Aetiology and 30-Year Long-Term Outcome of Children with Cardiomyopathy Necessitating Heart Transplantation.

Studies assessing the long-term outcome after heart transplantation HTX in patients with cardiomyopathy (CM) in the paediatric age range are rare. The aim of this study was to determine the survival rate of children with CM undergoing HTX and to analyse how aetiology of cardiomyopathy influenced morbidity and mortality. We retrospectively analysed the medical records of children; who were transplanted in our centre between June 1988 and October 2019.

Radixin Relocalization and Nonmuscle -Actinin Expression Are Features of Remodeling Cardiomyocytes in Adult Patients with Dilated Cardiomyopathy.

Background: Pediatric patients show an impressive capacity of cardiac regeneration. In contrast, severely deteriorated adult hearts do usually not recover. Since cardiac remodeling-involving the expression of fetal genes-is regarded as an adaptation to stress, we compared hearts of adult patients suffering from dilated cardiomyopathy (DCM) with remodeling of cultured neonatal (NRC) as well as adult (ARC) rat cardiomyocytes and the developing postnatal myocardium.

Anesthesia for bilateral pulmonary banding as part of hybrid stage I approach palliating neonates with hypoplastic left heart syndrome.

Background: Neonatal management of patients with hypoplastic left heart syndrome and complex remains a challenging task, whereby the "hybrid" palliation is often reserved for high-risk patients as a "rescue" procedure.

Aim: This study documents the anesthetic challenges and potential complications associated with the Giessen hybrid stage I approach.

Methods: The Giessen hybrid stage I approach is focused on surgical bilateral pulmonary artery banding.

Anesthesia for Pediatric Heart Transplantation: Are Patients With a Failing Hemi-Fontan- or Fontan-Physiology Different?

. The absolute number of patients presenting with failure of the hemi-Fontan or Fontan circulation for cardiac transplantation (cTx) will continuously rise. We aimed to analyze the intraoperative differences in patients undergoing cTx for a failing hemi-Fontan or Fontan circulation (group A) with those undergoing cTx for cardiomyopathy and congenital heart disease (group B).

Hybrid therapy for hypoplastic left heart syndrome: Myth, alternative, or standard?

Objective: This retrospective study presents our operative results, mortality, and morbidity with regard to pulmonary artery growth and reinterventions on the pulmonary artery and aortic arch, including key features of our institutional standards for the 3-stage hybrid palliation of patients with hypoplastic left heart syndrome.

Methods: Between June 1998 and February 2015, 182 patients with hypoplastic left heart structures underwent the Giessen hybrid stage I procedure. Among these, 126 patients with hypoplastic left heart syndrome who received a univentricular palliation or heart transplantation were included in the main analysis.

Long-term results of biventricular repair after initial Giessen hybrid approach for hypoplastic left heart variants.

Objective: This article presents the long-term results of our patients with a diagnosis of hypoplastic left heart syndrome (HLHS), hypoplastic left heart complex (HLHC), and variants who received a biventricular repair following hybrid stage I with ductal stenting and bilateral pulmonary artery banding.

Methods: Between June 1998 and June 2013, a total of 154 patients with hypoplastic left heart structures underwent a hybrid stage I procedure. Forty patients were definitely treated by creating a biventricular circulation.

Fifteen-year single center experience with the "Giessen Hybrid" approach for hypoplastic left heart and variants: current strategies and outcomes.

Presented is a retrospective outcome study of a 15-year single institutional experience with a contemporary cohort of patients with hypoplastic left heart syndrome and complex that underwent a "Giessen Hybrid" stage I as initial palliation. Hybrid approach consisting of surgical bilateral pulmonary artery banding and percutaneous duct stenting with or without atrial septum manipulation was developed from a rescue approach to a first-line procedure. Comprehensive Aristotle score defined pre-operative condition.

Giant remnant of fetal circulation leading to cyanosis: pseudo-cor triatriatum dexter.

Remnants of the right valve of the sinus venosus, which fail to regress may present as an obstructing membrane within the right atrium as a very rare congenital anomaly. When associated with an atrial septal defect, primary cyanosis may occur due to a large right-to-left shunt. We report on a 1-week old girl with a pseudo-cor triatriatum dexter and immediate cyanosis after an uncomplicated birth.

Pulmonary artery banding in infants and young children with left ventricular dilated cardiomyopathy: a novel therapeutic strategy before heart transplantation.

Background: Dilated cardiomyopathy (DCM) in childhood has a considerable morbidity and mortality and high incidence of heart transplantation. Pulmonary artery banding (PAB) has been proposed in patients with corrected transposition of the great arteries to retrain the sub-pulmonic left ventricle (LV) and to improve a failing sub-aortic right ventricle. We evaluated the short-term and medium-term effects of PAB in young patients with LVDCM.

Beneficial effects of residual right ventricular outflow tract obstruction on right ventricular volume and function in patients after repair of tetralogy of Fallot.

Preservation of the pulmonary valve, even at the expense of a mild residual stenosis, is the current surgical policy for the management of patients with tetralogy of Fallot (TOF). This study aimed to assess the long-term effect of a residual right ventricular outflow tract obstruction (RVOTO) on RV dimension and function. This study prospectively assessed 53 children (mean age, 13.

