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.

Non-contrast free-breathing 2D CINE compressed SENSE T1-TFE cardiovascular MRI at 3T in sedated young children for assessment of congenital heart disease.

Cardiac MRI is a crucial tool for assessing congenital heart disease (CHD). However, its application remains challenging in young children when performed at 3T. The aim of this retrospective single center study was to compare a non-contrast free-breathing 2D CINE T1-weighted TFE-sequence with compressed sensing (FB 2D CINE CS T1-TFE) with 3D imaging for diagnostic accuracy of CHD, image quality, and vessel diameter measurements in sedated young children. FB 2D CINE CS T1-TFE was compared with a 3D non-contrast whole-heart sequence (3D WH) and 3D contrast-enhanced MR angiography (3D CE-MRA) at 3T in 37 CHD patients (20♂, 1.5±1.4 years). Two radiologists independently assessed image quality, type of CHD, and diagnostic confidence. Diameters and measures of contrast and sharpness of the aorta and pulmonary vessels were determined. A non-parametric multi-factorial approach was used to estimate diagnostic accuracy for the diagnosis of CHD. Linear mixed models were calculated to compare contrast and vessel sharpness. Krippendorff's alpha was determined to quantify vessel diameter agreement. FB 2D CINE CS T1-TFE was rated superior regarding image quality, diagnostic confidence, and diagnostic sensitivity for both intra- and extracardiac pathologies compared to 3D WH and 3D CE-MRA (all p<0.05). FB 2D CINE CS T1-TFE showed superior contrast and vessel sharpness (p<0.001) resulting in the highest proportion of measurable vessels (740/740; 100%), compared to 3D WH (530/620; 85.5%) and 3D CE-MRA (540/560; 96.4%). Regarding vessel diameter measurements, FB 2D CINE CS T1-TFE revealed the closest inter-reader agreement (Krippendorff's alpha: 0.94-0.96; 3D WH: 0.78-0.94; 3D CE-MRA: 0.76-0.93). FB 2D CINE CS T1-TFE demonstrates robustness at 3T and delivers high-quality diagnostic results to assess CHD in sedated young children. Its ability to function without contrast injection and respiratory compensation enhances ease of use and could encourage widespread adoption in clinical practice.

Endovascular Recanalization of Aortic Isthmus Atresia with an "Electrified Wire Technique".

PURPOSE: For aortic coarctation in adults endovascular repair is the treatment of choice with an acceptable safety profile. Aortic isthmus atresia is a related condition with a complete occlusion of the aorta not allowing catheterization across the isthmus. This technical note describes a recanalization of an aortic isthmus atresia using radiofrequency with an "electrified wire technique." TECHNIQUE: A guidewire was selectively denuded of PTFE (polytetrafluoroethylene) at the distal end and was placed through a catheter distal to the aortic isthmus atresia. The denuded end of the wire was clamped to an electrosurgery pencil. By pushing the wire toward a tulip-snare, which was placed as a target proximal of the occlusion via left trans-brachial access, and shortly activating of the electrosurgery pencil the electrified wire recanalized the occlusion and was snared and used to guide implantation of a balloon-expandable covered stent. CONCLUSION: The electrified wire puncture technique can be used to recanalize adult aortic isthmus atresia after failed conventional attempts. CLINICAL IMPACT: The electrified wire technique offers an off-the shelf option to modify standard guidewires for the use with radiofrequency to cross a complete aortic isthmus occlusion after failed conventional attempts. This new technique may be applied also in other situations like dissection flap fenestration, transcaval access and similar.

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.

Myocardial Tissue Characterization in Cardiac Magnetic Resonance Studies of Patients Recovering From COVID-19: A Meta-Analysis.

