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

Abstract

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.