PATHFINDER-CHD: prospective registry on adults with congenital heart disease, abnormal ventricular function, and/or heart failure as a foundation for establishing rehabilitative, prehabilitative, preventive, and health-promoting measures: rationale, aims, design and methods.

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.

Thymic Atrophy and Immune Dysregulation in Infants with Complex Congenital Heart Disease.

Congenital heart disease (CHD) is the most common birth defect, and up to 50% of infants with CHD require cardiovascular surgery early in life. Current clinical practice often involves thymus resection during cardiac surgery, detrimentally affecting T-cell immunity. However, epidemiological data indicate that CHD patients face an elevated risk for infections and immune-mediated diseases, independent of thymectomy. Hence, we examined whether the cardiac defect impacts thymus function in individuals with CHD. We investigated thymocyte development in 58 infants categorized by CHD complexity. To assess the relationship between CHD complexity and thymic function, we analyzed T-cell development, thymic output, and biomarkers linked to cardiac defects, stress, or inflammation. Patients with highly complex CHD exhibit thymic atrophy, resulting in low frequencies of recent thymic emigrants in peripheral blood, even prior to thymectomy. Elevated plasma cortisol levels were detected in all CHD patients, while high NT-proBNP and IL-6 levels were associated with thymic atrophy. Our findings reveal an association between complex CHD and thymic atrophy, resulting in reduced thymic output. Consequently, thymus preservation during cardiovascular surgery could significantly enhance immune function and the long-term health of CHD patients.

TGFß level in healthy and children with Marfan syndrome-effective reduction under sartan therapy.

Introduction: Transforming growth factor ß (TGFß) metabolism plays an important role in the pathogenesis of Marfan syndrome (MFS). Accordingly, drug therapy uses TGFß receptor blockade to slow down the cardiovascular manifestations, above all aortic root dilatation. Angiotensin II type 1 receptor blockers (ARBs) have been shown to reduce TGFß levels in adults. Data on childhood are lacking and are now being investigated in the TiGer For Kids study presented here. Methods: We examined 125 children without chronic disease and 31 pediatric Marfan patients with a proven FBN1 variant with regard to TGFß levels. In addition, we measured TGFß levels during the initiation of ARB therapy in pediatric Marfan patients. Results: In children without chronic disease, TGFß levels were found to decrease from childhood to adolescence (p < 0.0125). We could not measure a relevantly increased TGFß level in pediatric Marfan patients. However, we showed a significant suppression of the TGFß level after treatment with ARBs (p < 0.0125) and a renewed increase shortly before the next dose. Discussion: The TGFß level in childhood changes in an age-dependent manner and decreases with age. The TGFß level drops significantly after taking ARBs. Based on our experience and data, a TGFß receptor blockade in childhood seems reasonable. So far, TGFß level cannot be used as an MFS screening biomarker.

[New aspects in pediatric pulmonary hypertension - Commented 2022ERS/ESC-PH guidelines]. / Neue Aspekte bei der pulmonalen Hypertonie im Kindesalter ­ kommentierte 2022ERS/ESC-PH-Guidelines.

Pulmonary hypertension (PH) in childhood differs from that of adulthood particularly in the specific pathophysiology of congenital heart disease-associated pulmonary arterial hypertension, the presence of developmental lung disease, and the frequent association with chromosomal, genetic, and syndromal abnormalities. Treatment of children with PH requires a modified diagnostic algorithm tailored to childhood, as well as pathophysiologically oriented therapeutic strategies. In the current 2022 ERS/ESC-PH guidelines, the specific features of PH in children are highlighted in its own chapter and commented on by the authorship group in this article.

[Pulmonary arterial hypertension in congenital heart disease - Part I]. / Pulmonale arterielle Hypertonie bei Erwachsenen mit angeborenen Herzfehlern ­ Teil I.

