The European Registry for Patients with Mechanical Circulatory Support (EUROMACS): first annual report†

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS) was founded on 10 December 2009 with the initiative of Roland Hetzer (Deutsches Herzzentrum Berlin, Berlin, Germany) and Jan Gummert (Herz- und Diabeteszentrum Nordrhein-Westfalen, Bad Oeynhausen, Germany) with 15 other founding international members. It aims to promote scientific research to improve care of end-stage heart failure patients with ventricular assist device or a total artificial heart as long-term mechanical circulatory support. Likewise, the organization aims to provide and maintain a registry of device implantation data and long-term follow-up of patients with mechanical circulatory support. Hence, EUROMACS affiliated itself with Dendrite Clinical Systems Ltd to offer its members a software tool that allows input and analysis of patient clinical data on a daily basis. EUROMACS facilitates further scientific studies by offering research groups access to any available data wherein patients and centres are anonymized. Furthermore, EUROMACS aims to stimulate cooperation with clinical and research institutions and with peer associations involved to further its aims. EUROMACS is the only European-based Registry for Patients with Mechanical Circulatory Support with rapid increase in institutional and individual membership. Because of the expeditious data input, the European Association for Cardiothoracic Surgeons saw the need to optimize the data availability and the significance of the registry to improve care of patients with mechanical circulatory support and its potential contribution to scientific intents; hence, the beginning of their alliance in 2012. This first annual report is designed to provide an overview of EUROMACS' structure, its activities, a first data collection and an insight to its scientific contribution

Chirurgische Techniken und Ergebnisse nach Rekonstruktion von Mitralklappenfehlern bei Säuglingen und Kindern

These cumulative studies described our entire surgical experience with congenital and acquired mitral valve anomalies in children with biventricular hearts and concordant connections inclusive of atrioventricular septal defects. These confirmed the diversity and severity of congenital MV anomalies, which characteristically involved multiple components of the mitral valve complex. In our institution, MV reconstruction is the surgical technique of choice for any kind of mitral disease in childhood. We firmly believe that repair allows continuous somatic and valve growth, and delays or eliminates the need for future valve replacement and lifelong anticoagulation, and obviating the known complications of valve replacement which frequently requires subsequent reoperation to implant a larger prosthesis. It must be assumed that the majority of, if not all, valves repaired during childhood, will eventually have to be replaced at some time in life. The concept of repair in childhood primarily aimed at growth of the patient to an age when, if necessary, an adult size prosthesis can be implanted. Strong predictors for poor overall survival and freedom from reoperation are age less than 3 months, urgency of surgery, concomitant procedures.Die vorliegenden kumulativen Studien beschreiben unsere gesamte operative Erfahrung mit angeborenen und erworbenen Mitralklappenfehlern bei Kindern mit Zweikammer-Herzen und konkordanten Verbindungen mit Ausnahme von atrioventriculären Septumdefekten. Diese Studien unterstreichen die Vielfältigkeit und den Schweregrad von angeborenen Mitralklappenfehlern, die typischerweise mehrere Teile des Mitralklappenapparats befallen. In unserer Institution ist die Mitralklappenrekonstruktion die chirurgische Technik der Wahl für jede Art von Mitralklappenfehlern im Kindesalter. Wir sind davon überzeugt, dass die Rekonstruktion das ungestörte Wachstum des gesamten Körpers und der Klappe erlaubt und dass die Notwendigkeit eines späteren Klappenersatzes in ein höheres Lebensalter verschoben werden kann. Damit werden dem Kind die Nachteile einer Ersatzklappe, wie dauernde Antikoagulation, mögliche erneute Operationen bei zu kleiner Klappe und andere Probleme erspart. Dabei muss man jedoch annehmen, dass die Mehrzahl, wenn nicht alle Klappen, die während der Kindheit rekonstruiert wurden, irgendwann im Leben doch zu einem Klappenersatz führen. Damit ist das Konzept der Rekonstruktion im Kindesalter darauf abgestellt, dass der Patient mit der Rekonstruktion ein Alter erreichen kann, wenn eine ausreichend große Prothese implantiert werden kann, die auch für ein Erwachsenenleben reicht. Ungünstige Prädiktoren für Überleben und Freiheit von Re-Operation sind Alter unter 3 Monaten, Dringlichkeit der Operation und Begleiteingriffe

State of the art in paediatric heart transplantation: the Berlin experience

Summary Enormous progress has been made in paediatric heart transplantation since the first unsuccessful effort by Kantrowitz in 1967. Early reports of children undergoing heart transplantation showed alarmingly high perioperative mortality rates of 25-60%, with the diagnosis of congenital heart disease (CHD) representing a particularly high-risk subset compared with cardiomyopathy. Many of these early failures were related to poor patient selection, suboptimal immunosuppression and technical problems. We learned a great deal from these earlier difficulties. Presently, with more refined techniques, better-defined patient selection criteria, excellent graft rejection monitoring and optimal immunosuppression, the ISHLT 2011 registry reported a 10-year survival rate of 60% for patients transplanted for end-stage CHD and >70% for those transplanted for cardiomyopathy. The technical dilemmas in complex CHD were overcome by surgical ingenuity and creativity, innovative solutions and careful surgical planning, adapting the complex recipient anatomy to the normal donor anatomy. The miniaturized Berlin Heart pulsatile ventricular assist devices in children as a bridge to transplantation have revolutionized treatment and become a significant contribution in heart-failure therapy. The intramyocardial electrogram and echocardiographic strain rate imaging have been employed as non-invasive techniques of rejection monitoring. Immunosuppressive drugs have a major impact on the development and progression of cardiac allograft vasculopathy, the main cause of cardiac allograft loss and a leading cause of mortality after the first year post-transplantation. The questions of whether a transplanted heart in a newborn grows to adult size along with the child and whether the dimensional cardiac growth allows adequate function over time have been largely answered in our previous investigations. As more transplanted children reach adulthood, concerns about their life expectancy when they have reached 10 years of life post-transplant are raised, particularly with respect to establishing partnerships and families, their ability to earn a living and the fulfilment of personal life perspectives. Some heart-transplanted patients require retransplantation to remain alive. The disparity between the demand for and supply of donor hearts makes retransplantation an ethical issue. We 'do not refuse' any patient who needs retransplantation. Mechanical circulatory support devices for long-term use are now largely available to accommodate such cases

