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IntroductionThe objective of this study was to examine the impact of ventricular assist device support as a bridge to heart transplantation in children with end-stage heart failure. In light of the limited availability of donor organs, particularly in Europe, the number of children requiring ventricular assist device support is rising at an unavoidable rate.MethodsWe performed a retrospective cohort study of patients who underwent a single and primary pediatric heart transplantation. Patients were divided into two groups: with pre transplant ventricular assist device (VAD) support and without VAD support. The primary outcome was survival at the follow-up evaluation. The time point designated as “time 0” was defined as the time of heart transplantation. Secondary outcome was examined by mean of univariable and multivariable logistic regression, severity of cardiac disease based on ECMO-support pre VAD-support, mean waiting time for transplantation, mean OR time and mean length of hospital stay before and after transplantation.Results144 patients could be included in the final analysis. The cumulative survival rate at follow-up period was 67 ± 10% in group 1 vs. 60 ± 6% in group 2 (P = 0.769). The mean waiting time (days) on the list was 205 ± 155 in group 1 and 119 ± 69 in group 2 (P = 0.002). The mean length of hospital stay (days) was 214 ± 209 in group 1 and 128 ± 91 days in group 2. Early primary-graft-failure was 10% in group 1 and 13% in group 2. Odds ratio [OR] is as follows: 1.992, 95% confidence interval [CI]: 0.983–1.007, p = 0.266, aortic clamp time per minutes: OR: 1.008, 95% CI: (0.997–1.019), p = 0.164, HLM time per minutes: OR: 0.996, 95% CI: (0.991–1.001), p = 0.146, Operation time per minutes: OR: 1.000, 95% CI: (0.995–1.004), p = 0.861.ConclusionThe provision of pre-HTx VAD support does not have an adverse effect on the short- and long-term survival of pediatric patients undergoing HTx. A higher mortality rate was observed among children under three months of age with congenital heart disease. The patients who received VAD support were in a critical condition and required more ECMO support. The results demonstrated a statistically significant correlation between prolonged waiting times and length of hospital stay in group 1. More homogeneous and adequately powered cohorts are needed to better understand the impact of VAD support on posttransplant outcomes.

ObjectiveTo investigate the long-term impact of cardiac surgery on the quality of life in adults with congenital heart disease (ACHDs).MethodsPatients who had undergone cardiac surgery for congenital heart disease (CHD) at the age of 18 years or more were recruited in a single-center, cross-sectional study. The enrolled subjects completed online questionnaires to assess patient-reported outcomes: perceived health status and life satisfaction, psychological functioning, health behaviors, and illness perception. Clinical variables were correlated to the score results, and results were compared to representative samples from international and German national surveys of healthy subjects and ACHDs.ResultsWe enrolled 196 ACHDs (54% women), including 55% with more than one cardiac surgery during their lifetime. The median age at the survey was 43 years, with a median of 13 years since their last cardiac surgery. The majority of patients reported improved subjective wellbeing after cardiac operation and were in functional New York Heart Association class I or II. The severity of underlying CHD, number of previous cardiac operations, and beta-blocker medication had the most substantial negative effects on illness perception. Measured quality of life and health risk behaviors were within the range of values internationally reported for healthy and CHD subjects, respectively.ConclusionsACHD, several years after cardiac surgery, reported a subjective improvement in their wellbeing, a life satisfaction comparable to that of healthy individuals, and low health risk behaviors. Illness perception is strongly correlated with the severity of the underlying CHD.