The number of adults with congenital heart disease (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary arterial hypertension (PAH), which may develop early in untreated CHD. Despite timely treatment of CHD, PAH often persists or recurs in older age and is associated with significant morbidity and mortality.The revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart defects" is addressed only relatively superficially in these guidelines. Therefore, this article addresses the perspective of congenital cardiology in greater depth.

Comparison of the Prolonged- and Immediate-Release Tacrolimus Capsule Formulation: The Patient's View and Medication Satisfaction of Patients After Pediatric Heart Transplantation.

BACKGROUND: Medication adherence is essential for long-term success after pediatric organ transplantation. Causes of reduced adherence should be detected early to improve the consequent medication intake. We describe the influence of switching from tacrolimus twice daily (tacrolimus-BID) to tacrolimus once daily (tacrolimus-QD) on medication satisfaction and medication adherence in patients after pediatric heart transplantation. METHODS: A retrospective analysis was conducted regarding patient satisfaction and adherence to the immunosuppressant tacrolimus after pediatric heart transplantation, before and after conversion from tacrolimus-BID to tacrolimus-QD, using questionnaires. RESULTS: Thirty-eight patients were enrolled (tacrolimus-BID: n = 35, mean age 15.7 ± 5.2 years; tacrolimus-QD: n = 38, mean age 16.2 ± 5.6 years). The amount of unadministered medication in the last 3 months did not differ between the 2 pharmaceutical forms. However, 17% (n = 6) reported unstable tacrolimus trough levels when taking tacrolimus-BID, vs 8% (n = 3) under tacrolimus-QD (P = .453). However, there was no statistically significant difference in the stability of the last 6 trough levels of each patient (P = .074). A total of 57% (n = 20) of patients had subjective side effects before conversion, compared to only 29% (n = 11) after conversion (P = .013). Regarding the intensity of the side effects, 6 patients reported strong/very strong side effects when taking tacrolimus-BID vs 1 patient when taking tacrolimus-QD (P = .250). In addition, the overall satisfaction with the immunosuppressant was higher under tacrolimus-QD (92% vs 83%; P = .508). However, this improvement was statistically not significant and may not be clinically relevant. CONCLUSIONS: The amount of forgotten medication was not reduced after conversion from tacrolimus-BID to tacrolimus-QD. However, subjective side effects as well as patient satisfaction improved under tacrolimus-QD.

Siblings with Gorlin-Goltz syndrome associated with cardiac tumors: a case report and review of literature.

Primary cardiac tumors in children are very rare and may be associated with severe arrhythmias and sudden infant death syndrome. These cardiac arrhythmias vary depending on the location and size of the tumor. Sixty-four percent of children with cardiac fibroma, the second most common benign cardiac tumor in children, have ventricular arrhythmias, affecting therapeutic management and risk profile of these children. We report on two siblings with cardiac fibromas whose clinical presentations differed depending on their locations and size of the tumors. The first child, a three-year-old girl, was diagnosed with a cardiac fibroma in the left ventricle at the age of 8 months after surviving resuscitation due to ventricular fibrillation. Secondary prophylactic implantation of an ICD was performed. On propranolol, no further malignant arrhythmias have occurred to date. The seven-month-old brother was diagnosed postnatally with a cardiac tumor adjacent to the right ventricle. A few weeks after birth, the boy had refractory supraventricular tachycardia and ventricular arrhythmia that only resolved with amiodarone. In genetic testing, Gorlin-Goltz syndrome was diagnosed in both children. Conservative pharmacological therapy is a therapeutic strategy for asymptomatic patients with cardiac fibromas. The anti-arrhythmic medication depends on the location of the tumor. Implantation of an ICD should be performed in cases of malignant arrhythmias. In rare cases, there is an association between cardiac tumors and genetic syndromes, such as Gorlin-Goltz syndrome. These should always be considered when such a tumor is diagnosed.

A Systematic Review and Meta-analysis on Stenting for Aortic Coarctation Management in Adults.

