Long QT syndrome in children and adolescents: risk factors and outcomes in a large German cohort.

Background And Aims: This study describes outcomes in a large contemporary national cohort of children and adolescents with long QT syndrome (LQTS).

Methods: Data analysis of 548 paediatric LQTS patients at 12 German tertiary care centres. Primary outcome was the occurrence of a major arrhythmic event (MAE) defined as sudden cardiac death (SCD), aborted cardiac arrest (ACA) and appropriate implantable cardioverter-defibrillator (ICD) therapy before age 18 years.

Imaging and evaluation of cervicothoracic lymphatic drainage pathways in single ventricle patients with Fontan circulation using the mDixon steady state MR angiography.

Background: Single ventricle (SV) Fontan patients are at risk for the development of pathological cervicothoracic lymphatic drainage pathways that are involved in the development of serious conditions such as plastic bronchitis or chylothorax. Visualization and categorization of cervicothoracic lymphatic drainage pathways might therefore help to stratify prognosis and to individualize therapy and follow-up for Fontan patients. This study aimed to show that the 3-dimensional (3D) modified Dixon (mDixon) steady state magnetic resonance (MR) angiography, commonly used to image cardiovascular anatomy, can visualize cervicothoracic lymphatic drainage pathways in high resolution in Fontan patients.

MRI Assessment of Energy Loss Within the Thoracic Aorta and Its Impact on Cardiac Function in Fontan Patients After Aortic Reconstruction.

Background: In Fontan patients undergoing aortic reconstruction, concerns regarding the impact of aortic function on ventricular function exist.

Purpose: 4D Flow MRI was used to compare energy loss (EL) within the thoracic aorta in patients with and without aortic reconstruction.

Study Type: Retrospective case control.

Four-Dimensional Flow in Fontan Patients: Advanced Haemodynamic Assessment.

Staged palliation with the creation of a Fontan circulation is the standard surgical approach in patients with a single ventricle. The Fontan circulation is a complex circuit that is associated with various complications that may present early or later in life and can limit life quality and expectancy. In this context, a good understanding of the Fontan physiology is important to improve outcomes for single-ventricle patients.

Endovascular treatment of a giant arteria lusoria aneurysm in a young female with Marfan syndrome.

Aneurysms of an aberrant right subclavian artery (ARSA) are rare but constitute a potentially lethal condition, especially with concomitant Marfan syndrome (MFS). A 27-year-old female with confirmed MFS presented with a relevant progression of a known aneurysm of an ARSA in MRI. The patient had undergone valve-sparing aortic root replacement (David procedure) 4 months prior.

Liver T1 mapping in Fontan patients and patients with biventricular congenital heart disease - insights into the effects of venous congestions on diffuse liver disease.

T1 relaxation time quantification on parametric maps is routinely used in cardiac imaging and may serve as a non-invasive biomarker for diffuse liver disease. In this study, we aimed to investigate the relationship between liver T1 values and cardiac function in patients with congenital heart disease (CHD) and compared patients with a biventricular circulation (BVC) to those with a Fontan circulation (FC). Magnetic resonance images from patients with CHD, obtained between June and December 2023 on a 1.

Management of a pregnant woman with Marfan syndrome and aortic root and aberrant right subclavian artery aneurysm: a case report.

Background: Marfan syndrome is a genetic connective tissue disorder that commonly affects the cardiovascular, skeletal, and ocular system. The increased risk of developing thoracic aortic aneurysms that can lead to aortic dissection and rupture is the main source of mortality in these patients. Pregnancy-induced changes can further increase the risk for aortic complications, especially in patients with an aortic root diameter > 45 mm.

Pediatric reference values for myocardial contraction fraction and global function index of the left ventricle: A cardiovascular magnetic resonance study.

Background: Cardiovascular magnetic resonance (CMR) derived global function index (GFI) and myocardial contraction fraction (MCF) were identified as useful imaging markers to assess left ventricular (LV) cardiac performance and can provide prognostic information for several cardiac diseases. As pediatric reference values are lacking, the aim of this retrospective study was to establish these values.

Methods: 154 CMR examinations of healthy children and adolescents (4-18 years) were included.

Quantifying and visualizing abdominal hemodynamics in patients with Fontan circulation by 4D phase-contrast flow magnetic resonance imaging at 1.5 T.