Modified repair of interrupted aortic arch utilizing retroesophageal right subclavian artery based on a neonatal hybrid approach in hypoplastic left heart complex.

Objective: Interrupted aortic arch (IAA) combined with an aberrant right subclavian artery (ARSA) is frequently associated with a hypoplastic ascending aorta. Neonatal surgical therapy carries a high risk particularly for aortic arch obstructions during the further follow-up.

Methods: We performed a modified reconstruction of the aortic arch utilizing the ARSA as a natural substitute in a staged surgical approach.

Large-diameter graft-stent (Advanta V12) implantation in various locations: early results.

Objectives: Transcatheter stent placement carries the risk of cardiovascular aneurysm or rupture. Covered stent implantation reduces these risks. The recently marketed Advanta V12 large-diameter-covered stent is pre-mounted and requires 9 (8)-11 Fr delivery systems.

Stent implantation of the arterial duct in newborns with a truly duct-dependent pulmonary circulation: a single-center experience with emphasis on aspects of the interventional technique.

Introduction: Ductal stenting for pulmonary blood supply in newborns with cyanotic congenital heart disease (CHD) might be a low risk and safe alternative to the surgical aorto-to-pulmonary artery (AP) shunt in dual-source lung perfusion. Ductal stenting in truly duct-dependent pulmonary circulation has not been evaluated.

Methods: Prospective interventional and clinical follow-up trial.

Fontan extracardiac tunnel connection: fenestration or not?

Background: The fenestration function is by allowing a right-to-left shunt resulting in an increased cardiac index, associated with mild arterial oxygen desaturation. Subsequent transcatheter fenestration closure can be performed after haemodynamic assessment. The purpose of this study was to compare the outcomes of extracardiac connection (EC) with or without fenestration.

Hemodynamic effects of dobutamine and dopexamine after cardiopulmonary bypass in pediatric cardiac surgery.

Background: After surgical repair of congenital heart disease, inotropic support is sometimes necessary to wean from cardiopulmonary bypass. In pediatric cardiac surgery, dobutamine and dopamine are often used as inotropic support. Dopexamine is a synthetic catecholamine, which has positive inotropic and vasodilating properties.

Total cavopulmonary connection: lateral tunnel anastomosis or extracardiac conduit?--an analysis of 114 consecutive patients.

Objective: To compare the postoperative outcomes of patients with the diagnostic univentricular heart undergoing lateral tunnel (LT) operation with extracardiac conduit (EC) operation.

Methods: From June 1996 to July 2007, 114 consecutive patients with a single ventricle underwent total cavopulmonary connection (TCPC) in Children's Heart Center, University Hospital Giessen and Marburg GmbH, Germany. A LT was performed in 19 (16.

Implantation of stents to ensure an adequate interatrial communication in patients with hypoplastic left heart syndrome.

Aims: To assess the feasibility of interatrial stenting for left atrial decompression in infants with hypoplastic left heart syndrome treated by a "hybrid-approach", with bilateral surgical banding of the pulmonary arteries and percutaneous stenting of the arterial duct.

Patients And Methods: We stented the atrial septum in 5 infants aged from 21 to 77 days, making the intervention as an elective procedure in 4, but as a rescue procedure in the fifth patient, who had a restrictive foramen. The stents, comprising 2 Jo-stents of 17 millimetres hand-crimped on a balloon catheter with dimensions of 10 by 20 millimetres, and 3 premounted Genesis stents with dimensions of 10 by 19 millimetres, were placed using a 6 French long or short sheath by femoral venous access.

Hybrid transcatheter-surgical palliation: basis for univentricular or biventricular repair: the Giessen experience.

The outcome of patients with hypoplastic left heart (HLH) is determined by many factors, particularly by the first-step palliative procedure in newborns undergoing the Norwood procedure, its Sano modification, or, rarely, through challenging biventricular repairs. Duct stenting combined with bilateral pulmonary artery banding (PAB) is a new method employed as an alternative first-step approach in a number of centers worldwide. We describe this interventional-surgical "hybrid approach" as an additional strategy for the treatment of newborns with HLH syndrome and HLH complex.

Transplantation in truncus arteriosus combined with interrupted aortic arch.

We present the case of an infant with truncus arteriosus combined with a dysplastic truncal valve not amenable to repair and interrupted aortic arch. Due to the disappointing results of truncal valve replacement we decided to perform cardiac transplantation. The infant survived transplantation and was discharged 4 weeks after surgery.

Percutaneous closure of congenital and acquired ventricular septal defects--considerations on selection of the occlusion device.

Nonsurgical closure of congenital and acquired ventricular septal defects (VSD) has become increasingly acceptable with the availability of various occlusion systems that allow percutaneous treatment of muscular and membranous defects. This study describes a series of 12 patients (0.2-74-years-old) who underwent defect closure with six different occlusion systems.

Visualisation of tissue kallikrein, kininogen and kinin receptors in human skin following trauma and in dermal diseases.

During dermal injury and inflammation the serine proteases kallikreins cleave endogenous, multifunctional substrates (kininogens) to form bradykinin and kallidin. The actions of kinins are mediated by preferential binding to constitutively expressed kinin-B2 receptors or inducible kinin-B1 receptors. A feature of the kinin-B1 receptors is that they show low levels of expression, but are distinctly upregulated following tissue injury and inflammation.