PURPOSE: Endovascular treatment of aortic coarctation (CoA) constitutes a valuable alternative with low morbidity and mortality. The aim of this systematic review and meta-analysis was to assess the technical success, re-intervention, and mortality after stenting for CoA in adults. MATERIALS AND METHODS: The Preferred Reporting Items for Systematic Reviews and Meta-analysis statement and PICO (patient, intervention, comparison, outcome) model were followed. An English literature data search was conducted, using PubMed, EMBASE, and CENTRAL, until December 30, 2021. Only studies reporting on stenting, for native or recurrent CoA, in adults were included. The risk of bias was assessed using the Newcastle-Ottawa Scale. A proportional meta-analysis was performed to assess the outcomes. Primary outcomes were technical success, intra-operative pressure gradient and complications, and 30-day mortality. RESULTS: Twenty-seven articles (705 patients) were included (64.0% males, 34.0±13.6 years). Native CoA was present in 65.7%. Technical success was 97% (95% confidence interval [CI], 0.96%-0.99%; p<0.001, I2=9.49%). Six (odds ratio [OR]: 1%; 95% CI, 0.00%-0.02%; p=0.002, I2=0%) ruptures and 10 dissections (OR: 2%; 95% CI, 0.001%-0.02%; p<0.001, I2=0%) were reported. The intra-operative and 30-day mortality were 1% (95% CI, 0.00%-0.02%; p=0.003, I2=0%) and 1% (95% CI, 0.00%-0.02%; p=0.004, I2=0%), respectively. The median follow-up was 29 months. Sixty-eight re-interventions (OR: 8%; 95% CI, 0.05%-0.10%; p<0.001, I2=35.99%) were performed; 95.5% were endovascular. Seven deaths were reported (OR: 2%; 95% CI, 0.00%-0.03%; p=0.008, I2=0%). CONCLUSION: Stenting for CoA in adults presents high technical success and the intra-operative and 30-day mortality rates were acceptable. During the midterm follow-up, the re-intervention rate was acceptable, and mortality was low. CLINICAL IMPACT: Aortic coarctation is a quite common heart defect that may be diagnosed in adult patients, as a first diagnosis in native cases or as a recurrent after previous repair. Endovascular management using plain angioplasty has been associated to a high intra-operative complication and re-intervention rate. Stenting in this analysis seems to be safe and effective as is related a high technical success rate, exceeding 95%, with a low intra-operative complication and death rate. During the mid-term follow-up, the re-interventions rate is estimated at less than 10% while most cases are managed using endovascular means. Further analyses are needed on the impact of stent type on endovascular repair outcomes.

Outcomes of extracorporeal membrane oxygenation and cardiopulmonary bypass in children after drowning-related resuscitation.

Drowning is one of the leading causes of accidental deaths in children worldwide. However, the use of long-term extracorporeal life support (ECLS) in this setting is not widely established, and rewarming is often achieved by short-term cardiopulmonary bypass (CPB) treatment. Thus, we sought to add our experience with this means of support as a bridge-to-recovery or to-decision. This retrospective single-center study analyzes the outcome of 11 children (median 23 months, minimum-maximum 3 months-6.5 years) who experienced drowning and subsequent cardiopulmonary resuscitation (CPR) between 2005 and 2016 and who were supported by veno-arterial extracorporeal membrane oxygenation (ECMO), CPB, or first CPB then ECMO. All but one incident took place in sweet water. Submersion time ranged between 10 and 50 minutes (median 23 minutes), water temperature between 2°C and 28°C (median 14°C), and body core temperature upon arrival in the emergency department between 20°C and 34°C (median 25°C). Nine patients underwent ongoing CPR from the scene until ECMO or CPB initiation in the operating room. The duration of ECMO or CPB before successful weaning/therapy withdrawal ranged between 2 and 322 hours (median 19 hours). A total of four patients (36%) survived neurologically mildly or not affected after 4 years of follow-up. The data indicate that survival is likely related to a shorter submersion time and lower water temperature. Resuscitation of pediatric patients after drowning has a poor outcome. However, ECMO or CPB might promote recovery in selected cases or serve as a bridge-to-decision tool.

