an important protective resource. Parental support in childhood affects the development of SOC which is in line with social support related to lower PTSS. With regard to resilience and SOC, a brief and manageable screening option for personal resources is available to refer potentially vulnerable patients to specialized psychosocial care. Care offers should address coping styles and life with CHD. Offering multidisciplinary care, integrating the patient’s social network, and education for patients to increase resilience and SOC might provide a way to enhance psychosocial outcomes, quality of life, and adherence in ACHD.Results: Due to various reasons explored in previous studies, adults with congenital heart disease (ACHD) are at risk of developing post-traumatic stress symptoms (PTSS). The aim of this study is to explore multiple potential psychosocial protective factors in ACHD and to understand their role in different complexities of congenital heart disease (CHD) and PTSS.This study was part of the “ABS-AHF” study, where 234 ACHD were recruited from November 2021 to August 2022 at a tertiary CHD care center. Data were collected on PTSS (PDS), resilience (RS-13), sense of coherence (SOC-L9), and social support (F-SozU K-14).The mean scores were 70.55 + / − 12.31 [21–91] for resilience, 35.83 + / − 4.81 [15–60] for sense of coherence (SOC), and 4.30 + / − 0.79 [0.93–5] for social support. SOC (OR, .91; p = .024 [.84; 9.98]) and social support (OR, .48; p = .001 [.29; 7.96]) were shown to reduce the likelihood of PTSS. Low resilience (OR, 2.40, p = .0248 [1.18; 5.18]) seems to increase this likelihood.Integrating parents and relatives early on seems to be an important protective resource. Parental support in childhood affects the development of SOC which is in line with social support related to lower PTSS. With regard to resilience and SOC, a brief and manageable screening option for personal resources is available to refer potentially vulnerable patients to specialized psychosocial care. Care offers should address coping styles and life with CHD. Offering multidisciplinary care, integrating the patient’s social network, and education for patients to increase resilience and SOC might provide a way to enhance psychosocial outcomes, quality of life, and adherence in ACHD.Conclusion: Due to various reasons explored in previous studies, adults with congenital heart disease (ACHD) are at risk of developing post-traumatic stress symptoms (PTSS). The aim of this study is to explore multiple potential psychosocial protective factors in ACHD and to understand their role in different complexities of congenital heart disease (CHD) and PTSS.This study was part of the “ABS-AHF” study, where 234 ACHD were recruited from November 2021 to August 2022 at a tertiary CHD care center. Data were collected on PTSS (PDS), resilience (RS-13), sense of coherence (SOC-L9), and social support (F-SozU K-14).The mean scores were 70.55 + / − 12.31 [21–91] for resilience, 35.83 + / − 4.81 [15–60] for sense of coherence (SOC), and 4.30 + / − 0.79 [0.93–5] for social support. SOC (OR, .91; p = .024 [.84; 9.98]) and social support (OR, .48; p = .001 [.29; 7.96]) were shown to reduce the likelihood of PTSS. Low resilience (OR, 2.40, p = .0248 [1.18; 5.18]) seems to increase this likelihood.Integrating parents and relatives early on seems to be an important protective resource. Parental support in childhood affects the development of SOC which is in line with social support related to lower PTSS. With regard to resilience and SOC, a brief and manageable screening option for personal resources is available to refer potentially vulnerable patients to specialized psychosocial care. Care offers should address coping styles and life with CHD. Offering multidisciplinary care, integrating the patient’s social network, and education for patients to increase resilience and SOC might provide a way to enhance psychosocial outcomes, quality of life, and adherence in ACHD. [ABSTRACT FROM AUTHOR]

