German Registry for Cardiac Operations and Interventions in Congenital Heart Disease: Annual Report 2022.

Background:  The German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease is a voluntary registry initiated by the German Society for Thoracic and Cardiovascular Surgery and the German Society for Pediatric Cardiology and Congenital Heart Defects. Since 2012, the registry collects data for the assessment of treatment and outcomes of surgical and interventional procedures in patients with congenital heart disease (CHD) of all age groups.

Methods:  This real-world, prospective all-comers registry collects clinical and procedural characteristics, adverse events (AEs), mortality, and medium-term outcomes (up to 90 days) of patients undergoing surgical and interventional.

Left Ventricular Physiology and Ventricular-Vascular Interactions in Young Patients After Heart Transplantation.

Background In patients after heart transplantation, systemic arterial hypertension and enhanced central aortic stiffness contribute to increased ventricular afterload, which might lead to graft dysfunction. The aim of our study was to characterize systemic arterial elastance and its impact on left ventricular function and ventriculo-arterial coupling in a cohort of children, adolescents, and young adults after heart transplantation using invasive conductance catheter technique. Methods and Results Thirty patients who had heart transplants (age, 20.

Functional regeneration of dilated cardiomyopathy by transcatheter bilateral pulmonary artery banding: first-in-human case series.

Background: Dilated cardiomyopathy (DCM) is a leading cause of heart transplantation (HTx) in children. Surgical pulmonary artery banding (PAB) is used worldwide to achieve functional heart regeneration and remodelling.

Case Summary: We report for the first-time successful bilateral transcatheter implantation of bilateral pulmonary artery flow restrictors in a case series of three infants with severe DCM based on left-ventricular non-compaction morphology associated with Barth syndrome in one and a non-classified syndrome in another.

Socio-demographic parameters and non-cardiac comorbidity related to self-perceived quality of life in young adults after neonatal arterial switch operation for transposition of the great arteries.

Evaluating the relation of non-cardiac comorbidity and socio-demographic factors to physical and mental health-related quality of life (QOL) which has been partially found at elevated risk in young adults after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA). In a prospective reassessment study, results of 92 unselected young adults (22.8 ± 2.

Anxiety and Depression in Adults With Congenital Heart Disease.

Introduction: Anxiety and depression can worsen outcome in patients with heart disease. We elucidate the prevalence of anxiety and depression in a cohort of adults with congenital heart disease (ACHD).

Materials And Methods: Prospective screening for anxiety or depression was performed in 204 consecutive patients of the outpatient clinic of our tertiary care center using the Hospital Anxiety and Depression Scale (HADS) questionnaire and the distress thermometer (DT) as a potential ultra-short screening test.

Rare Case of an Adult With Double-Chambered Left Ventricle.

The rare case of an adult with a double-chambered left ventricle was revealed using multimodality imaging using echocardiography and cardiac magnetic resonance imaging in a 38-year-old asymptomatic male patient. The congenital malformation was dominated by a second, coarsely trabeculated muscular shelf dividing the left ventricle into 2 chambers without signs for left ventricular inflow or outflow tract obstruction. The partition wall did not show any signs for intramyocardial fibrosis in late gadolinium enhancement cardiovascular magnetic resonance imaging.

NYHA class and cardiopulmonary exercise capacity impact self-rated health-related quality of life in young adults after arterial switch operation for transposition of the great arteries.

Background: Evaluating the impact of subjective physical activity status and cardiopulmonary exercise capacity on physical and mental health-related quality of life (QOL) in young adults after neonatal arterial switch operation for transposition of the great arteries.

Methods: In a prospective reassessment study, 92 young adults (age 22.8 ​± ​2.

Proximal Aortic Dilatation and Pulmonary Valve Replacement in Patients with Repaired Tetralogy of Fallot: Is There a Relationship? A Cardiac Magnetic Resonance Imaging Study.

Aortopathy is a known complication whose incidence is growing within the population of tetralogy of Fallot (TOF) patients. Its pathology and relationship with other comorbidities remain unclear. This study was designed to determine the prevalence and predictors of proximal aortic dilatation after TOF repair.

Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects.

Background: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.

Methods: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.

Cardiovascular Drug Therapy during Interstage After Hybrid Approach: A Single-Center Experience in 51 Newborns with Hypoplastic Left Heart.

Background: Newborns with hypoplastic left heart (HLH) are usually palliated with the Norwood procedure or a hybrid stage I procedure. Hybrid is our preferred approach. Given the critical relationship between stage I, interstage, and comprehensive stage II or advanced biventricular repair, we hypothesized that appropriate drug treatment is a significant therapeutic cornerstone, especially for the management of the high-risk interstage.

Acute myocardial infarction due to spontaneous coronary artery dissection in a 6-year-old boy with ADHD on the third day of treatment with methylphenidate.

Methylphenidate (MPH) is an indirect-acting sympathomimetic drug and structurally related to amphetamine. It is widely used to treat children aged 6 years and older, as well as adolescents who have attention-deficit/hyperactivity disorder (ADHD). We report on a 6-year-old boy who presented with typical angina symptoms occurring several hours after intake of an increased dose of MPH, which had been initiated for ADHD treatment 2 days earlier.

Fatal outcome after heart surgery in PMM2-CDG due to a rare homozygous gene variant with double effects.