Impact of delayed sternal closure on wound infections following neonatal and infant cardiac surgery.

OBJECTIVES: Delayed sternal closure is a routine procedure to reduce hemodynamic and respiratory instability in pediatric patients following cardiac surgery, particularly in neonates and infants. In this setting, the possible links between sternal wound infection and delayed sternal closure are still a matter of debate. As a part of our routine, there was a low threshold for delayed sternal closure, so we reviewed our experience with sternal wound infections with a focus on potentially related perioperative risk factors, particularly delayed sternal closure. METHODS: We retrospectively identified 358 operated neonates (37%) and infants (mean age 3.6 months) in our local congenital heart disease database between January 2013 and June 2017. Potential risk factors for sternal wound infections, such as age, gender, complexity (based on Aristotle- and STS-EACTS mortality category), reoperation, use of cardiopulmonary bypass, extracorporeal membrane oxygenation, mortality and delayed sternal closure (163/358, 46%), were subjected to uni- and multivariate analysis. RESULTS: A total of 26/358 patients (7.3%) developed a superficial sternal wound infection. There were no deep sternal wound infections, no mediastinitis or sepsis. Applying univariate analysis, the prevalence of sternal wound infections was related to younger age, more complex surgery and delayed sternal closure. However, in multivariate analysis, sternal wound infection was only associated with delayed sternal closure (p = 0.013, odds ratio 8.6). Logistic regression revealed the prevalence of delayed sternal closure to be related to younger age, complexity, and the use of extracorporeal membrane oxygenation. CONCLUSION: In patients younger than one year, sternal wound infections are clearly related to delayed sternal closure. However, in our cohort, all sternal wound infections were superficial and acceptable, considering the improved postoperative hemodynamic stability.

Paediatric and adult congenital cardiology education and training in Europe.

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

Tricuspid valve prolapse as an early predictor for severe phenotype in children with Marfan syndrome.

AIM: In Marfan syndrome, various cardiovascular pathologies, such as aortic dilatation and mitral valve pathologies, already occur in childhood and determine course of the disease. This study aimed to establish additional cardiovascular risk markers for severe Marfan phenotypes. We investigated tricuspid valve prolapse (TVP) and its predictive value for outcome of paediatric Marfan disease. METHODS: In this retrospective, observational cohort study, we identified 130 paediatric Marfan patients (10.7 ± 4.8 years) with FBN1 variants. We divided patients into two groups based on TVP presence and performed a cross-sectional analysis to investigate the association of TVP with other cardiovascular, ocular and systemic pathologies, at first and last visit. A longitudinal analysis was performed with follow-up data. RESULTS: At baseline, patients with TVP had higher incidence of aortic root dilatation (p = 0.013), mitral valve prolapse (p = 0.0001) and systemic manifestations (p = 0.025) than patients without TVP. At follow-up, previous presence of TVP predicted higher probability of aortic root dilatation (p = 0.002), mitral valve prolapse (p = 0.0001) and systemic manifestations (p = 0.002). CONCLUSION: This shows that TVP is linked to both cardiac and extracardiac Marfan manifestations and TVP is an important marker for a disease severity in these children. Therefore, TVP should be assessed routinely using echocardiography in paediatric Marfan patients.

Retrieval of a Dislocated Stent Using a Grasping Forceps and a Steerable Sheath in a Fontan Patient.