To evaluate the prevalence of veno-venous collaterals (VVCs) after total cavopulmonary connection (TCPC) and analyze their impact on outcomes. Patients undergoing TCPC between 1994 and 2022 were evaluated. VVCs were identified using angiograms of cardiac catheterizations and their impact on outcomes was analyzed. A total of 635 patients were included. Median age at TCPC was 2.3 (interquartile ranges (IQR): 1.8–3.3) years. The most frequent diagnosis was hypoplastic left heart syndrome in 173 (27.2%) patients. Prior bidirectional cavopulmonary shunt was performed in 586 (92.3%) patients at a median age of 5.3 (3.6–9.9) months. VVCs were found in 94 (14.8%) patients at a median of 2.8 (0.1–11.8) years postoperatively. The prevalence of VVCs was similar between the dominant right and left ventricle (14.7 vs. 14.9%, p = 0.967). Mean pulmonary artery pressure (16.2 vs. 16.0 mmHg, p = 0.902), left atrial pressure (5.5 vs. 5.7 mmHg, p = 0.480), transpulmonary gradient (4.0 vs. 3.8 mmHg, p = 0.554) and oxygen saturation (81.4 vs. 82.6%, p = 0.103) before TCPC were similar between patients with and without VVCs. The development of VVCs did not affect survival after TCPC (p = 0.161). Nevertheless, VVCs were a risk for the development of plastic bronchitis (PB, p < 0.001). Interventional closure of VVCs was performed in 60 (9.4%) patients at a median of 8.9 (0.6–15.1) years after TCPC, and improvement of oxygen saturation was observed in 66% of the patients. The prevalence of VVCs after TCPC was 15%. VVCs had no impact on survival following TCPC but were associated with a high prevalence of PB. [Display omitted] [ABSTRACT FROM AUTHOR]

Objective: Late-onset small-for-gestational-age (SGA) fetuses usually show normal uterine artery Doppler and were long considered to have a good peri- and postnatal outcome. Recently, these fetuses were identified to have a risk factor for cardiovascular disease. The aim of our study was to evaluate former SGA children concerning their cardiovascular risk and nutrition behavior at the age of 1 year. Methods: We performed a prospective longitudinal cohort study at the University Hospital "Klinikum rechts der Isar" of the Technical University of Munich. Singleton pregnancies from 32 weeks with suspicion of SGA and healthy control pregnancies were included. Results: A total of 100 former SGA children and 113 controls with normal weight (AGA) were examined at 1 year of age. Drop-out for 1-year follow-up was 27%. SGA children had significantly higher systolic (92.8 ± 9.8 mmHg vs. 87.5 ± 10.7 mmHg, p = 0.001), diastolic (63.1 ± 8.5 mmHg vs. 60.0 ± 10.3 mmHg, p = 0.028) and mean (73.0 ± 7.8 vs. 69.2 ± 9.7 mmHg, p = 0.004) blood pressure than AGA children. Comparing two breastfeeding periods (0–4 months vs. > 7 months), a downward trend in blood pressure values for longer breastfeeding periods was shown. Conclusion: Our study showed that even late-onset small-for-gestational-age fetuses seem to have cardiovascular problems, although they were previously thought to be "healthy". Up to now, blood pressure measurement is not part of indicated health checks in former SGA or even fetal growth-restricted children which should be changed. Further studies are needed to investigate cardiovascular prevention programs in children. [ABSTRACT FROM AUTHOR]

Background: Arrhythmias are a well known complication in patients with single ventricle physiology (SVP). However, there is still a lack of data regarding arrhythmias in older patients. The aim of this study was to analyze arrhythmia type and frequency, treatment and recurrence rates in patients with SVP over the age of 40 years. Methods: Data was obtained retrospectively from clinical records. All patients > 40 years with SVP with arrhythmias between 2005 and 2018 were included in the study. Treatment was classified as medical, interventional (electrophysiological studies (EPS) in combination with catheter ablation) or direct current cardioversion (DCCV). Results: Altogether, 29 patients (11 female; mean 47.5 ± 4.6 years) with 85 arrhythmia episodes were identified. The median follow-up time was 6.3 years. Cavo-tricuspid (CTI) and non-CTI related intra-atrial reentrant tachycardia (IART) and atrial fibrillation (AF) were most common (48.2% and 37.6%, respectively). In total, 18 EPS/ablations were performed in 9 patients and 52 DCCVs in 20 patients. Acute success was 98% for DCCV and 72.2% for EPS/ablation. Recurrence rate was high (70% for DCCV and 55% for EPS). AT recurrences occurred after a median of 8 and 2.5 months, respectively. On multivariate analyses, age was the only risk factor for arrhythmia recurrence (HR 0.58, 95% C.I. 0.43–0.78, p < 0.0001). Pacemaker implantation was necessary in seven patients (AV block n = 4, sinus node dysfunction n = 3) and one patient received an ICD for secondary prophylaxis. Sudden death occurred in three patients. Conclusions: The most common arrhythmias in patients with SVP > 40 years are IART and AF. Arrhythmia recurrence following EPS or DCCV is frequent. Older age is an independent risk factor for arrhythmia recurrence. [ABSTRACT FROM AUTHOR]

