Recent research in patients with functionally univentricular hearts (UVH) is focusing on pathologies of the lymphatic vessels. Morphology of the abdominal lymphatic vessels was analyzed by MRI in patients with UVH following total cavopulmonary connection (TCPC) and it was examined, if clinical and laboratory parameters correlate with changes after TCPC. We prospectively examined 33 patients at the age of 19.
View Article and Find Full Text PDFNeonatal aortic arch surgery remains one of the most challenging procedures in congenital cardiac surgery. In recent years, there has been a trend away from selective cerebral perfusion (SCP) and arrest of body circulation towards whole-body perfusion (WBP), a combination of SCP and lower-body perfusion (LBP), to facilitate arch surgery and preserve organ function. Retrospective, single-centre analysis was conducted of patients under one year of age undergoing aortic arch surgery from January 2014 until December 2022.
View Article and Find Full Text PDFBMC Cardiovasc Disord
March 2024
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.
View Article and Find Full Text PDFBackground: Endovascular treatment of aortic coarctation (CoA) in children and adults frequently requires stent implantation. The aim of this study was to analyze long-term results after CoA treatment with bare and covered Cheatham-Platinum (CP) stents in our institution and to derive recommendations for the differential use of these stent types.
Methods: In this retrospective single institution study, 212 patients received endovascular CoA treatment with bare (n = 71) and covered (n = 141) CP stents between September 1999 and July 2021, respectively.
Device thromboses after patent foramen ovale (PFO) and atrial septal defect (ASD) closure are rare but serious complications. They have been reported in different devices of virtually all manufacturers. Here, we report three cases of left atrial device thrombosis after atrial defect closure with the Gore® Cardioform septal occluder (GSO) in our recent institutional experience.
View Article and Find Full Text PDFBackground: Transcatheter correction of superior sinus venosus atrial septal defect (SVASD) with partial anomalous pulmonary venous drainage (PAPVD) of the right upper and/or right middle pulmonary vein (RUPV/RMPV) has recently been described as an alternative to surgical approach in a substantial number of patients. We describe a modified technical approach for transcatheter correction of SVASD using transoesophageal echocardiography (TOE) to confirm adequate stent landing zone and apposition and evaluate its feasibility, safety, and procedural success.
Case Summary: From 2019 to 2021, three consecutive patients received a transcatheter correction of SVASD with PAPVD by redirecting the superior vena cava and RUPV/RMPV to the left atrium by implantation of a custom-made covered Cheatham platinum stent (10-zig, length: 60-80 mm).
Background: Nowadays, transcatheter device closure of an atrial septal defect (ASD) is a standard approach in children. Potential early and long-term side effects or complications related to the metal framework of the devices are a known issue. A bioresorbable device such as the Carag Bioresorbable Septal Occluder™ (CBSO) could resolve such complications.
View Article and Find Full Text PDFAm J Physiol Heart Circ Physiol
November 2022
Animal studies show a pivotal role of dihydrotestosterone (DHT) in pressure overload-induced myocardial hypertrophy and dysfunction. The aim of our study was to evaluate the role of DHT levels and myocardial hypertrophy and myocardial protein expression in patients with severe aortic valve stenosis (AS). Forty-three patients [median age 68 (41-80) yr] with severe AS and indication for surgical aortic valve replacement (SAVR) were prospectively enrolled.
View Article and Find Full Text PDFSeven adult patients underwent a two-stage treatment of complex coarctation (CoA), including surgical revascularization of the left subclavian artery (LSA) to left common carotid artery (LCCA), followed by transcatheter covered stent implantation. The majority of patients (5 of 7, 71%) received 1 covered stent (covered Cheatham Platinum stent: 8 zig/45 mm [ = 2], 10 zig/60 mm [ = 1], 10 zig/65 mm [ = 1]; BeGraft: 24/48 mm [ = 2]). In 1 patient (14%), the implantation of 2 covered stents (BeGraft 20/48 mm) was necessary.
View Article and Find Full Text PDFObjectives: Despite the outstanding success of the Fontan operation, it is a palliative procedure and a substantial number of patients experience late failure of the Fontan circulation. Clinical presentation and hemodynamic phenotypes of Fontan failure are considerably variable. While various parameters have been identified as risk factors for late Fontan failure, a feasible score to classify Fontan failure and possibly allow timely risk stratification is lacking.
View Article and Find Full Text PDFBackground Infective endocarditis (IE) after pulmonary valve replacements in congenital heart disease is a significant concern. This study aimed to identify specific long-term risk factors for IE after percutaneous pulmonary valve implantation or surgical pulmonary valve replacement. Methods and Results All patients with congenital heart disease from the National Register for Congenital Heart Defects with at least 1 pulmonary valve replacement before January 2018 were included.