In Fontan patients, any pulmonary stenosis may impede free passive inflow into the pulmonary circuit and elevate central venous pressure. When stenting such pulmonary stenosis, dislocation of a stent is a feared complication. Here, we report on a successful retrieval of a dislocated Cook Formula stent into the peripheral left pulmonary artery in a Fontan patient with protein-losing enteropathy using a steerable sheath and a grasping forceps. Furthermore, a successful stent implantation for treating the stenosis was possible. Subsequently, we simulated the retraction technique ex vivo. To manage stent dislocation during complex pediatric catheter interventions, we can recommend the use of a steerable sheath guiding a grasping forceps. A dislocated stent can be grasped, completely longitudinally refolded, and safely retrieved.

Outcomes of Tricuspid Valve Detachment for Isolated Ventricular Septal Defect Closure.

Partial detachment of the septal and anterior leaflets of the tricuspid valve (TV) is a technique to visualize a perimembranous ventricular septal defect (VSD) for surgical closure in cases where the VSD is obscured by TV tissue. However, TV incision bears the risk of causing relevant postoperative TV regurgitation and higher degree atrioventricular (AV) block. A total of 40 patients were identified retrospectively in our institution who underwent isolated VSD closure between January 2013 and August 2015. Visualization of the VSD was achieved in 20 patients without and in 20 patients with additional partial detachment of the TV. The mean age of patients with partial tricuspid valve detachment (TVD) was 0.7 ± 0.1 years compared with 1 ± 0.3 years (p = 0.22) of patients without TVD. There was no difference in cardiopulmonary bypass time between patients of both groups (123 ± 11 vs. 103 ± 5 minutes, p = 0.1). Cross-clamp time was longer if the TV was detached (69 ± 5 vs. 54 ± 4 minutes, p = 0.023). There was no perioperative mortality. Echocardiography at discharge and after 2.5 years (2 months-6 years) of follow-up showed neither a postoperative increase of tricuspid regurgitation nor any relevant residual shunt. Postoperative electrocardiograms were normal without any sign of higher degree AV block. TVD offers enhanced exposure and safe treatment of VSDs. It did not result in higher rates of TV regurgitation or relevant AV block compared with the control group.

Fatal COVID-19 in a Child with Persistence of SARS-CoV-2 Despite Extensive Multidisciplinary Treatment: A Case Report.

Critical Coronavirus disease 2019 (COVID-19) developed in a 7-year-old girl with a history of dystrophy, microcephaly, and central hypothyroidism. Starting with gastrointestinal symptoms, the patient developed severe myocarditis followed by progressive multiple organ failure complicated by Pseudomonas aeruginosa bloodstream infection. Intensive care treatment consisting of invasive ventilation, drainage of pleural effusion, and high catecholamine therapy could not prevent the progression of heart failure, leading to the implantation of venoarterial extracorporeal life support (VA-ECLS) and additional left ventricle support catheter (Impella® pump). Continuous venovenous hemofiltration (CVVH) and extracorporeal hemadsorption therapy (CytoSorb®) were initiated. Whole exome sequencing revealed a mutation of unknown significance in DExH-BOX helicase 30 (DHX30), a gene encoding a RNA helicase. COVID-19 specific antiviral and immunomodulatory treatment did not lead to viral clearance or control of hyperinflammation resulting in the patient's death on extracorporeal life support-(ECLS)-day 20. This fatal case illustrates the potential severity of pediatric COVID-19 and suggests further evaluation of antiviral treatment strategies and vaccination programs for children.

AntimiR-132 Attenuates Myocardial Hypertrophy in an Animal Model of Percutaneous Aortic Constriction.