Background: COVID-19 might pose a risk for adults with congenital heart disease (ACHD). However, data regarding the rate of infection as well as myocardial involvement in ACHD patients are currently lacking. Methods: During the study period from January to June 2021, all consecutive outpatients from our ACHD clinic were eligible to participate. Clinical data were collected. An antibody test for COVID-19 was performed in all patients. Cardiovascular magnetic resonance imaging (CMR) was offered to those with a positive antibody test. Results: Overall, 420 patients (44.8% female, mean age 36.4 ± 11.6 years) participated. Congenital heart defect (CHD) complexity was simple in 96 (22.9%), moderate in 186 (44.3%), complex in 117 (27.9%), and miscellaneous in 21 (5.0%) patients. Altogether, 28 (6.7%) patients had a positive antibody test. Out of these, 14 had an asymptomatic course. The others had mainly mild symptoms and were managed as outpatients. Furthermore, 11 patients (39.3%) had even not been aware of their infection. Fourteen patients underwent a CMR without signs of myocardial involvement in any of them. Conclusions: We observed a number of undetected cases of COVID-19 infections in our ACHD population. Reassuringly, in all cases, the infection had a mild clinical course. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES We sought to identify the prevalence of surgical reintervention on the neo-aorta after Norwood procedure and its impact on long-term outcomes. METHODS We reviewed the medical records of all patients who underwent Norwood procedure. The impacts of surgical neoaortic reintervention on outcomes were analysed in each stage of palliation. RESULTS A total of 335 patients were included in this study. Thirty patients underwent surgical reintervention on the neo-aorta after Norwood procedure. The timing of initial reintervention was before stage II in 13 patients, at stage II in 7, between stage II and stage III in 5, at stage III in 3 and after stage III in 2. A reintervention before stage II was significantly associated with mortality (HR 14.4, 95% confidence interval 6.00–34.6, P < 0.001). In patients who underwent stage II (n = 251), reintervention had no significant impact on mortality. In patients who underwent stage III (n = 188), the previous reintervention was significantly associated with higher mean pulmonary pressure (P = 0.05) and a higher rate of reduced ventricular function (P = 0.002). Greater than mild atrioventricular valve regurgitation was significantly associated with the development of a neoaortic arch stenosis after stage II (P = 0.03). CONCLUSIONS Surgical reinterventions on the neo-aorta were required in each inter-stage phase. A surgical neoaortic reintervention was not related to increased mortality after stage II but significantly associated with a higher rate of reduced ventricular function and elevated mean pulmonary artery pressure. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES We sought to investigate the impact of early postoperative low arterial oxygen saturation on mortality and morbidity after bidirectional cavopulmonary shunt (BCPS). METHODS The medical records of all patients who underwent BCPS between 2013 and 2018 were reviewed. RESULTS A total of 164 patients were included in this study. Forty-seven patients underwent reintervention during hospital stay at median 7 days after BCPS. Before reintervention, 30 patients were intubated or had SpO2 of <75%. All re-interventions for Glenn pathway obstruction and 4 out of 5 venovenous coil embolization resulted in hospital discharge, while high mortality was observed after other re-interventions (atrioventricular valve