Univentricular Heart

This prospective cohort study evaluates differences in clinical outcomes between pulmonary oligemia and pulmonary plethora in patients with cyanotic congenital heart disease and univentricular heart physiology undergoing staged palliation culminating in the Fontan procedure.Fifty-two patients will be classified into two groups based on pulmonary blood flow characteristics and followed for approximately 1.5 years after the Fontan procedure. Outcomes include mortality, morbidity, pulmonary hemodynamics, functional capacity, neurocognitive status, and quality of life.

The goal of this observational clinical study look at the safety and effectiveness of the Occlutech Atrial Flow Regulator in the treatment of patients with failing Fontan circulation. This study will consist of a case series of congenital patients with Fontan failure who have been treated with an Occlutech AFR under the FDA's Expanded Access process. The scope of this study is to gain clinical experience on the Occlutech AFR in Fontan failure patients. Cases in this study will be evaluated to evaluate the safety and effectiveness of the device. The objective of this study is to evaluate the Occlutech AFR with respect to device safety and shunt patency at 1 year. Shunt patency defined by persistent right to left shunt assessed by echocardiography at 1-year post-procedure.

The goal of this clinical trial is to test the safety of lab-grown heart cells made from stem cells in subjects with congenital heart disease. The main questions it aims to answer are: * Is this product safe to deliver to humans * Is the conduct of this trial feasible Participants will be asked to: * Agree to testing and monitoring before and after product administration * Receive investigational product * Agree to lifelong follow-up Researchers will compare subjects from the same pool to see if there is a difference between treated and untreated subjects.

The purpose of the study is to monitor the clinical safety and performance of the EXCOR Venous Cannula in context of an EXCOR VAD therapy to ensure continued acceptability of identified risks, to enable detecting emerging risks and to assess clinical improvement on both short- and long-term.

Univentricular heart (UVH) is a severe congenital heart disease. Accurate advanced non-invasive diagnostic methods is limited. Cardiovascular magnetic resonance (CMR) imaging has evolved as a particularly useful tool for the study of patients with adult congenital heart disease (ACHD) considering its ability to determine detailed anatomy and detect early cardiac dysfunction without the need for radiation exposure. Most of contemporary treatment recommendations are based on consensus opinions/documents and small studies from local, or national registries. Improved knowledge is needed in all these areas to facilitate clinical decisions regarding treatment, monitoring and follow-up. This study seeks to answer if early detection of deterioration in cardiac function, venous pressure and microvascular dysfunction can identify patients before the symptoms progress and thus help to initiate early treatment. The hypothesis is that quantitative myocardial stress-perfusion maps improves the pathophysiological insight in patients with UVH. The overall goal with this research proposal is to implement combined advanced CMR imaging for a comprehensive non-invasive mapping of functional cardiovascular behavior in patients with complex UVH disease. The outcome of this research may benefit this young adult patient population due to early detection of cardiac disease, less hospitalizations because of heart failure, and eventually decrease morbidity and mortality.

Univentricular heart defects are among the most complex congenital malformations. The treatment of these patients usually includes 3 operations and accompanying diagnostics, some of which are invasive, within the first 3 to 4 years of life until the "fontan circulation" is established. Unfortunately, the group of patients with a univentricular cardiac malformation is also the group with the highest mortality rate until the Fontan circulation is established. The expected mortality varies depending on the complexity of the underlying intracardiac and extracardiac malformations and possible concomitant morbidity; According to data from the National Quality Assurance in Germany, it is currently up to 30% in patients with hypoplastic left heart syndrome. However, the treatment methods are well standardized and there has been a clinical protocol in Erlangen since 2008 that includes diagnostic examinations using cardiac catheterization (HKU) and magnetic resonance imaging (MRT), as well as non-invasive, instrument-based diagnostics and targeted blood tests in a consistent sequence before and after the operations regulates. The Erlangen protocol ends with the cardiac MRT six months after the Fontan operation (syn: total cavopulmonary connection \[TCPC\]). If MRT imaging is not possible (e.g. if a cardiac pacemaker is present) or if there is a clinical indication (e.g. an obstruction noticeable on MRT or the presence of an "overflow with right-left shunt"), HKU is performed in the first year after TCPC. These examinations are used to depict hemodynamics, cardiac function, vascular development, and lymphatic drainage disorders in order to evaluate the preoperative risk, adapt treatment strategies to the individual, and identify complications at an early stage. This concept is based on clinical experience and current scientific findings and has proven itself in everyday clinical practice since its introduction for Erlangen-Fontan patients and has proven to be very helpful for individualized treatment. After a successful Fontan operation, the mortality risk in childhood is only low. In the long term, however, some of the patients unfortunately suffer from Fontan-typical long-term complications with protein-loss enteropathy having to be mentioned in particular, which is described in the literature with an incidence of 3-14% and today still a 5-year incidence-has a mortality risk of 6-12%.

After successful Fontan surgery, the risk of mortality in childhood is only low. Unfortunately, some of the patients suffer from Fontan-typical long-term complications in the long-term course, whereby protein loss neuropathy must be mentioned in particular, which is described in the literature with an incidence of 3-14% (1, 2) and still has a 5-year risk of death of 6-12% today (2, 3). Protein loss tereopathy leads to loss of protein in the intestine and subsequently to diarrhea and edema. Other problems concern the liver, which can develop cirrhosis due to chronic congestion (4-6). Cardiac can lead to heart failure and arrhythmias. The registry study described in this protocol is intended to identify factors that influence the treatment outcome of patients in the Fontan circulation in the long term through systematic prospective documentation of the data from our standardized and guideline-oriented treatment.