Background: Liver fibrosis has been recognized as a long-term morbidity associated with Fontan circulation (Fontan-associated liver disease, FALD). The pathophysiology of FALD is not completely understood and abnormal flow dynamics may be associated with this condition. Liver hemodynamics can be quantitatively evaluated with four-dimensional phase-contrast flow magnetic resonance imaging (4D PC flow MRI).

[Pulmonary hypertension in adults with congenital heart disease in light of the 2022-ESC-PAH guidelines - Part II: Supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesions, left ventricular disorders, univentricular hearts), interventions, intensive care, follow-up, future perspectives].

The number of adults with congenital heart defects (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary hypertension (PH), which may develop early in untreated CHD. Despite timely treatment of CHD, PH not infrequently persists or recurs in older age and is associated with significant morbidity and mortality.

Cardiovascular magnetic resonance reference values of right ventricular volumetric variables in patients with hypoplastic left heart syndrome.

Background: Cardiovascular magnetic resonance (CMR) has established itself as the gold standard for serial assessment of systemic right ventricular (RV) performance but due to the lack of standardized RV reference values for hypoplastic left heart syndrome (HLHS) patients, the interpretation of RV volumetric data in HLHS remains difficult. Therefore, this study aimed to close this gap by providing CMR reference values for the systemic RV in HLHS patients.

Methods: CMR scans of 160 children, adolescents, and young adults (age range 2.

Detection of hypoplastic left heart syndrome anatomy from cardiovascular magnetic resonance images using machine learning.

Objective: The prospect of being able to gain relevant information from cardiovascular magnetic resonance (CMR) image analysis automatically opens up new potential to assist the evaluating physician. For machine-learning-based classification of complex congenital heart disease, only few studies have used CMR.

Materials And Methods: This study presents a tailor-made neural network architecture for detection of 7 distinctive anatomic landmarks in CMR images of patients with hypoplastic left heart syndrome (HLHS) in Fontan circulation or healthy controls and demonstrates the potential of the spatial arrangement of the landmarks to identify HLHS.

[Pulmonary arterial hypertension in congenital heart disease - Part 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.

Pulmonary valve reconstruction for acquired pulmonary regurgitation in patients with treated congenital heart disease.

Objectives: Pulmonary valve regurgitation is a common problem after relief of right ventricular outflow tract (RVOT) obstruction with a transannular patch. Pulmonary valve replacement with a homograft or xenograft is the routine treatment. Longevity of biological valves and the availability of homografts are limited.

Genetic Screening Reveals Heterogeneous Clinical Phenotypes in Patients with Dilated Cardiomyopathy and Troponin T2 Variants.

Background: Cardiomyopathies (CMs) are a heterogeneous and severe group of diseases that shows a highly variable cardiac phenotype and an incidence of app. 1/100.000.

Pediatric Cardiac Magnetic Resonance Reference Values for Biventricular Volumes Derived From Different Contouring Techniques.

Background: Measurement of ventricular volumes and function using MRI is an important tool in pediatric congenital heart disease. However, normal values for children are sparce and analysis methods are inconsistent.

Purpose: To propose biventricular reference values in children for two MRI postprocessing (contouring) techniques.

Reference Values for Pediatric Atrial Volumes Assessed by Steady-State Free-Precession Magnetic Resonance Imaging Using Monoplane and Biplane Area-Length Methods.

Background: Measurement of atrial volumes by MRI is becoming increasingly important in pediatric cardiac disorders. However, MRI normal values for atrial volumes in children are lacking.

Purpose: To establish pediatric reference values for atrial volumes.

Reference Values for Wristband Accelerometry Data in Children Aged 6-11 Years of Age.

Objectives: Wristband activity trackers (accelerometers) could serve as a convenient monitoring tool to continuously quantify physical activity throughout the day. We aim to provide reference values for the use of these devices in healthy children.

Methods: Children were recruited at a local school and provided with activity trackers (Fitbit Charge 2).

Serial Assessment of Right Ventricular Deformation in Patients With Hypoplastic Left Heart Syndrome: A Cardiovascular Magnetic Resonance Feature Tracking Study.

Background As right ventricular dysfunction is a major cause of adverse outcome in patients with hypoplastic left heart syndrome, the aim was to assess right ventricular function and deformation after Fontan completion by performing 2-dimensional cardiovascular magnetic resonance feature tracking in serial cardiovascular magnetic resonance studies. Methods and Results Cardiovascular magnetic resonance examinations of 108 patients with hypoplastic left heart syndrome (female: 31) were analyzed. Short-axis cine images were used for right ventricular volumetry.