Transcatheter intervention of modified Blalock-Taussig shunts in patients with hypoplastic left heart syndrome undergoing stage 1 palliation.

Background: While several studies have explored the outcomes of transcatheter interventions for modified Blalock-Taussig shunts (MBTSs) in a broad range of congenital heart diseases, none have specifically examined the interventions in patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood palliation (NP).

Methods: This retrospective study was conducted between 2020 and 2024, when 24 urgent interventions were performed on 17 patients at our center. We recorded several key outcomes, including early and late intervention-related complications, the need for reintervention, the interval between the NP and the first intervention, shunt patency following the intervention, associated morbidities, and thrombosis-related sudden events.

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.

Duct Stenting vs. Modified Blalock-Taussig Shunt: New Insights Learned From High-Risk Patients With Duct-Dependent Pulmonary Circulation.

Background: As no data were available on the comparison of outcomes between modified Blalock-Taussig shunts (MBTs) vs. duct-stenting (DS) in patients with pulmonary atresia (PA) and an increased ductal tortuosity and in patients with pulmonary atresia and intact septum (PA-IVS) with right ventricle-dependent coronary circulation (RVDCC), we aimed to perform a single-center retrospective evaluation.

Methods: Between 2010 and 2019, 127 patients with duct-dependent pulmonary circulation (DDPC) underwent either MBTs (without additional repairs) ( = 56) or DS ( = 71).

German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease: Report 2020-Comprehensive Data from 6 Years of Experience.

Background: Based on a quality assurance initiative of the German Society for Thoracic and Cardiovascular Surgery (DGTHG) and the German Society for Pediatric Cardiology and Congenital Heart Defects (DGPK), a voluntary registry was founded for assessment of treatment and outcomes of patients with congenital heart disease in Germany. This evaluation by the German Registry for cardiac operations and interventions in patients with congenital heart disease reports the data and the outcome over a 6-year period in patients undergoing invasive treatment.

Methods: This real-world database collects clinical characteristics, in-hospital complications, and medium-term outcome of patients who underwent cardiac surgical and interventional procedures within the prospective, all-comers registry.

Repair of Complex Transposition of Great Arteries: Up to 30 Years of Follow-up.

Background: To characterize treatment of transposition of great arteries with ventricular septal defect and left ventricular outflow tract obstruction (LVOTO) in Germany and to analyze late outcomes.

Methods: German Registry for Congenital Heart Defects data were searched for transposition of great arteries with ventricular septal defect and LVOTO. One hundred thirty-nine patients were treated at 15 institutions between 1968 and 2016.

Elastica degeneration and intimal hyperplasia lead to Contegra® conduit failure.

Objectives: Currently, Contegra® grafts (processed bovine jugular vein conduits) are widely used for reconstructive surgery of the right ventricular outflow tract in patients with congenital heart disease (CHD). We analysed explanted Contegra conduits from 2 institutions histologically to get a possible hint at the underlying pathomechanisms of degenerative alterations and to find histological correlations of graft failure. Additionally, we compared the explants with a non-implanted processed graft and a native jugular vein obtained from a young bull.

A new way to determine correct depth of central venous catheter insertion using a real-time ultrasound-guided insertion technique in pediatric patients.

Background: Several formulae or methods are reported to predict the ideal central venous catheter insertion depth. However, they are complicated and often unsuitable in cases requiring rapid management.

Aims: This study aimed to determine a simple and practical method to predict the ideal central venous catheter insertion depth after the real-time ultrasound-guided right internal jugular vein, or left or right supraclavicular approach in pediatric patients.

Aortic stenosis of the neonate: A single-center experience.

Objectives: Because data for neonates are limited, optimal management of critical aortic stenosis remains controversial (balloon valvotomy [BV] or open valvoplasty [OV]). In a center with balanced experience in both methods, we hypothesized that OV can provide a better individualized approach than blunt BV and better serve long-term outcomes.

Methods: A retrospective review of data and follow-up (survival, freedom from operation/replacement) of all neonates, suitable for biventricular repair, undergoing aortic valve procedure (1989-2015), was performed.

Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study.

Objectives: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS).

Methods: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations.

Contemporary results of aortic valve repair for congenital disease: lessons for management and staged strategy.

Objectives: Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease.

Are adults with congenital heart disease informed about their risk for infective endocarditis and treated in accordance to current guidelines?

Background: Adults with congenital heart disease (ACHD) have an increased risk for infective endocarditis (IE). In the last decade, the recommendations for IE prophylaxis have changed substantially. The knowledge level of patients about IE and IE prophylaxis has not been studied.

Multicenter midterm follow-up results using the gore septal occluder for atrial septal defect closure in pediatric patients.

Objectives: To assess the safety and efficacy of the Gore Septal Occluder (GSO) used for device-closure of significant secundum-type atrial septal defects (ASD II) focusing on pediatric patients.