View Article and Find Full Text PDFCoronary artery lesions represent rare conditions in pediatric congenital heart disease and mainly include coronary artery stenoses (CAS) or coronary artery fistulae (CAF). Due to the small vessel size, pediatric percutaneous coronary interventions (PCI) are demanding and studies concerning long-term results are missing. In this retrospective study, we analyzed indications, procedural details, and post-procedural outcomes in pediatric patients who underwent PCI in our institution.
View Article and Find Full Text PDFBackground: The Fontan operation is a palliative procedure and a substantial number of patients eventually experiences late Fontan circulation failure. Previous concepts of Fontan failure implicate increasing pulmonary vascular resistance (PVR) as a key contributor to late circulatory failure. However, data to support this assumption are sparse.
View Article and Find Full Text PDFBackground: CNI-free immunosuppression with conversion to mTORi-based immunosuppression has been demonstrated to reduce CNI-toxicity and to exhibit anti-proliferative properties. However, the experience of CNI-free immunosuppression in paediatric heart transplantation is limited.
Methods: A retrospective analysis was conducted of 129 paediatric heart transplants performed between 1997 and 2015.
Objectives: The aim of this study was to report our initial experience when using Matrix Patch™ a cell-free equine-derived pericardium for the augmentation of branch pulmonary arteries (PAs) in children.
Methods: Between September 2016 and September 2019, Matrix Patch was used for the augmentation of branch PAs in 96 patients and implanted in 147 separate locations. The median age at implantation was 3.
Objectives: The Fontan operation aims at reducing cyanosis and cardiac volume overload in patients with complex univentricular heart malformations. However, persisting or reoccurring cyanosis is frequently observed. We sought to systematically determine the prevalence and clinical consequences of persisting and secondary cyanosis after Fontan operation.
View Article and Find Full Text PDFBackground: Pharmacological stress testing can help to uncover pathological hemodynamic conditions and is, therefore, used in the clinical routine to assess patients with structural heart diseases such as aortic coarctation with borderline indication for treatment. The aim of this study was to develop and test a reduced-order model predicting dobutamine stress induced pressure gradients across the coarctation.
Methods: The reduced-order model was developed based on n=21 imaging data sets of patients with aortic coarctation and a meta-analysis of subjects undergoing dobutamine stress testing.
Objectives: The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season.
Methods: We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1-42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October).
Aims: The post-approval MELODY Registry aimed to obtain multicentre registry data after transcatheter pulmonary valve implantation (TPVI) with the Melody™ valve (Medtronic plc.) in a large-scale cohort of patients with congenital heart disease (CHD).
Methods And Results: Retrospective analysis of multicentre registry data after TPVI with the Melody™ valve.
Treatment of aneurysmal lesions in the context of coarctation of the aorta (CoA) is a challenging task and these lesions are rare in children. An 11-year-old boy was incidentally diagnosed with native CoA and concomitant complex aneurysmal lesions during medical check-up for arterial hypertension. Pre-catheterization imaging was performed with computed tomography (CT), which showed a mild CoA and two native aneurysms that were juxtaposed to the origin of the left subclavian artery.
View Article and Find Full Text PDFEur J Cardiothorac Surg
December 2018
Objectives: The search for an optimal patch material for aortic valve reconstruction (AVR) is an ongoing challenge. In this study, we report our experience of AVR using decellularized bovine pericardial patch material in congenital heart surgery.
Methods: Data of 40 consecutive patients who underwent AVR using the CardioCel® patch (Admedus Regen Pty Ltd, Perth, WA, Australia) between February 2014 and August 2016 were retrospectively reviewed.
Cardiol Young
February 2018
Background: We sought to assess left ventricular regional function in patients with and without left ventricular wall scar tissue in the long term after repair of an anomalous origin of the left coronary artery from the pulmonary artery.
Methods: A total of 20 patients aged 12.8±7.
Objectives: We aimed to compare early postoperative outcome after extracardiac (EC) Fontan operation between patients with right (RV) or left (LV) systemic ventricles.
Methods: In total, 173 consecutive patients (median age 4 years, median weight 14 kg) underwent EC Fontan between 1995 and 2013. Pre- and intraoperative data as well as detailed postoperative haemodynamic variables were compared between patients with LV [ n = 109 (63%)] and RV [ n = 64 (37%)].
The recovery of cardiopulmonary variables from peak exercise in patients with pulmonary stenosis (PS) or regurgitation (PR) is delayed, but the impact of treating PS or PR on exercise recovery kinetics is unknown. 43 patients (median age 14 years) with PS (n = 23) or PR (n = 20) after repair of congenital heart disease underwent successful percutaneous pulmonary valve implantation (PPVI). Cardiopulmonary exercise tests (CPET) were performed both before and within 1 month after PPVI.
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