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Importance: The ability to predict sudden cardiac death (SCD) in children and adolescents with hypertrophic cardiomyopathy (HCM) is currently inadequate. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance (CMR) imaging is associated with SCD events in adults with HCM., Objective: To examine the prognostic significance of LGE in patients with HCM who are younger than 21 years., Design, Setting, and Participants: This multicenter, retrospective cohort study was conducted from April 8, 2015, to September 12, 2022, in patients with HCM who were younger than 21 years and had undergone CMR imaging across multiple sites in the US, Europe, and South America. Observers of CMR studies were masked toward outcomes and demographic characteristics., Exposure: Natural history of HCM., Main Outcome and Measures: The primary outcome was SCD and surrogate events, including resuscitated cardiac arrest and appropriate discharges from an implantable defibrillator. Continuous and categorical data are expressed as mean (SD), median (IQR), or number (percentage), respectively. Survivor curves comparing patients with and without LGE were constructed by the Kaplan-Meier method, and likelihood of subsequent clinical events was further evaluated using univariate and multivariable Cox proportional hazards models., Results: Among 700 patients from 37 international centers, median (IQR) age was 14.8 (11.9-17.4) years, and 518 participants (74.0%) were male. During a median (IQR) [range] follow-up period of 1.9 (0.5-4.1) [0.1-14.8] years, 35 patients (5.0%) experienced SCD or equivalent events. LGE was present in 230 patients (32.9%), which constituted an mean (SD) burden of 5.9% (7.3%) of left ventricular myocardium. The LGE amount was higher in older patients and those with greater left ventricular mass and maximal wall thickness; patients with LGE had lower left ventricular ejection fractions and larger left atrial diameters. The presence and burden of LGE was associated with SCD, even after correcting for existing risk stratification tools. Patients with 10% or more LGE, relative to total myocardium, had a higher risk of SCD (unadjusted hazard ratio [HR], 2.19; 95% CI, 1.59-3.02; P < .001). Furthermore, the addition of LGE burden improved the performance of the HCM Risk-Kids score (before LGE addition: 0.66; 95% CI, 0.58-0.75; after LGE addition: 0.73; 95% CI, 0.66-0.81) and Precision Medicine in Cardiomyopathy score (before LGE addition: 0.68; 95% CI, 0.49-0.77; after LGE addition: 0.73; 95% CI, 0.64-0.82) SCD predictive models., Conclusions and Relevance: In this retrospective cohort study, quantitative LGE was a risk factor for SCD in patients younger than 21 years with HCM and improved risk stratification.

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., (© 2024. The Author(s).)

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., Competing Interests: The authors declare no competing interests. SZ is an employee of Philips but had no control of the data. No financial support for this study was provided by industry. This does not alter our adherence to the PLOS ONE policies on sharing data and materials., (Copyright: © 2024 Ristow et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

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., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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, I 2 =9.49%). Six (odds ratio [OR]: 1%; 95% CI, 0.00%-0.02%; p=0.002, I 2 =0%) ruptures and 10 dissections (OR: 2%; 95% CI, 0.001%-0.02%; p<0.001, I 2 =0%) were reported. The intra-operative and 30-day mortality were 1% (95% CI, 0.00%-0.02%; p=0.003, I 2 =0%) and 1% (95% CI, 0.00%-0.02%; p=0.004, I 2 =0%), respectively. The median follow-up was 29 months. Sixty-eight re-interventions (OR: 8%; 95% CI, 0.05%-0.10%; p<0.001, I 2 =35.99%) were performed; 95.5% were endovascular. Seven deaths were reported (OR: 2%; 95% CI, 0.00%-0.03%; p=0.008, I 2 =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.