Abstract Aims The aim of this study is to evaluate and compare the impact of the bicaval technique versus the biatrial technique (by Lower and Shumway) in paediatric heart transplant patients. Only a few studies investigate this matter regarding the long‐term outcome after paediatric heart transplantation. We compared the two surgical methods regarding survival, the necessity of pacemaker implantation. Methods and results All 134 patients (aged

IntroductionThe aim of this study was to analyze the results after pediatric heart transplantation (pHTx) at our single center differentiating between ABO-incompatible (ABOi) and -compatible (ABOc) procedures.Methods and patientsWe retrospectively analyzed outcomes of ABO-incompatible HTx procedures performed at our center and compared the data to ABO-compatible HTx of the same era. Eighteen children (

Abstract Background Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. Aims The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). Methods This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. Design The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. Processes Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. Assessments Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. Discussion of the design The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. Conclusion The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Objective: To evaluate the exercise capacity in patients following Fontan–Kreutzer, Fontan–Björk, and total cavopulmonary connection (TCPC). Methods: Patients who performed exercise capacity tests at least once after the Fontan procedure between 1979 and 2007 were included. Patients after Fontan–Björk procedure were divided into 2 groups according to the pulmonary blood flow (PBF) pattern: patients with pulsatile PBF and those without. Peak oxygen uptake (VO2) was measured and percent-predicted VO2 was calculated. Results: A total of 227 patients were nominated. The types of Fontan procedure included Fontan–Kreutzer in 48 (21.1%) patients, Fontan–Björk in 38 (16.7%); 11 (4.8%) with pulsatile PBF and 27 (11.9%) without pulsatile PBF; and TCPC in 141 (62.1%). Median age at the Fontan procedure was 4.5 years (interquartile range, 2.1-8.2 years). A total of 978 cardiopulmonary exercise tests were performed at median follow-up of 17.7 years (interquartile range, 11.3-23.4 years) postoperatively. Analysis using linear mixed-effects models demonstrated that percent-predicted VO2 was greater in patients with pulsatile PBF after Fontan–Björk compared with patients after other types of Fontan procedure (P

ObjectiveWhile early outcome after the Norwood operation for hypoplastic left heart syndrome has improved, interstage mortality until bidirectional cavopulmonary shunt (BCPS) remains a concern. Our aim was to institute a home monitoring program to (HMP) decrease interstage mortality.MethodsAmong 264 patients who survived Norwood procedure and were discharged before BCPS, 80 patients were included in the HMP and compared to the remaining 184 patients regarding interstage mortality. In patients with HMP, events during the interstage period were evaluated.ResultsInterstage mortality was 8% (n = 21), and was significantly lower in patients with HMP (2.5%, n = 2), compared to those without (10.3%, n = 19, p = 0.031). Patients with interstage mortality had significantly lower birth weight (p

Objectives: Severe hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS. Methods: The medical records of all patients who underwent BCPS between 2007 and 2020 were reviewed. Patients who underwent partial takedown were extracted and their outcomes were analyzed. Results: Of 441 BCPS patients, 27 patients (6%) required partial takedown. Most frequent diagnosis was hypoplastic left heart syndrome (n = 14; 52%). Additional complicating factors included pulmonary artery hypoplasia (n = 12) and pulmonary venous obstruction (n = 3). Thirteen patients (48%) underwent partial takedown on the same day of BCPS, and all of them survived the procedure. The remaining 14 patients (52%) underwent partial takedown between postoperative 1 to 64 days. The reasons for partial takedown were: postoperative high pulmonary vascular resistance (n = 4), early BCPS (

Pulmonary hypertension (PH) is a known and life limiting complication of preterm born young adults with bronchopulmonary dysplasia (BPD), ultimately leading to progressive right ventricular (RV) failure. Prognosis remains poor, especially in patients unresponsive to modern vasoactive pharmacotherapy. Therefore, lung transplantation presents the treatment of choice to avert cardiac failure. With limited donor organ availability and long waiting times, the implantation of a paracorporeal lung assist device (PLAD) is a way to bridge the patient as an alternative to veno-arterial ECMO. Herein, we present the case of a prematurely born 23-year-old female, who developed severe PH due to BPD and consequently experienced therapy refractory RV failure. Urgent PLAD implantation was performed and the patient successfully underwent double-lung transplantation after 215 days of PLAD support. No major PLAD-associated complications occurred and full recovery of RV function could be observed after double-lung transplantation.