Variants in Phosphomannomutase 2 (PMM2) lead to PMM2-CDG, the most frequent congenital disorder of glycosylation (CDG). We here describe the disease course of a ten-month old patient who presented with the classical PMM2-CDG symptoms as cerebellar hypoplasia, retinitis pigmentosa, seizures, short stature, hepato- and splenomegaly, anaemia, recurrent vomiting and inverted mamillae. A severe form of tetralogy of Fallot was diagnosed and corrective surgery was performed at the age of 10 months.

Pulmonary artery banding in dilative cardiomyopathy of young children: review and protocol based on the current knowledge.

Dilated cardiomyopathy (DCM) is a leading cause of cardiac death in children. Current therapeutic strategies are focused on improving symptoms of congestive heart failure (CHF); the potentials of cardiac regeneration especially in infants and young children are neglected in particular when DCM is classified as "end-stage". Heart transplantation (HTx) serves as the only life-saving option, despite is palliative character with limited survival time.

Percutaneous pulmonary valve implantation (PPVI) in non-obstructive right ventricular outflow tract: limitations and mid-term outcomes.

Background: Percutaneous pulmonary valve implantation (PPVI) has been established as a safe and effective alternative to surgery treating patients with a failing pulmonary valve conduit. Nevertheless, the majority of patients in need of a valve have a native, non-obstructive right ventricular outflow tract (RVOT). The current approved stent-valves have a balloon-expandable design.

Evaluation of pulmonary endothelial function in Fontan patients.

Objectives: Pulmonary endothelial dysfunction due to a loss of pulsatile pulmonary blood flow is thought to be a major contributor in the development of increased pulmonary vascular resistance (PVR) in patients with a Fontan circulation. We sought to evaluate pulmonary endothelial dysfunction by vasodilator response to acetylcholine in children and adolescents with Fontan hemodynamics.

Methods: Twenty-one Fontan patients with a median age of 10.

Hemodynamic Evaluation of Children with Persistent or Recurrent Pulmonary Arterial Hypertension Following Complete Repair of Congenital Heart Disease.

Persistent or recurrent pulmonary arterial hypertension (PAH) following complete surgical repair of congenital heart disease (CHD) represents one of the largest group of PAH associated with CHD (PAH-CHD) in recent registry studies and seems to have a particularly poor prognosis. However, little is known about this fourth clinical subclass of PAH-CHD, especially in children. The purpose of this study was to assess specific characteristics of invasive hemodynamics of this disease in children, including acute vasodilator testing (AVT) and pulmonary endothelial function (PEF) and to compare to patients with idiopathic PAH (IPAH), who usually present with a similar fatal clinical course.

Transcatheter Valve-in-Ring Implantation for the Treatment of Residual or Recurrent Tricuspid Valve Dysfunction After Prior Surgical Repair.

Objectives: This study sought to describe the results of transcatheter tricuspid valve-in-ring (TVIR) implantation for treatment of tricuspid regurgitation (TR).

Background: Off-label use of transcatheter valves within surgically placed tricuspid annuloplasty prostheses has only been described in small reports. An international multicenter registry was developed to collect data on TVIR implantation.

Internal Cardioverter Defibrillator Indications and Therapies after Atrial Baffle Procedure for d-Transposition of the Great Arteries: A Multicenter Analysis.

Background: Sudden cardiac death (SCD) is the most important cause of late mortality after atrial baffle procedure for d-transposition of the great arteries (d-TGA). Experience with internal cardioverter defibrillator (ICD) therapy in this population is limited. We conducted a multicenter cohort study to determine the current state of ICD therapy in individuals after atrial baffle procedure.

Effect of Inhaled Nitric Oxide on Blood Flow Dynamics in Patients After the Fontan Procedure Using Cardiovascular Magnetic Resonance Flow Measurements.

Invasive hemodynamic studies have shown that nitric oxide (NO), a selective pulmonary vasodilator, can lower pulmonary vascular resistance in Fontan patients. Because oximetry-derived flow quantification may be unreliable, we sought to detect changes in blood flow within the Fontan circulation after inhalation of NO using cardiovascular magnetic resonance (CMR). Thirty-three patients (mean age 12.

Creation of a transcatheter fenestration in children with failure of Fontan circulation: Focus on extracardiac conduit connection.

Objectives: We report our experience with a transcatheter technique to bypass the lung and to thus improve single-ventricle preload and reduce venous congestion in Fontan patients.

Background: In the absence of a dedicated power source to serve the pulmonary circulation and a significantly elevated transpulmonary pressure gradient, fenestration of the Fontan circulation is an option to improve hemodynamics in patients by relieving excessive systemic venous pressure.

Methods And Results: From 2005 to 2011, 22 transcatheter fenestrations were performed without any major complications in 19 patients (median age 3.

Transcatheter Closure of Perimembranous Ventricular Septal Defects with Left Ventricular to Right Atrial Shunt.

During the development of so-called aneurysmal transformation of perimembranous ventricular septal defects (pmVSD), tricuspid valve (TV) morphology and function may be altered resulting in left ventricular (LV) to right atrial (RA) shunting. The feasibility and outcome of interventional closure of these pmVSD has not been investigated so far. Interventional closure of pmVSD associated with mild to moderate LV-to-RA shunt was performed in four patients (aged 6.