Background Meta-analysis can identify biological factors that moderate cardiac magnetic resonance myocardial tissue markers such as native T 1 (longitudinal magnetization relaxation time constant) and T 2 (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 T 1 , T 2 mapping, extracellular volume, and late gadolinium enhancement were identified by database searches. Pooled effect sizes and interstudy heterogeneity (I 2 ) were estimated with random effects models. Moderators of interstudy heterogeneity were analyzed by meta-regression of the percent difference of native T 1 and T 2 between COVID-19 and control groups (%ΔT 1 [percent difference of the study-level means of myocardial T 1 in patients with COVID-19 and controls] and %ΔT 2 [percent difference of the study-level means of myocardial T 2 in patients with COVID-19 and controls]), extracellular volume, and the proportion of late gadolinium enhancement. Interstudy heterogeneities of %ΔT 1 (I 2 =76%) and %ΔT 2 (I 2 =88%) were significantly lower than for native T 1 and T 2 , respectively, independent of field strength, with pooled effect sizes of %ΔT 1 =1.24% (95% CI, 0.54%-1.9%) and %ΔT 2 =3.77% (95% CI, 1.79%-5.79%). %ΔT 1 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 %ΔT 1 and/or %ΔT 2 . 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 T 1 and T 2 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.

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., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cdt.amegroups.com/article/view/10.21037/cdt-22-230/coif). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V” was commissioned by the editorial office without any funding or sponsorship. YVK, HK and KN served as the unpaid Guest Editors of the series. HK has received honoraria for lectures and/or consultancy from Actelion, Bristol Myers Squibb, Janssen. CS receives research funding from the German Foundation for Heart Research and the Dr. Rolf Schwiete Stiftung. Further, he received speaker fees from AstraZeneca and Johnson & Johnson outside the submitted work. EZ reports speaker fees received from AstraZeneca outside the submitted work. CM reports research funding from the German Center for Cardiovascular Research (DZHK) within the Promotion of women scientist program, the Deutsche Stiftung fuer Herzforschung and the Dr. Rolf Schwiete Stiftung, and speaker fees from AstraZeneca, Novartis, Heinen&Loewenstein, Boehringer Ingelheim/Lilly, Bayer, Pfizer, Sanofi, Aventis, Apontis, Abbott outside of this study. SB reports grants and personal fees from Abbott Diagnostics, Bayer, Thermo Fisher, grants from SIEMENS, Singulex, personal fees from Abott, Astra Zeneca, AMGEN, Medtronic, Pfizer, Roche, Novartis, Siemens Diagnostics, outside the submitted work. PK receives research support for basic, translational, and clinical research projects from European Union, British Heart Foundation, Leducq Foundation, Medical Research Council (UK), and German Centre for Cardiovascular Research, from several drug and device companies active in atrial fibrillation, and has received honoraria from several such companies in the past, but not in the last three years. PK is listed as inventor on two patents held by University of Birmingham (Atrial Fibrillation Therapy WO 2015140571, Markers for Atrial Fibrillation WO 2016012783). HR is a consultant to Medtronic Inc. and received speaker- and travel honoraria from Abiomed Inc. and Edwards Inc. The authors have no other conflicts of interest to declare., (2022 Cardiovascular Diagnosis and Therapy. All rights reserved.)

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., Competing Interests: Conflict of Interest None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ ).)

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., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-650). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. YvK serves as an unpaid editorial board member of Cardiovascular Diagnosis and Therapy from February 2018 to January 2022 and served as the unpaid Guest Editor of the series. Dr. SB reports grants and personal fees from Abbott Diagnostics, grants and personal fees from Bayer, grants from SIEMENS, grants from Singulex, grants and personal fees from Thermo Fisher, personal fees from Abott, personal fees from Astra Zeneca, personal fees from AMGEN, personal fees from Medtronic, personal fees from Pfizer, personal fees from Roche, personal fees from Novartis, personal fees from Siemens Diagnostics, outside the submitted work; Dr. PK reports non-financial support from the European Union, British Heart Foundation, Leducq Foundation, Medical Research Council (UK), and German Centre for Heart Research, outside the submitted work; In addition, Dr. PK is listed as inventor on two patents held by University of Birmingham (Atrial Fibrillation Therapy WO 2015140571, Markers for Atrial Fibrillation WO 2016012783). He received research support for basic, translational and clinical research projects from several drug and device companies active in atrial fibrillation, and has received honoraria from several such companies in the past, but not in the last 3 years. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