Chronic thromboembolic pulmonary hypertension is a rare but life-threatening complication of long-term central venous catheters (CVC) in children. However, evidence in terms of potential treatment strategies and outcome data remains scarce. We describe two cases of CVC-related thrombosis (Hickman-catheter) complicated by recurrent pulmonary emboli. One patient experienced a complete thromboembolic obstruction of the right pulmonary artery with normal pulmonary pressures and the second patient suffered from a central thromboembolic obstruction of both pulmonary arteries associated with severe pulmonary hypertension. Both patients successfully underwent surgical thromboendarterectomy with deep hypothermic circulatory arrest.

ObjectiveCurrently used patch materials in congenital cardiac surgery do not grow, renew, or remodel. Patch calcification occurs more rapidly in pediatric patients eventually leading to reoperations. Bacterial cellulose (BC) as a biogenic polymer offers high tensile strength, biocompatibility, and hemocompatibility. Thus, we further investigated the biomechanical properties of BC for use as patch material.MethodsThe BC-producing bacteria Acetobacter xylinum were cultured in different environments to investigate optimal culturing conditions. For mechanical characterization, an established method of inflation for biaxial testing was used. The applied static pressure and deflection height of the BC patch were measured. Furthermore, a displacement and strain distribution analysis was performed and compared to a standard xenograft pericardial patch.ResultsThe examination of the culturing conditions revealed that the BC became homogenous and stable when cultivated at 29°C, 60% oxygen concentration, and culturing medium exchange every third day for a total culturing period of 12 days. The estimated elastic modulus of the BC patches ranged from 200 to 530 MPa compared to 230 MPa for the pericardial patch. The strain distributions, calculated from preloaded (2 mmHg) to 80 mmHg inflation, show BC patch strains ranging between 0.6% and 4%, which was comparable to the pericardial patch. However, the pressure at rupture and peak deflection height varied greatly, ranging from 67 to around 200 mmHg and 0.96 to 5.28 mm, respectively. The same patch thickness does not automatically result in the same material properties indicating that the manufacturing conditions have a significant impact on durabilityConclusionsBC patches can achieve comparable results to pericardial patches in terms of strain behavior as well as in the maximum applied pressure that can be withstood without rupture. Bacterial cellulose patches could be a promising material worth further research.

Ventricular assist device implantation presents a possible bridge to heart transplantation for patients with failing Fontan physiology. However, evidence regarding outcome and possible pitfalls associated with the Fontan circulation is still insufficient. We describe the course of a 13-year-old male, who was born with hypoplastic left heart syndrome and underwent HeartMate III implantation due to refractory failure of the systemic right ventricle.

BackgroundThis study investigated the volume and duration of pleural and mediastinal effusions following extracardiac total cavopulmonary connection, as well as preoperative risk factors and their impact on outcome.Materials and methodsA total of 210 patients who underwent extracardiac total cavopulmonary connection at our center between 2012 and 2020 were included in this study. Postoperative daily amount of pleural and mediastinal drainage were collected and factors influencing duration and amount of effusions were analyzed. The impact of effusions on adverse events was analyzed.ResultsMedian age at extracardiac total cavopulmonary connection was 2.2 (interquartile range, 1.8–2.7) years with median weight of 11.6 (10.7–13.0) kg. Overall duration of drainage after extracardiac total cavopulmonary connection was 9 (6–17) days. The total volume of mediastinal, right pleural, and left pleural drainage was 18.8 (11.9–36.7), 64.4 (27.4–125.9), and 13.6 (0.0–53.5) mL/kg, respectively. Hypoplastic left heart syndrome (p = 0.004) and end-diastolic pressure (p = 0.044) were associated with high volume of drainages, and hypoplastic left heart syndrome (p = 0.007), presence of aortopulmonary collaterals (p = 0.002), and high end-diastolic pressure (p = 0.023) were associated with long duration of drainages. Dextrocardia was associated with higher volume (p < 0.001) and longer duration (p = 0.006) of left pleural drainage. Duration of drainage was associated with adverse events following extracardiac total cavopulmonary connection (p = 0.015).ConclusionVolume and duration of pleural and mediastinal effusions following extracardiac total cavopulmonary connection were related with hypoplastic left heart syndrome, aortopulmonary collaterals, and end-diastolic pressure. The duration of drainage for effusions was a risk factor for adverse events after total cavopulmonary connection.