Influence of size disparity of transplanted hearts on cardiac growth in infants and children

OBJECTIVE: We aimed to evaluate the influence of size disparity of the transplanted heart on cardiac growth in infant and child recipients by comparing donor body surface area (BSA) and cardiac dimensions during transplantation to the corresponding parameters of the recipient over a period of time. METHODS: A retrospective review of medical and echocardiographic records of 147 children (5.3 ± 4.0; median, 4.1; range, 1 month-15 years) who underwent orthotopic heart transplantation was done. The patients were divided into age groups as follows: less than 1 year (n = 23), 1 to 2 years (n = 26), more than 2 to 5 years (n = 18), more than 5 to 10 years (n = 27), and more than 10 to 15 years (n = 53). Donor/recipient BSA ratio was determined during transplantation. Cardiac dimensions were measured 30 days after transplantation and compared at 1 year, 2 to 5 years, and 5 to 10 years after transplantation. RESULTS: There were no significant differences in the ventricular end-diastolic diameter, volumes, and mass among those with a donor/recipient BSA ratio of less than 0.80, 0.8 to 1.2, and more than 1.2 (P = .80, .44, and .48, respectively). In all the cardiac dimensions and volumes measured, donor-recipient mismatch did not influence the continuous growth of the heart, as indicated by the measured parameters, in accordance with the recipients' increase in BSA over time. All calculated Z-scores at 1 year, 2 to 5 years, and 6 to 10 years after transplantation were normal when indexed to BSA. CONCLUSIONS: This study demonstrates that despite size disparity of a transplanted heart, it undergoes normal growth in diastolic dimensions, volumes, and myocardial mass over time as appropriate for body growth after cardiac transplantation in infants and children

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS): first annual report†

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS) was founded on 10 December 2009 with the initiative of Roland Hetzer (Deutsches Herzzentrum Berlin, Berlin, Germany) and Jan Gummert (Herz- und Diabeteszentrum Nordrhein-Westfalen, Bad Oeynhausen, Germany) with 15 other founding international members. It aims to promote scientific research to improve care of end-stage heart failure patients with ventricular assist device or a total artificial heart as long-term mechanical circulatory support. Likewise, the organization aims to provide and maintain a registry of device implantation data and long-term follow-up of patients with mechanical circulatory support. Hence, EUROMACS affiliated itself with Dendrite Clinical Systems Ltd to offer its members a software tool that allows input and analysis of patient clinical data on a daily basis. EUROMACS facilitates further scientific studies by offering research groups access to any available data wherein patients and centres are anonymized. Furthermore, EUROMACS aims to stimulate cooperation with clinical and research institutions and with peer associations involved to further its aims. EUROMACS is the only European-based Registry for Patients with Mechanical Circulatory Support with rapid increase in institutional and individual membership. Because of the expeditious data input, the European Association for Cardiothoracic Surgeons saw the need to optimize the data availability and the significance of the registry to improve care of patients with mechanical circulatory support and its potential contribution to scientific intents; hence, the beginning of their alliance in 2012. This first annual report is designed to provide an overview of EUROMACS' structure, its activities, a first data collection and an insight to its scientific contributions.status: publishe

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS): first annual report

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS) was founded on 10 December 2009 with the initiative of Roland Hetzer (Deutsches Herzzentrum Berlin, Berlin, Germany) and Jan Gummert (Herz- und Diabeteszentrum Nordrhein-Westfalen, Bad Oeynhausen, Germany) with 15 other founding international members. It aims to promote scientific research to improve care of end-stage heart failure patients with ventricular assist device or a total artificial heart as long-term mechanical circulatory support. Likewise, the organization aims to provide and maintain a registry of device implantation data and long-term follow-up of patients with mechanical circulatory support. Hence, EUROMACS affiliated itself with Dendrite Clinical Systems Ltd to offer its members a software tool that allows input and analysis of patient clinical data on a daily basis. EUROMACS facilitates further scientific studies by offering research groups access to any available data wherein patients and centres are anonymized. Furthermore, EUROMACS aims to stimulate cooperation with clinical and research institutions and with peer associations involved to further its aims. EUROMACS is the only European-based Registry for Patients with Mechanical Circulatory Support with rapid increase in institutional and individual membership. Because of the expeditious data input, the European Association for Cardiothoracic Surgeons saw the need to optimize the data availability and the significance of the registry to improve care of patients with mechanical circulatory support and its potential contribution to scientific intents; hence, the beginning of their alliance in 2012. This first annual report is designed to provide an overview of EUROMACS' structure, its activities, a first data collection and an insight to its scientific contributions