Background: The GSO is a patch-like double disc device. Due to its design, it is assumed to be safe, even when implanted in ASDs with deficient retro-aortic rims.

Bilateral Congenital Iris Sphincter Agenesis Diagnosed After Massive Bleeding Episode During Repair of Aneurysmal Dilation of Patent Ductus Arteriosus: A Case Report.

We report a rare case of an infant with both an aneurysmal dilation of the patent ductus arteriosus (PDA) and bilateral congenital iris sphincter agenesis. Her mydriasis without pupillary light reflex was first noted after a massive intraoperative bleeding episode during the PDA ligation. The assumption that the mydriasis was a sign of cerebral ischemia led to additional examinations and intensive medical therapies that in retrospect were unnecessary.

Reconstruction of a Previously Repaired Aortic Valve Destroyed by Infective Endocarditis: Case Report.

Infective endocarditis (IE) is an uncommon condition among patients with congenital heart disease, however it can be life threatening. The usual management includes replacement of the affected valve, especially in patients with aortic valve compromise, and is even more common in previously repaired valves. In this case report, we describe the successful reconstruction of an aortic root destroyed by IE, in a patient with history of ballooning of a congenital aortic stenosis.

Current mid-term outcome with an integrated surgical strategy for correction of d-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction.

Objectives: The optimal surgical treatment strategy for transposition of the great arteries (TGA) associated with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) leading to the best long-term outcome has not been established yet. The wide range of anatomical variability has led to the development of different surgical treatment options. In a retrospective single-centre review, we aimed to report the long-term outcome in patients who underwent biventricular repair.

Three parties, one direction: Research priorities in adults with congenital heart disease. What do professionals, patients and relatives want to know?

Background: Patients, their relatives, as well as medical specialists from the fields of paediatric cardiology, cardiology and cardiac surgery were surveyed to investigate the current research needs in the field of congenital heart disease (CHD) focussing specifically on the needs of those affected.

Material And Methods: An online survey including four groups of patients with CHD (Fontan circulation, transposition of the great arteries [TGA] after atrial switch [AS] and after arterial switch operation [ASO], tetralogy of Fallot [TOF]) was performed. Each questionnaire comprised twelve topics.

Expanding the Limits of Posterior Aortic Translocation: Biventricular Correction of Complex Transposition With Complete Atrioventricular Septal Defect and Heterotaxy.

This case report describes successful repair of d-transposition of the great arteries with severe left ventricular outflow tract obstruction and complete atrioventricular septal defect associated with heterotaxy by the use of posterior aortic translocation combined with repair of the atrioventricular septal defect and systemic venous anomalies.

Enhanced left ventricular training in corrected transposition of the great arteries by increasing the preload.

Objectives: Patients with congenitally corrected transposition of the great arteries (ccTGA) without ventricular septal defect or left ventricular outflow tract obstruction need training of the morphological left ventricle (mLV) to undergo a double switch operation (DS) (atrial plus arterial switch). Establishing a sufficient gradient by pulmonary artery banding (PAB) is difficult and multiple re-pulmonary artery bandings (re-PABs) may be necessary to achieve the required physiology. We evaluate a new approach, consisting of a loose PAB in combination with an artificial atrial septal defect (aASD) to not only increase afterload but to dynamically enhance preload as well, in terms of the effectiveness of training of the mLV and the subsequent functional outcome after a DS.

A Case of Acute Upper Airway Obstruction in a Pediatric Hemophilia A Patient Because of Spontaneous Retropharyngeal Hemorrhage.

We report a rare case of acute upper airway obstruction caused by spontaneous retropharyngeal hemorrhage as a result of hemophilia A in a 16-year-old pediatric patient who routinely received factor VIII replacement. Initial diagnosis was delayed because the patient presented with symptoms, such as throat pain and odynophagia, similar to those of common benign upper airway infections. Within 2 days of the initial presentation of symptoms, the patient went into respiratory failure as a result of retropharyngeal hemorrhage.

Prolonged Activated Clotting Time after Protamine Administration Does Not Indicate Residual Heparinization after Cardiopulmonary Bypass in Pediatric Open Heart Surgery.

Background: In open heart surgery, heparinization is commonly neutralized using an empirical heparin:protamine ratio ranging between 1:1 and 1:1.5. However, these ratios may result in protamine overdose that should be avoided for its negative side effects on the coagulation system.

Decompression of thoracic duct: new approach for the treatment of failing Fontan.

In failing Fontan circulation, the elevated central venous pressure increases lymphatic production and simultaneously retards lymphatic return to the central venous system, thus unbalancing lymphatic homoeostasis. To improve lymphatic drainage, a new concept based on decompression of the thoracic duct to the lower pressure levels of the common atrium, with concomitant increase of preload, has been developed. The thoracic duct, which in the majority of patients enters the circulation at the left subclavia-jugular junction, is decompressed by diverting the innominate vein directly to the common atrium.