surgery, thrombolysis, systemic ventricular outflow obstruction relief, extracorporeal membrane oxygenation implantation and diaphragmatic plication). Additional aortopulmonary shunt with pulmonary artery discontinuation was performed in 8 patients who showed severe cyanosis with median SpO2 of 59% under maximal ventilation support. In the univariable Cox regression analysis, the associated factors for mortality before total cavopulmonary connection were reduced ventricular function [hazard ratio (HR) 6.89, 95% confidence interval (CI) 1.76–26.9, P -value 0.006], greater than moderate atrioventricular valve regurgitation (HR 5.89, 95% CI 1.70–20.4, P -value 0.005), SpO2 1 h after extubation (HR 0.87, 95% CI 0.80–0.96, P -value 0.004) and mean pulmonary artery pressure 1 h after extubation (HR 1.14, 95% CI 1.02–1.26, P -value 0.016). CONCLUSIONS After BCPS, unacceptable cyanosis persisted with various aetiologies. Low arterial oxygen saturation within 1 h after extubation is significantly associated with high mortality after BCPS. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Despite improvements in the surgical management of the hypoplastic left heart syndrome and its variant, the Norwood procedure is still associated with substantial mortality and morbidity and extracorporeal membrane oxygenation support is required in some patients. METHODS We reviewed patients with the Norwood procedure between 2007 and 2019. The primary end point of the study was mortality during extracorporeal membrane oxygenation. Secondary end points included morbidity, bidirectional cavopulmonary shunt and Fontan completion. RESULTS Of the 257 patients in whom the Norwood procedure was performed, mechanical support was required in 41 patients (16%). Indications for extracorporeal membrane oxygenation were low cardiac output (n = 16, 39%), hypoxaemia (n = 12, 29%) and inability to wean from cardiopulmonary bypass (n = 9, 22%). The median age at extracorporeal membrane oxygenation was 10.9 days (interquartile range, 7.9–21.2) and veno-arterial support was required in 37 patients (90.2%). Weaning from extracorporeal membrane oxygenation was achieved in 61% (n = 25). Survival to hospital discharge and 1-year survival was 34.6% (standard deviation: 17.1) and 25.7% (standard deviation: 7), respectively. Bidirectional cavopulmonary shunt was performed in 24% (n = 10) and Fontan completion in 7% (n = 3). Preoperative moderate or greater atrioventricular valve regurgitation was independently associated with mechanical support. Implantation of extracorporeal membrane oxygenation in the paediatric catheter laboratory was identified as an independent risk factor for mortality. CONCLUSIONS Moderate or greater atrioventricular valve regurgitation is an independent risk factor for mechanical support after the Norwood procedure. Mechanical support is associated with substantial in-hospital mortality; however, successful Fontan completion was accomplished in some patients. [ABSTRACT FROM AUTHOR]

Objective To assess feasibility, safety and effectiveness of right ventricular outflow tract (RVOT) stenting in symptomatic young infants. Methods Multicentre evaluation of 35 patients intended to undergo RVOT stenting in 11 pediatric cardiac centres from 2009 to August 2011. Results Median age and weight at the time of first stent implantation were 8 weeks and 3.3 kg, with 40% of patients <3 kg. A total of 19 patients had suffered from hypoxemic spells, 8 patients were ventilated, 6 on inotropic support and 5 on prostaglandin infusion. Severe concomitant malformations were present in 11 patients, and acute infections in 2. Stenting of the RVOT was successfully performed in 33 patients, improving oxygen saturation from a median of 77 to 90% 2 days after intervention. Besides the 2 patients in whom RVOT stenting was not successful for technical reasons, there were no procedural complications. In 17 of 33 patients, 1-3 reinterventions were performed during follow-up, less than half of those were reinterventions in the RVOT. A total of 27 patients have undergone successful surgical repair 4-162 (median 19.5) weeks after initial RVOT stent implantation, 2 patients are still waiting. There were no perioperative deaths. Conclusions Stenting of the RVOT provides a safe and effective management strategy for initial palliation in symptomatic young infants, including those patients not suitable or at higher risk for surgical therapy. (J Interven Cardiol 2015;28:279-287) [ABSTRACT FROM AUTHOR]