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., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-770). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. Dr. Kheradvar reports grants from Humboldt Foundation, during the conduct of the study. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

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., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-767). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. DDG reports having a patent application 18752420.2 - 1010 pending. AK reports grants from Alexander von Humboldt Foundation, during the conduct of the study. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

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., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-631). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. SB reports grants and personal fees from Abbott Diagnostics, grants and personal fees from Bayer, grants from SIEMENS, grants from Singulex, grants and personal fees from Thermo Fisher, personal fees from Abott, personal fees from Astra Zeneca, personal fees from AMGEN, personal fees from Medtronic, personal fees from Pfizer, personal fees from Roche, personal fees from Novartis, personal fees from Siemens Diagnostics, outside the submitted work. PK reports non-financial support from European Union, non-financial support from British Heart Foundation, non-financial support from Leducq Foundation, non-financial support from Medical Research Council (UK), non-financial support from German Centre for Cardiovascular Research, outside the submitted work; In addition, Dr. Kirchhof has a patent WO 2015140571 issued, and a patent WO 2016012783 issued and research support for basic, translational, and clinical research projects from several drug and device companies active in atrial fibrillation, and has received honoraria from several such companies in the past, but not in the last three years. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

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., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-771). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

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., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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.

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., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-632). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part III” was commissioned by the editorial office without any funding or sponsorship. YVK served as the unpaid Guest Editor of the series and an unpaid editorial board member of Cardiovascular Diagnosis and Therapy from Feb 2018 to Jan 2020. Dr. Blankenberg reports grants and personal fees from Abbott Diagnostics, grants and personal fees from Bayer, grants from SIEMENS, grants from Singulex, grants and personal fees from Thermo Fisher, personal fees from Abott, personal fees from Astra Zeneca, personal fees from AMGEN, personal fees from Medtronic, personal fees from Pfizer, personal fees from Roche, personal fees from Novartis, personal fees from Siemens Diagnostics, outside the submitted work. Dr. Kirchhof reports non-financial support from European Union, non-financial support from British Heart Foundation, non-financial support from Leducq Foundation, non-financial support from Medical Research Council (UK), non-financial support from German Centre for Cardiovascular Research, outside the submitted work; In addition, Dr. Kirchhof has a patent WO 2015140571 issued, and a patent WO 2016012783 issued and research support for basic, translational, and clinical research projects from several drug and device companies active in atrial fibrillation, and has received honoraria from several such companies in the past, but not in the last three years. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Various forms of vortex formation in the cardiovascular system convey valuable information regarding the function of heart and great vessels. The vortex ring that forms during systole in the aortic sinus is the first that was recognized and the asymmetric transmitral vortex ring that forms in the left ventricle during diastole has been most commonly used for diagnosis and follow up of heart failure patients. Adverse vortex interaction in the heart can also occur due to valvular regurgitation and may have energetic consequences to the heart. Furthermore, vortices do exist in other chambers such as the right ventricle and may even arise in the great arteries and veins due to congenital heart disease. Here, we summarize diagnostic and prognostic significance of vortices and vortex imaging in the heart, their applications in clinical medicine, and discuss how these flow features can be used to assess functional status of the heart., (Copyright © 2019 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

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., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare., (2019 Cardiovascular Diagnosis and Therapy. All rights reserved.)

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., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare., (2019 Cardiovascular Diagnosis and Therapy. All rights reserved.)

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., Competing Interests: Conflicts of Interest: C Meyer: speaker for Boston Scientific and Abbott; consultant for Biosense Webster. The other authors have no conflicts of interest to declare., (2019 Cardiovascular Diagnosis and Therapy. All rights reserved.)