Background: Congenital heart disease (CHD) is the most common birth defect worldwide. Depending on complexity of the CHD, around 50% of these children require cardiovascular surgery, which may involve removal of the thymus for a better access to the heart. Given the pivotal role of the thymus in shaping a functional adaptive immune response, thymectomy may have detrimental health consequences.

AimsIn genetic aortopathies (GA) particular attention is paid to aortic root dilatation which has an impact on morbidity and mortality. This study focuses on the effects of therapy with angiotensin-II-receptor-blockers (ARB) or beta-blockers (BB) on aortic root growth and the question which therapy should be initiated at which dosage and at what age.MethodsSince 1998 we diagnosed 208 patients with GA (170 FBN-1). 81 patients between 5 months and 18 years receiving either ARB or BB therapy were included. We retrospectively analyzed the progression of the dilatation of Sinus Valsalva aortae (SV) using calculated z-scores before and after therapy initiation and compared BB and ARB treatment.ResultsBoth ARB and BB (p < 0.05) therapy showed significant improvement in aortic root growth, while the effect is significantly more pronounced in ARB (p < 0.01) independent of age and genetic cause. A detailed comparison of the two drug groups showed a more sustained effect in limiting the progression of the dilatation of the aortic root in patients treated with ARB. Progression of dilatation of the SV was significantly lower in children treated with ARBs compared to BB (delta z-score, p < 0.05). In addition, ARBs were better tolerated and had a significantly lower discontinuation rate (3%) compared to BB (50%) (p < 0.01). Independently of age at initiation all children and adolescents were able to reach the target dose under ARB.ConclusionWe demonstrated a significant change in both treatment options, with the effect of ARB being more pronounced while being better tolerated throughout the treatment period.

Background: Systemic-to-pulmonary shunts are technically demanding and yet are associated with a considerable risk of failure. Shunt failure may be related to stenosis with consecutive hypoxemia and eventually death. We retrospectively revised our results taken a comprehensive dataset into account to identify the impact of early shunt obstruction on survival and on which parameters may influence early shunt obstruction defined as necessity for surgical or interventional measures within 30 days after implantation.

Background: Congenital heart defects (CHDs) are among the most common birth defects, often requiring an individualized and multidisciplinary treatment approach depending on their complexity. In particular, comprehensive diagnostics are necessary for the treatment of patients with complex and combined defects. To plan an optimal treatment concept, it is necessary to become aware of the exact anatomy of the respective structures. Therefore, modeling and printing of three-dimensional (3D) models from DICOM (Digital Imaging and COmmunication in Medicine) data allows the surgeon to get an optimal image of the pathologies present. Due to the excellent contrast and spatial resolution, computed tomography (CT) and magnetic resonance imaging (MRI) are the most suitable imaging modalities for 3D imaging.

Background: Primary heart transplantation ruled out, the Fontan principle in all its variations is the only surgical treatment option for patients with single ventricle anatomy. With the systemic and pulmonary circulation connected in series, blood flows passively into the pulmonary vasculature. There is evidence from a chronic porcine model of cavopulmonary connection that even weak pulsatile stimuli positively affect vascular tone, endothelial function, and the development of pulmonary arteriovenous malformations. We aimed to generate a contractile subpulmonary neoventricle from engineered heart tissue (EHT) to treat patients with single ventricle anatomy in the future.

Background: Therapy for hypoplastic left heart syndrome and complex (HLHS and HLHC, respectively) is still associated with significant morbidity and mortality. Our aim is to improve the outcome of these vulnerable patient groups by minimizing neonatal surgical trauma and postponing surgeries to later infancy. Here, we describe four patients who underwent a compassionate stage-1 procedure (S1P) using a novel, fully transcatheter approach.

Background: We present the case of a 7-year-old girl (15 kg and 135 cm) with SARS-CoV-2 infection who developed severe heart failure due to myocarditis and acute respiratory distress syndrome resulting in multisystem inflammatory syndrome in children (MIS-C) requiring extracorporeal membrane oxygenation (ECMO) support.