Propofol is a short-acting, intravenously administered hypnotic agent which is used in procedural sedation in children. Propofol is known to decrease systemic vascular resistance, arterial blood pressure and can lead to desaturations and decreased systemic perfusion in children with cardiac shunting. This may result in a reduction in cerebral blood flow and oxygenation. Near-infrared spectroscopy (NIRS) can monitor cerebral tissue oxygenation in the frontal neocortex. The objective of our study was to measure the changes in cerebral oxygen and blood supply after Propofol infusion in children with congenital heart disease. Propofol infusion may reduce cerebral oxygenation in children with congenital heart disease. The study group consisted of 32 children (f:m = 18:14), with median age of 49 (5-112) months and median weight of 15 (5-34) kg. We performed NIRS derived continuous measurement of cerebral oxygenation and cardiac output using Electrical velocimetry for 5 min before and after sedation with Propofol (1-2 mg/kg i.v.) for cardiac catheterization. Simultaneously, non-invasive arterial blood pressure and transcutaneous oxygen saturation were measured. Propofol sedation led to a significant decrease in mean arterial pressure (79 ± 16 vs. 67 ± 12 mmHg) ( p = 0.01) and cardiac index (3.2 ± 0.8 vs. 2.9 ± 0.6 ml/min/m) ( p = 0.03). In contrast, cerebral tissue oxygenation index, increased significantly from 57 ± 11 to 59 ± 10 % ( p < 0.05). Sedation with Propofol increased cerebral tissue oxygenation despite a decrease in cardiac index and arterial blood pressure. This may be caused by a decreased oxygen consumption of the sedated brain with intact cerebral auto-regulation. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES This study was performed to determine the clinical and haemodynamic variables associated with early adverse outcomes after the neonatal Norwood procedure. METHODS Patients who underwent the neonatal Norwood procedure between 2001 and 2019 were included. The patient diagnosis, morphological characteristics and haemodynamic parameters were analysed to identify factors associated with length of stay (LOS) in the intensive care unit (ICU) and mortality during the stay. RESULTS A total of 322 patients were depicted. The median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Certain morphological and preoperative parameters, such as birth weight below 2.5 kg, restrictive atrial septal defect, extracardiac anomalies and the diameter of the ascending aorta, were found to be associated with the LOS in the ICU. Analysis using early postoperative haemodynamic variables revealed that systolic arterial pressure, diastolic arterial pressure, serum lactate levels and reduced ventricular function at 2 days postoperatively were associated with the LOS in the ICU. Birth weight <2.5 kg (P  = 0.010), a restrictive atrial septal defect (P  = 0.001) and smaller ascending aorta (P  = 0.039) were associated with death in the ICU. Reduced ventricular function, lower systolic aortic pressure and higher lactate levels at various time points (P  < 0.05) were also associated with ICU deaths. The LOS in the ICU was significantly associated with late mortality (P  < 0.001, Hazard Ratio (HR) = 1.015). CONCLUSIONS The LOS in the ICU after the Norwood procedure was predicted by early postoperative haemodynamic variables, suggesting that good early postoperative haemodynamics determine early recovery. A prolonged stay in the ICU after the Norwood procedure was associated with late mortality. [ABSTRACT FROM AUTHOR]

Despite advances in surgical techniques, right ventricular outflow tract (RVOT) conduits are prone to fail over time. Percutaneous pulmonary valve implantation was introduced to expand the lifetime of these conduits and to decrease the number of open heart operations during a patient's lifetime. The procedure can be performed with excellent results; however, serious complications such as coronary arterial compression and conduit rupture have been reported. We present percutaneous treatment of a patient after Ross-Konno operation with RVOT conduit dysfunction and a potentially problematic course of the left anterior descending artery. [ABSTRACT FROM AUTHOR]