BACKGROUND: Pathological cardiac hypertrophy is a result of afterload-increasing pathologies including untreated hypertension and aortic stenosis. It features progressive adverse cardiac remodeling, myocardial dysfunction, capillary rarefaction, and interstitial fibrosis often leading to heart failure. OBJECTIVES: This study aimed to establish a novel porcine model of pressure-overload-induced heart failure and to determine the effect of inhibition of microribonucleic acid 132 (miR-132) on heart failure development in this model. METHODS: This study developed a novel porcine model of percutaneous aortic constriction by implantation of a percutaneous reduction stent in the thoracic aorta, inducing progressive remodeling at day 56 (d56) after pressure-overload induction. In this study, an antisense oligonucleotide specifically inhibiting miR-132 (antimiR-132), was regionally applied via intracoronary injection at d0 (percutaneous transverse aortic constriction induction) and d28. RESULTS: At d56, antimiR-132 treatment diminished cardiomyocyte cross-sectional area (188.9 ± 2.8 vs. 258.4 ± 9.0 µm2 in untreated hypertrophic hearts) and improved global cardiac function (ejection fraction 48.9 ± 1.0% vs. 36.1 ± 1.7% in control hearts). Moreover, at d56 antimiR-132-treated hearts displayed less increase of interstitial fibrosis compared with sham-operated hearts (Δsham 1.8 ± 0.5%) than control hearts (Δsham 10.8 ± 0.6%). Of note, cardiac platelet and endothelial cell adhesion molecule 1+ capillary density was higher in the antimiR-132-treated hearts (647 ± 20 cells/mm2) compared with in the control group (485 ± 23 cells/mm2). CONCLUSIONS: The inhibition of miR-132 is a valid strategy in prevention of heart failure progression in hypertrophic heart disease and may be developed as a treatment for heart failure of nonischemic origin.

Impact of hepatopathy in pediatric patients after surgery for complex congenital heart disease.

Patients undergoing complex pediatric cardiac surgery in early infancy are at risk of postoperative secondary end-organ dysfunction. The aim of this study was to determine specific risk factors promoting the development of peri- and postoperative hepatopathy after surgery for congenital heart disease. In this retrospective study, we identified 20 consecutive patients operated between 2011 and 2019 from our institutional cohort who developed significant postsurgical hepatic dysfunction. These patients were compared to a control group of 30 patients with comparable initial cardiac conditions and STS-EACTS risk score. Patients who developed hepatopathy in the intensive care unit have chronic cholestasis and decreased liver synthesis. The impact of postoperative hepatopathy on morbidity was marked. In six patients (30%), liver transplantation was executed as ultima ratio, and two (10%) were listed for liver transplantation. The overall mortality related to postoperative hepatopathy is high: We found nine patients (45%) having severe hepatopathy and mostly multiple organ dysfunction who died in the postoperative course. According to risk analysis, postoperative right and left heart dysfunction in combination with a postoperative anatomical residuum needing a re-operation or re-intervention in the postoperative period is associated with a high risk for the development of cardiac hepatopathy. Furthermore, postoperative complications (pleural effusion, heart rhythm disorders, etc.), postoperative infections, and the need for parenteral nutrition also raise the risk for cardiac hepatopathy. Further investigations are needed to reduce hepatic complications and improve the general prognosis of such complex patients.

Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry.

BACKGROUND: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. METHODS: The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD. RESULTS: A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of follow-up, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of "Non-Eisenmenger PAH" patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described. CONCLUSIONS: Analyzing "real life data" from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the "Non-Eisenmenger PAH" group and to patients with complex CHD, including Fontan patients. TRIAL REGISTRATION: www.clinicaltrials.gov, study identifier: Clinicaltrials.gov NCT01347216.

Narrative review of Ebstein's anomaly beyond childhood: Imaging, surgery, and future perspectives.

Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.

Autologous Tissue Technique to Repair Unilateral Isolated Absence of a Pulmonary Artery.

Isolated unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly in which one branch pulmonary artery has no connection to the main pulmonary trunk (most often there is ductal origin). Without treatment, it may lead to ipsilateral pulmonary hypoplasia and contralateral pulmonary artery hypertension. To avoid these complications, early surgical repair of UAPA is necessary. Surgical strategies include direct anastomosis between the "isolated" branch pulmonary artery (PA) and the main pulmonary trunk or creation of an interposition graft using prosthetic material or flap techniques. We describe a surgical technique using a totally autologous interposition tube graft.