Background Meta-analysis can identify biological factors that moderate cardiac magnetic resonance myocardial tissue markers such as native T1 (longitudinal magnetization relaxation time constant) and T2 (transverse magnetization relaxation time constant) in cohorts recovering from COVID-19 infection. Methods and Results Cardiac magnetic resonance studies of patients with COVID-19 using myocardial T1, T2 mapping, extracellular volume, and late gadolinium enhancement were identified by database searches. Pooled effect sizes and interstudy heterogeneity (I2) were estimated with random effects models. Moderators of interstudy heterogeneity were analyzed by meta-regression of the percent difference of native T1 and T2 between COVID-19 and control groups (%ΔT1 [percent difference of the study-level means of myocardial T1 in patients with COVID-19 and controls] and %ΔT2 [percent difference of the study-level means of myocardial T2 in patients with COVID-19 and controls]), extracellular volume, and the proportion of late gadolinium enhancement. Interstudy heterogeneities of %ΔT1 (I2=76%) and %ΔT2 (I2=88%) were significantly lower than for native T1 and T2, respectively, independent of field strength, with pooled effect sizes of %ΔT1=1.24% (95% CI, 0.54%-1.9%) and %ΔT2=3.77% (95% CI, 1.79%-5.79%). %ΔT1 was lower for studies in children (median age: 12.7 years) and athletes (median age: 21 years), compared with older adults (median age: 48 years). Duration of recovery from COVID-19, cardiac troponins, C-reactive protein, and age were significant moderators for %ΔT1 and/or %ΔT2. Extracellular volume, adjusted by age, was moderated by recovery duration. Age, diabetes, and hypertension were significant moderators of the proportion of late gadolinium enhancement in adults. Conclusions T1 and T2 are dynamic markers of cardiac involvement in COVID-19 that reflect the regression of cardiomyocyte injury and myocardial inflammation during recovery. Late gadolinium enhancement and to a lesser extent extracellular volume, are more static biomarkers moderated by preexisting risk factors linked to adverse myocardial tissue remodeling.

Treatment of advanced heart failure in adults with congenital heart disease: a narrative review and clinical cases.

Background and Objective: The number of adults with congenital heart disease (ACHD) is increasing worldwide. Almost all congenital cardiac lesions can be successfully treated due to the progress in neonatal surgery and pediatric cardiology with a high likelihood of surviving until adulthood. However, ACHD frequently develop sequelae related to the initial cardiac anomaly. Heart failure (HF) is one of the most common complications associated with a high morbidity and mortality. Methods: The authors did search the PubMed database regarding relevant content covering publications up to March 2022. Relevant manuscripts were classified according to the impact factor of the journal, being a guideline manuscript, a position paper by a society or a comprehensive review of the current literature. Key Content and Findings: Optimal HF treatment remains an unmet need in ACHD. In particular, advanced HF therapy with cardiac resynchronization therapy, ventricular assist devices or organ transplantation is still very different and more specific in ACHD compared to non-ACHD. This review aims to compile international views and evidence from the literatures on the treatment of advanced HF in ACHD. Current challenges, but also the success of different treatment strategies in ACHD are illustrated by clinical cases. Conclusions: The main finding of the review is that data is still scarce regarding ACHD with advanced HF and international efforts to collect data regarding these patients needed to improve the current standard of care.

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.

Myocardial Perfusion in Hypoplastic Left Heart Syndrome.

BACKGROUND: The status of the systemic right ventricular coronary microcirculation in hypoplastic left heart syndrome (HLHS) is largely unknown. It is presumed that the systemic right ventricle's coronary microcirculation exhibits unique pathophysiological characteristics of HLHS in Fontan circulation. The present study sought to quantify myocardial blood flow by cardiac magnetic resonance imaging and evaluate the determinants of microvascular coronary dysfunction and myocardial ischemia in HLHS. METHODS: One hundred nineteen HLHS patients (median age, 4.80 years) and 34 healthy volunteers (median age, 5.50 years) underwent follow-up cardiac magnetic resonance imaging ≈1.8 years after total cavopulmonary connection. Right ventricle volumes and function, myocardial perfusion, diffuse fibrosis, and late gadolinium enhancement were assessed in 4 anatomic HLHS subtypes. Myocardial blood flow (MBF) was quantified at rest and during adenosine-induced hyperemia. Coronary conductance was estimated from MBF at rest and catheter-based measurements of mean aortic pressure (n=99). RESULTS: Hyperemic MBF in the systemic ventricle was lower in HLHS compared with controls (1.89±0.57 versus 2.70±0.84 mL/g per min; P<0.001), while MBF at rest normalized by the rate-pressure product, was similar (1.25±0.36 versus 1.19±0.33; P=0.446). Independent risk factors for a reduced hyperemic MBF were an HLHS subtype with mitral stenosis and aortic atresia (P=0.017), late gadolinium enhancement (P=0.042), right ventricular diastolic dysfunction (P=0.005), and increasing age at total cavopulmonary connection (P=0.022). The coronary conductance correlated negatively with systemic blood oxygen saturation (r, -0.29; P=0.02). The frequency of late gadolinium enhancement increased with age at total cavopulmonary connection (P=0.014). CONCLUSIONS: The coronary microcirculation of the systemic ventricle in young HLHS patients shows significant differences compared with controls. These hypothesis-generating findings on HLHS-specific risk factors for microvascular dysfunction suggest a potential benefit from early relief of frank cyanosis by total cavopulmonary connection.