Abstract Progressive stenosis is one of the main factors that limit the lifetime of bioprosthetic valved conduits. To improve long-term performance we aimed to identify targets that inhibit pannus formation on conduit walls. From 11 explanted, obstructed, RNAlater presevered pulmonary valved conduits, we dissected the thickened conduit wall and the thin leaflet to determine gene expression-profiles using ultra deep sequencing. Differential gene expression between pannus and leaflet provided the dataset that was screened for potential targets. Promising target candidates were immunohistologically stained to see protein abundance and the expressing cell type(s). While immunostainings for DDR2 and FGFR2 remained inconclusive, EGFR, ErbB4 and FLT4 were specifically expressed in a subset of tissue macrophages, a cell type known to regulate the initiation, maintenance, and resolution of tissue repair. Taken toghether, our data suggest EGFR, ErbB4 and FLT4 as potential target candidates to limit pannus formation in bioprosthestic replacement valves.

Background Teratoma is a tumor derived from fetal germ cells with aberrant differentiation. Case Description A 3-month-old infant with a mediastinal tumor was referred to our heart center. She presented with progressive dyspnea, cyanosis, and the need to be manually ventilated. The computed tomography scan displayed a huge tumor restricting the distal trachea including the bifurcation. An emergent operation was performed and the tumor was completely removed. Histological examination confirmed a mature teratoma. Conclusion In such life-threatening situation, the early detection and the immediate operation are very important for the management of rapidly-progressing mediastinal teratomas compressing the respiratory tract.

Patients with congenital heart disease (CHD) are at increased risk for severe arrhythmia and sudden cardiac death (SCD). Although implantable cardioverter defibrillators (ICD) effectively prevent SCD, risk stratification for primary prophylaxis in patients with CHD remains challenging. Patients with complex CHD undergoing CPET were included in this single-center study. Univariable and backwards stepwise multivariable logistic regression models were used to identify variables associated with the endpoint of severe arrhythmic event during three years of follow-up. Cut-off values were established with receiver operating characteristic (ROC) curve analysis. Survival analysis was conducted via Kaplan–Meier plots. Severe Arrhythmia was documented in 97 of 1194 patients (8.1%/3 years). Independent risk factors for severe arrhythmia during follow-up were old age and a low peak oxygen uptake (V.O2peak) on multivariable analysis. Patients with more advanced age and with V.O2peak values of less than 24.9 mL/min/kg were at significantly increased risk for the occurrence of severe arrhythmias during follow-up. The combined analysis of both risk factors yielded an additional benefit for risk assessment. Age at CPET and V.O2peak predict the risk for severe arrhythmic events and should be considered for risk stratification of SCD in patients with complex CHD.

Endothelial progenitor cells (EPC) may influence the integrity and stability of the vascular endothelium. The association of an altered total EPC number and function with cardiovascular diseases (CVD) and risk factors (CVF) was discussed; however, their role and applicability as biomarkers for clinical purposes have not yet been defined. Endothelial dysfunction is one of the key mechanisms in CVD. The assessment of endothelial dysfunction in vivo remains a major challenge, especially for a clinical evaluation of the need for therapeutic interventions or for primary prevention of CVD. One of the main challenges is the heterogeneity of this particular cell population. Endothelial cells (EC) can become senescent, and the majority of circulating endothelial cells (CEC) show evidence of apoptosis or necrosis. There are a few viable CECs that have properties similar to those of an endothelial progenitor cell. To use EPC levels as a biomarker for vascular function and cumulative cardiovascular risk, a correct definition of their phenotype, as well as an update on the clinical application and practicability of current isolation methods, are an urgent priority.