Background: Congenital heart disease (CHD) is the most common birth defect worldwide affecting approximately 1% of newborns. Patients with CHD are prone to infections, especially respiratory tract infections and suffer from higher hospitalization rates and mortality. Recently, it was shown that newborns with CHD display lower T-cell receptor excision circle (TREC) levels, a marker for T-cell maturation in the thymus, compared with the general population. Therefore, we hypothesize that congenital heart disease negatively affects early T cell development, probably before birth, with possible long-term consequences for the health of CHD patients.

Background: Due to deficient lipoate synthesis, variants within the BOLA3 gene result in a deficiency of 2-oxoacid dehydrogenases accompanied by defects of the mitochondrial respiratory chain. Patients typically show a severe and progressive multisystem phenotype with hypertrophic cardiomyopathy, white matter lesions, epilepsy, and involvement of visceral organs. Around 25 patients can be found in scientific literature. We describe the first patient with a severe phenotype and homozygosity for a 3 bp-deletion in BOLA3.

Background: Ebstein's Anomaly (EA) is a rare congenital heart disease with an incidence of 0.5%. Throughout the last five decades, multiple surgical techniques for tricuspid valve (TV) surgery have been reported. Therefore, the aim of our study was to investigate outcome and risk factors for mortality and morbidity after surgery for EA.

Background: After birth, cardiomyocytes (CM) leave the cell cycle, lose their ability to proliferate and remain predominantly in a postmitotic state throughout life. It is not known which stimuli lead to the cell cycle arrest. One reason could be the change in postnatal afterload. Postpartum, left-ventricular afterload increases while right-ventricular afterload decreases. Thus, the comparison of both ventricles allows investigation of the influence of afterload on cell proliferation rate.

Background: The impact of size (diameter) on longevity of biological valves in the RVOT remains a matter of debate. We report on our experience in patients with ToF-type anatomy, including double outlet right ventricle (DORV), and pulmonary atresia (PA)/ventricular septal defect (VSD) with or without major aorto-pulmonary collaterals (MAPCA).

Objectives: Bronchogenic cyst (BC) is rare and indication for early invasive treatment for BC is still under discussion. Nevertheless, life-threatening complications due to compression, infection, hemorrhage, and rupture are outlined in the literature.

Objectives: Delayed sternal closure (DSC) is a well-established procedure to reduce postoperative hemodynamic and respiratory instability following neonatal or infant cardiac surgery. Though, DSC is thought to be associated with increased rates of sternal wound infection (SWI), sepsis and mortality. Indication and duration of DSC vary between pediatric cardiac centers worldwide. Therefore, we reviewed our experience with DSC to determine the incidence of SWI and to evaluate potential perioperative risk factors.

Objectives: Unilateral isolated absence of a pulmonary artery (UAPA) is a rare congenital lesion, which can lead to pulmonary hypoplasia and pulmonary hypertension. To avoid these complications, early surgical repair of UAPA is recommended. Surgical approaches include creation of an aorto-pulmonary shunt, direct anastomosis between pulmonary artery and main pulmonary artery (MPA) or bridging the “gap” with prosthetic interposition grafts. We present a novel surgical technique using a total autologous interposition tube graft using tissue from the roof of the MPA.

Objectives: Alpha-actin is an important contractile protein in vascular smooth muscle cells (SMCs). SMCs are found in walls of arteries and hollow visceral organs as well as in lacrimal ducts, hair follicles and the iris. Mutations in the most abundant form, α-actin2 (ACTA2), result in fixed dilated pupils, large dilated persistent ductus arteriosus and pulmonary hypertension. Surgical closure of a patent ductus arteriosus (PDA) in neonates, routinely performed via lateral thoracotomy, may not be advisable in patients ACTA2-mutations. We present the case of a patient referred to our institution for a giant arterial duct and pulmonary hypertension.

Objectives: Branch pulmonary artery (BPA) stenosis is a common finding in patients with congenital heart disease (CHD), particularly in patients after repair of tetralogy of Fallot (ToF)/pulmonary atresia (PA). In this subset of patients ~10% require re-intervention for recurrent BPA stenosis. Valuable treatment modalities for BPA stenosis include surgical patch angioplasty, percutaneous BPA stenting and hybrid stent placement. The purpose of this study was to report our institutional experience with hybrid stenting to treat BPA stenosis.