Heart failure in adults with congenital heart disease: a narrative review.

The number of adults with congenital heart disease (ACHD) has increased over the last decades due to advancements in medical care, including interventional and surgical therapies. We are therefore more frequently challenged by the long-term consequences of palliative or corrective surgery carried out during childhood. Although patients with ACHD may develop conditions related to general cardiovascular risk factors, such as coronary artery disease, the most common complications leading to morbidity and mortality are arrhythmias, heart failure and thromboembolic events. For the management of arrhythmias, current recommendations regarding ablation and device therapy must be considered, whilst also taking into account the anatomical limitations of their congenital heart defect or surgical pathways. Heart failure treatment in acute and chronic settings must also consider the particular anatomy present, including the nature of the systemic ventricle. Treatments strategies for ACHD are typically extrapolated from the respective guidelines in non-ACHD patients, despite a lack of evidence to support this strategy. Right heart failure can be especially challenging to manage in conditions where either a systemic right ventricle or shunt lesions resulting in volume and/or pressure loading of the right ventricle are present. All physicians and cardiologists in particular should be acquainted with the most common diseases in ACHD, their complications and management regime, especially with regards to heart failure as this is a common reason for acute presentation in the emergency department.

Right aortic arch forming a true vascular ring: a clinical review.

OBJECTIVES: This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided ligamentum forming a complete vascular ring around the oesophagus and trachea. METHODS: A systematic database search for appropriate literature was conducted on PubMed/MEDLINE. Articles were considered relevant when providing details on the presentation, diagnosis and surgical treatment of this specific congenital arch anomaly in human beings. RESULTS: Affected patients present with respiratory and/or oesophageal difficulties due to tracheoesophageal compression. Conservative treatment might be reasonable in asymptomatic or mildly symptomatic cases; however, once moderate-to-severe symptoms develop, surgical intervention is definitely indicated. Surgery is commonly performed through a left thoracotomy or median sternotomy and includes the division of the left ductal ligamentum; if a Kommerell's diverticulum is present that is >1.5 times the diameter of the subclavian artery, then concomitant resection of the large diverticulum and translocation of the aberrant left subclavian artery is also conducted. Postoperative morbidity and mortality are low and are rather related to concomitant intracardiac and extracardiac anomalies than to the procedure itself. In a majority of patients, full resolution of symptoms is seen within months to years from the surgery. Nevertheless, there is also a subset of patients who remain with some tracheobronchial narrowing, sometimes even requiring reintervention during follow-up due to persisting or recurring symptoms. CONCLUSIONS: Overall, the surgical management of a right aortic arch forming a true vascular ring in infancy, childhood and adulthood seems relatively safe and effective in providing symptomatic relief despite some persistent tracheobronchial and/or oesophageal narrowing in some cases.

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.

Anticoagulation management in adult patients with congenital heart disease: a narrative review.