Objective Health-related quality of life questionnaires are frequently used to involve patients' impressions and feelings in the outcome evaluation. In patients with congenital heart disease (CHD) methodological heterogeneities, assessment in different age and diagnostic groups led to controversial findings. This study aims to give a comprehensive answer to the health-related quality of life in patients with CHD. Patients and methods From July 2001 to June 2013, 2360 patients (1058 female, 28.6±11.2-years, range 14-75-years) with various kinds of CHD underwent a quality of life assessment with the SF-36 questionnaire and underwent a cardiopulmonary exercise test as part of their routine follow-up. Results Physical component summary score (PCS) develops from 96.1 %predicted in patients younger than 20-years, to 96.2 %predicted in patients aged 20 to 30-years, 92.3 %predicted in patients aged 30 to 40-years, and 92.6 %predicted in patients 40-years or older (r=-.114; p<.001). The decline was more prominent in the mental component summary score (MCS) declining from 104.1 %predicted in patients younger than 20years, to 103.4 %predicted, 99.9 %predicted, and 97.5 %predicted (r=-.132; p<.001). Exercise capacity was impaired with 80.1±23.0 %predicted and also declined slowly with age (r=-.084; p<.001). Conclusions Health-related quality of life in patients with CHD is progressively reduced in older age-groups. The decline is small, but more prominent in the mental components. This should be considered in the interpretation of studies, and strategies have to be developed to reduce this effect in future. [ABSTRACT FROM AUTHOR]

To assess the impact of low birth weight on early and late outcomes after staged palliation for single ventricle. Patients after stage 1 palliation for single ventricle in our institution were retrospectively included and divided into two weight groups: 2.5 kg or less (low birth weight) and more than 2.5 kg. The impact of low birth weight on mortality and on the progression to further palliation stages (bidirectional Glenn, stage 2, and total cavopulmonary connection, stage 3) was assessed. A total of 452 patients were included. Patients with low birth weight (n = 37, 8 %) had more frequently associated prematurity and extracardiac anomalies. Early and inter-stage mortality after stage 1 was higher in patients with low birth weight, so that less of these patients reached the next palliation stage (57 % vs. 77 %, p = 0.01, and 38 % vs. 56 %, p = 0.05, for stage 2 and stage 3, respectively). After 5 years, overall survival was inferior in patients with low birth weight (48 % vs. 73 %, p < 0.001). Survival conditioned by stage 2 palliation was inferior in patients with low birth weight compared to the reference group (76 % vs. 89 % after 5 years, p = 0.04). Low birth weight was a risk factor for death in most patients' subgroups, inclusive those with restricted pulmonary blood flow after a systemic-to-pulmonary shunt procedure. During staged palliation of single-ventricle physiology, low birth weight has a detrimental impact on survival extending to beyond stage 2. This study calls for increased vigilance of these patients beyond the first interstage. [Display omitted] • Single-ventricle patients have a higher mortality if born with less than 2500 g. • Their chances of reaching the next palliation stages are decreased. • Hypoplastic left heart syndrome is not exclusively impacted. [ABSTRACT FROM AUTHOR]