With improvements in treatment of congenital heart disease more paediatric patients are surviving with palliative or corrective interventions during childhood, thus becoming adults with congenital heart disease (ACHD). Overall, the ACHD population is at a higher risk of arrhythmias and stroke. The abnormal structure and function of their corrected hearts suggests that in addition to established stroke risk factors, such as prior stroke or older age, additional stroke risk factors need to be considered to determine the risk and establish the indication for oral anticoagulation (OAC) in ACHD patients. In structurally normal hearts non-vitamin-K oral anticoagulants (NOACs) offer at least equal stroke prevention with a better safety profile compared to vitamin K antagonists (VKA) in patients with atrial fibrillation (AF) or pulmonary embolism. Current guidelines recommend NOACs in ACHD patients with simple lesions and indication for OAC, while there is less certainty about their safety in ACHD patients with moderate or complex congenital heart disease such as patients with transposition of the great arteries (TGA) after atrial switch operation (Senning or Mustard operation), Fontan circulation or congenital corrected transposition of the great arteries (ccTGA). This review summarises the available evidence characterising stroke risk in patients with ACHD and the use of anticoagulants and interventional therapies to reduce that risk.

Outcome of pregnancy in a contemporary cohort of adults with congenital heart disease-a 10-year, single-center experience.

BACKGROUND: Pregnancy may be associated with adverse outcome in women with congenital heart disease (CHD). However, data regarding the outcome of pregnancy in women with CHD who receive care in cardiac-obstetric expert units are limited. METHODS: We retrospectively analyzed baseline characteristics and outcome of pregnancy in 67 females with CHD who received medical care in our tertiary center for 61 singleton and 6 twin pregnancies between 2009 and 2018. RESULTS: According to the modified World Health Organization (mWHO) risk scale for pregnancy, CHD lesions in 39 enrolled women (58%) were classified as mWHO class I or II, and in 28 females (42%) as mWHO class III or IV. Preterm births were more frequent in mWHO classes III or IV (P=0.003). Cardiac signs and complications occurred more often in mWHO classes III or IV than in women with cardiac lesions assigned to mWHO classes I or II (42.9% vs. 7.7%, P=0.002). N-terminal pro B-type natriuretic peptide (NT-proBNP) levels during pregnancy were higher in mWHO classes III or IV than in mWHO classes I or II (median 269.0 vs. 115.5 pg/mL, P=0.019). Presence of functional NYHA class III [odds ratio (OR) per standard deviation (SD) 8.8, 95% confidence interval (CI): 2.2-57.2, P=0.008] and mWHO classes III/IV (OR per SD 3.4, 95% CI: 1.2-9.9, P=0.018) prior to pregnancy were identified as independent predictors of adverse cardiac outcome of pregnancy. CONCLUSIONS: Adverse cardiac events and preterm deliveries should be anticipated in pregnant women with CHD, especially in those with mWHO classes III or IV. Therefore, these pregnancies should be under close surveillance and managed in specialized, multidisciplinary tertiary referral centers. Preconception counseling including individualized risk assessment is strongly recommended in women with CHD.

MRI-based comprehensive analysis of vascular anatomy and hemodynamics.

BACKGROUND: Standardized methods for mapping the complex blood flow in vessels are essential for processing the large data volume acquired from 4D Flow MRI. We present a method for systematic and efficient analysis of anatomy and flow in large human blood vessels. To attain the best outcomes in cardiac surgery, vascular modifications that lead to secondary flow patterns such as vortices should be avoided. In this work, attention was paid to the undesired cancelation of vortices with opposite directions of rotation, known as Dean flow patterns, using hemodynamic parameters such as circulation and helicity density. METHODS: Our approach is based on the multiplanar reconstruction (MPR) of a multi-dimensional feature-space along the blood vessel's centerline. Hemodynamic parameters and anatomic information were determined in-plane from the reconstructed feature-space and from the blood vessel's centerline. A modified calculation of circulation and helicity density and novel parameters for quantifying Dean flow were developed. To test the model performance, we applied our methods to three test cases. RESULTS: Comprehensive information on position, magnitude and interrelation of vascular anatomy and hemodynamics were extracted from 4D Flow MRI datasets. The results show that the Dean flow patterns can be efficiently assessed using the novel parameters. CONCLUSIONS: Our approach to comprehensively and simultaneously quantify multiple parameters of vascular anatomy and hemodynamics from 4D Flow MRI provides new insights to map complex hemodynamic conditions.

Abnormal torsion and helical flow patterns of the neo-aorta in hypoplastic left heart syndrome assessed with 4D-flow MRI.

BACKGROUND: The Norwood procedure is the first stage of correction for patients with hypoplastic left heart syndrome (HLHS) and may lead to an abnormal neoaortic anatomy. We prospectively studied the neoaorta's fluid dynamics and the abnormal twist of the neoaorta by MRI examinations of HLHS patients in Fontan circulation. This study for the first time investigates the hypothesis that the neoaorta twist is associated with increased helical flow patterns, which may lead to an increased workload for the systemic right ventricle (RV) and ultimately to RV hypertrophy. METHODS: A group of forty-two HLHS patients with a median age of 4.9 (2.9-17.0) years, at NYHA I was studied along with a control group of eleven subjects with healthy hearts and a median age of 12.1 (4.0-41.6). All subjects underwent MRI of the thoracic aorta including ECG-gated 2D balanced SSFP cine for an axial slice stack and 4D-flow MRI for a sagittal volume slab covering the thoracic aorta. The twist of the neoaortic arch was quantified by the effective geometric torsion, defined as the product of curvature and geometric torsion. Fluid dynamics and geometry in the neoaorta, including the flow helicity index, were evaluated using an in-house analysis software (MeVisLab-based). Myocardial mass of the systemic ventricle at end-diastole was estimated by planimetry of the short-axis stack. RESULTS: Compared to the control group, the neoaorta in the HLHS patients shows an increased twist (P=0.04) and higher peak helicity density (P=0.03). The maximum helicity density was correlated with maximum effective torsion of the ascending neoaorta (P<0.001). The degree of maximum twist correlated with the increase in RV myocardial mass (P<0.01). CONCLUSIONS: This study shows that the abnormal twist of the neoaortic arch in HLHS patients is associated with abnormal helical flow patterns, which may contribute to increased RV afterload and may adversely affect the systemic RV by stimulation of myocardial hypertrophy. These findings suggest that further improvements of surgical aortic reconstruction, guided by insights from 4D-flow MRI, could lead to better neoaortic fluid dynamics in patients with HLHS.

Advanced mapping strategies for ablation therapy in adults with congenital heart disease.

BACKGROUND: Ultra-high density mapping (HDM) is a promising tool in the treatment of patients with complex arrhythmias. In adults with congenital heart disease (CHD), rhythm disorders are among the most common complications but catheter ablation can be challenging due to heterogenous anatomy and complex arrhythmogenic substrates. Here, we describe our initial experience using HDM in conjunction with novel automated annotation algorithms in patients with moderate to great CHD complexity. METHODS: We studied a series of consecutive adult patients with moderate to great CHD complexity and an indication for catheter ablation due to symptomatic arrhythmia. HDM was conducted using the Rhythmia™ mapping system and a 64-electrode mini-basket catheter for identification of anatomy, voltage, activation pattern and critical areas of arrhythmia for ablation guidance. To investigate novel advanced mapping strategies, postprocedural signal processing using the Lumipoint™ software was applied. RESULTS: In 19 patients (53±3 years; 53% male), 21 consecutive ablation procedures were conducted. Procedures included ablation of atrial fibrillation (n=7; 33%), atrial tachycardia (n=11; 52%), atrioventricular accessory pathway (n=1; 5%), the atrioventricular node (n=1; 5%) and ventricular arrhythmias (n=4; 19%). A total of 23 supraventricular and 8 ventricular arrhythmias were studied with the generation of 56 complete high density maps (atrial n=43; ventricular n=11, coronary sinus n=2) and an average of 12,043±1,679 mapping points. Multiple arrhythmias were observed in n=7 procedures (33% of procedures; range of arrhythmias detected 2-4). A total range of 1-4 critical areas were defined per procedure and treated within a radiofrequency application time of 16 (interquartile range 12-45) minutes. Postprocedural signal processing using Lumipoint™ allowed rapid annotation of fractionated signals within specific windows of interest. This supported identification of a practical critical isthmus in 20 out of 27 completed atrial and ventricular tachycardia activation maps. CONCLUSIONS: Our findings suggest that HDM in conjunction with novel automated annotation algorithms provides detailed insights into arrhythmia mechanisms and might facilitate tailored catheter ablation in patients with moderate to great CHD complexity.