In congenital aortic valve disease, quantifying aortic regurgitation (AR) varies by the measurement site. Our study aimed to identify the optimal site for AR assessment using 2D and 4D MR flow measurements, with a focus on vortices. We retrospectively analysed 31 patients with congenital aortic valve disease, performing 2D and 4D MR flow measurements at the aortic valve, sinotubular junction (STJ), ascending aorta (AAo), and using midpulmonary artery measurements as a reference. We assessed percentage AR and net forward volumes, calculated linear correlations, and plotted Bland-Altman plots. Net forward flow at all aortic sites were correlated with the main pulmonary artery. Differences in AR between 2D and 4D flows were linked to vortices detected by 4D streamlines. The best agreement in % AR between 2D and 4D flows was at the aortic valve (mean difference 4D 2D -2.9%, limits of agreement 8.7% to −14.3%; r2 = 0.7). Correlations weakened at STJ and AAo. Vortices in the ascending aorta led to AR overestimation in 2D measurements. Net forward flow at the aortic valve by 4D flow correlated closer with main pulmonary artery than did 2D flow. (Mean difference for 2D and 4D MR flow 7.5 ml and 4.2 ml, respectively). F or congenital aortic valve disease, the most accurate AR quantification occurs at the aortic valve using 2D and 4D MR flow. Notably, vortices in the ascending aorta can result in AR overestimation with 2D MR flow. • Measuring aortic regurgitation (AR) by 2D and 4D MR flow agrees best at the aortic valve. • Vortices in the ascending aorta lead to overestimation of regurgitant volumes by 2D MR • Aortic net forward flow in AR by 4D MR flow is more accurate than by 2D forward flow [ABSTRACT FROM AUTHOR]

Children with univentricular hearts (UVH) undergo up to three palliative surgical procedures to achieve complete circulatory separation (Fontan circulation). As a marker of cardiac wall stress, NT-proBNP is a promising tool to assess systemic ventricular load in these patients. However, different reference intervals (RI) apply to each stage, as NT-proBNP is highly age-dependent. Children undergoing systemic-to-pulmonary (SP) shunt placement (stage 1), bidirectional cavopulmonary shunt (BCPS, stage 2) or total cavopulmonary connection (TCPC, stage 3) between 2011 and 2021 with NT-proBNP measurement within 7 days before surgery were included. Furthermore, outpatients after TCPC with NT-proBNP measurement were enrolled. Biomarker levels were evaluated using its age-adjusted z-score ("zlog-NT-proBNP"; age-independent RI, −1.96 to +1.96), allowing comparison between different stages and revealing changes in systemic ventricular load independent of the marked physiological decline in RI with age. Overall, 289 children (227 before, 62 after TCPC) met the eligibility criteria. Median time between blood sampling and surgery (SP shunt/BCPS/TCPC) was 2 [1–3] days and 3.2 [2.0–4.5] years after TCPC. Age-adjusted zlog-NT-proBNP levels were 3.47 [2.79–3.93] in children with native UVH (before SP shunt), 3.10 [1.89–3.58] at stage 1 (before BCPS), 1.08 [0.51–1.88] at stage 2 (before TCPC), and 1.09 [0.72–1.75] at stage 3 (after TCPC/Fontan completion). Consequently, BCPS revealed the strongest decrease (median − 2.02 logarithmized standard deviations, p < 0.001). In children with UVH undergoing staged Fontan palliation, zlog-NT-proBNP is a highly promising tool for course assessment of systemic ventricular load, independent of the age-related decline in physiological NT-proBNP concentration. • Age-adjusted zlog-NT-proBNP helps to quantify ventricular stress in children with UVH. • Strongest decrease of zlog-NT-proBNP was observed with bidirectional cavopulmonary shunt. • Children after TCPC mostly reveal physiological zlog-NT-proBNP levels. [ABSTRACT FROM AUTHOR]

Objective: This study compared objectively measured and self-reported physical activity (PA) in adults with congenital heart disease (ACHD) to a healthy reference cohort (RC). Patients and Methods: From May 2017 to August 2020, 211 ACHD (39.9 ± 9.7 years, 101 female) and 141 healthy adults (35.9 ± 14.7 years, 76 female) participated in a wearable-based and self-reported PA assessment. Moderate-to-vigorous PA (MVPA) and the step count were recorded with the Garmin vivofit® 3 device for 7 consecutive days. Additionally, subjects were asked to report the number of days they are active for ≥30 min throughout the week. Results: Only 33 (17%) ACHD and 36 (26%) healthy controls (p = 0.030) accumulated the World Health Organization's (WHO) recommendation of 150 min MVPA per week. ACHD were less active per week (ACHD: 40.0 [0.0; 101.0] min. MVPA vs. RC: 75.0 [22.5; 152.5] min. MVPA, p = 0.002) and walked fewer daily steps (ACHD: 8,246 [6,505; 10,434] vs. RC: 9,413 [7,621; 11,654], p = 0.001) than healthy controls. Especially, patients with moderate (p = 0.030), complex (p < 0.001), or surgically corrected (p = 0.008) congenital heart disease accumulated significantly less MVPA than healthy peers throughout the week. A large majority of 72% of ACHD and 58% of the RC overestimated their weekly active days by more than one day. Conclusions: ACHD walked quite a few steps daily but lacked intensity. ACHD was less active than healthy controls and failed to reach international recommendations. They therefore need encouragement toward more intense movement to improve the exercise capacity and lower cardiovascular risk. Self-reported PA showed no agreement to the objectively measured PA. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES A common atrioventricular valve (CAVV) is considered to be a risk factor for early and late deaths in patients with functional single ventricle (FSV). CAVV surgery in patients with FSV is challenging and there is limited knowledge of the outcomes of CAVV repair with univentricular physiology. METHODS We reviewed all CAVV surgical procedures in patients with FSV who underwent univentricular palliation. End points of the study were survival after CAVV surgery and cumulative incidence of reoperation. RESULTS Between 1984 and 2019, 66 children with CAVV and FSV underwent single-ventricle palliation, of whom 45.5% (30/66) required CAVV surgery. Indication for surgery was moderate CAVV regurgitation in 40% (12/30) and severe CAVV regurgitation in 60% (18/30). CAVV repair was performed in 93.3% (28/30) and CAVV replacement in 6.7% (2/30). The median age and weight at surgery were 0.9 years (interquartile range 0.3–1.8) and 6.5 kg (interquartile range 3.9–8.7), respectively. Operative and late mortality were 23.3% and 8.7%, respectively. Survival and cumulative incidence of reoperation at 4 years after CAVV surgery were 68.9% [standard deviation (SD): 8.7] and 35.8% (SD: 9.1), respectively. Fontan completion was achieved in 60% (18/30). Survival at 4 years after birth was 69.7% (SD: 8.5) in 30 patients with CAVV surgery, whereas it was 83% (SD: 6.3) in 36 patients without CAVV surgery (P  = 0.32). CONCLUSIONS CAVV surgery in patients with FSV is associated with substantial mortality and a high incidence of reoperation. [ABSTRACT FROM AUTHOR]

Ventricular assist devices (VAD) are increasingly used in patients with end-stage heart failure due to acquired heart disease. Limited data exists on the use and outcome of this technology in children. All children (<18 years of age) with VAD support included in the German National Register for Congenital Heart Defects were identified and data on demographics, underlying cardiac defect, previous surgery, associated conditions, type of procedure, complications and outcome were collected. Overall, 64 patients (median age 2.1 years; 45.3% female) receiving a VAD between 1999 and 2015 at 8 German centres were included in the analysis. The underlying diagnosis was congenital heart disease (CHD) in 25 and cardiomyopathy in 39 children. The number of reported VAD implantations increased from 13 in the time period 2000–2004 to 27 implantations in the time period 2010–2014. During a median duration of VAD support of 54 days, 28.1% of patients experienced bleeding complications (6.3% intracerebral bleeding), 14.1% thrombotic (10.9% VAD thrombosis) and 23.4% thromboembolic complications (including cerebral infarction in 18.8% of patients). Children with cardiomyopathy were more likely to receive a cardiac transplantation (79.5% vs. 28.0%) compared to CHD patients. Survival of cardiomyopathy patients was significantly better compared to the CHD cohort (p < 0.0001). Multivariate Cox-proportional analysis revealed a diagnosis of CHD (hazard ratio [HR] 4.04, p = 0.001), age at VAD implantation (HR 1.09/year, p = 0.04) and the need for pre-VAD extracorporeal membrane oxygenation (ECMO) support (HR 3.23, p = 0.03) as independent predictors of mortality. The uptake of VAD therapy in children is increasing. Morbidity and mortality remain high, especially in patients with congenital heart disease and those requiring ECMO before VAD implantation. • The uptake of VAD therapy in children is increasing. • Morbidity and mortality remain high. • Congenital heart disease and ECMO before VAD implantation are risk factors. [ABSTRACT FROM AUTHOR]