Artificial intelligence in pediatric and adult congenital cardiac MRI: an unmet clinical need.

Cardiac MRI (CMR) allows non-invasive, non-ionizing assessment of cardiac function and anatomy in patients with congenital heart disease (CHD). The utility of CMR as a non-invasive imaging tool for evaluation of CHD have been growing exponentially over the past decade. The algorithms based on artificial intelligence (AI), and in particular, deep learning, have rapidly become a methodology of choice for analyzing CMR. A wide range of applications for AI have been developed to tackle challenges in various aspects of CMR, and significant advances have also been made from image acquisition to image analysis and diagnosis. We include an overview of AI definitions, different architectures, and details on well-known methods. This paper reviews the major deep learning concepts used for analyses of patients with CHD. In the end, we have summarized a list of open challenges and concerns to be considered for future studies.

Pregnancy in adults with congenital heart disease.

Congenital heart disease (CHD) is present in 0.8-0.9% of live births. Prevalence of CHD is constantly increasing during the last decades in line with the treatment options for patients ranging from the surgical as well to the interventional spectrum. Most of the women with underlying CHD reach adulthood due to excellent surgical and interventional treatment options and most of them desire pregnancy. In Western countries, maternal heart disease is the major cause of mortality during pregnancy, thus risk estimation should be therefore individualized depending on the underlying CHD, functional class and treatment options. Pre-pregnancy counselling is mandatory in all women, especially in patients with high risk but even in patients with low risk, a plan for labor and delivery should be outlined in a heart pregnancy team. This review addresses the key element of pre-counselling, planning and successful disease management in patients with CHD during pregnancy.

Publisher Correction: Heart beat but not respiration is the main driving force of the systemic venous return in the Fontan circulation.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Determinants of Left Ventricular Dysfunction and Remodeling in Patients With Corrected Tetralogy of Fallot.

Background The aim of this study was to identify in asymptomatic patients with repaired tetralogy of Fallot the prevalence and determinants of impaired left-sided cardiac function and adverse ventricular remodeling and the relation of left ventricular (LV) dysfunction and remodeling with cardiopulmonary exercise capacity. Methods and Results In a cross-sectional study, 103 patients with tetralogy of Fallot (median age, 16.3 years) in New York Heart Association class 1, with surgical repair at a median age of 1.1 years, and 63 age-matched controls were studied. LV, right ventricular function and geometry, LV myocardial extracellular volume (n=57), and left atrial function were quantified with cardiac magnetic resonance. Peak oxygen consumption was measured by a standardized cardiopulmonary exercise test (n=70). Patients with tetralogy of Fallot had lower LV ejection fraction (P=0.001; 49% below age-adjusted fifth percentile for controls), lower LV mass index (P=0.003), lower LV mass/volume ratio (P<0.01), and impaired left atrial function. Right ventricular mass/volume ratio was the best predictor for LV systolic dysfunction and for a lower LV mass/volume ratio. Compared with controls, LV extracellular volume was higher (P<0.001), particularly in female patients, and associated with subnormal peak oxygen consumption (P=0.037). A peak oxygen consumption below the third percentile reference level was more likely with decreasing LV ejection fraction (P=0.008), and lower LV mass index (P=0.024), but independent of right ventricular ejection fraction. Conclusions In New York Heart Association class 1 patients with tetralogy of Fallot, frequent impaired systolic and diastolic LV function, LV adverse remodeling with LV atrophy, a decreased mass/volume ratio, and extracellular matrix expansion suggest cardiomyopathic changes. The best predictor for LV systolic dysfunction was the right ventricular mass/volume ratio. The subnormal peak oxygen consumption indicates that monitoring of LV status may be important for long-term prognosis.