Mechanical circulatory support (MCS) remains a last resort that is not initiated until all the conservative treatment options to save a patient’s life have failed. With the rapid and recent progress in MCS technology and management strategies we were able to improve the extended survival and the quality of life of patients suffering advanced heart failure. MCS includes two different kinds of devices for different indication and patient age. First, extracorporeal circulatory life support (ECLS) and extracorporeal membrane oxygenation (ECMO) are playing a growing and important role in our temporary bridging concepts, providing life-saving support for critically ill children.
The Deltastream® DP3 (Xenios, Medos, Germany) is an easy-to-handle miniaturized pump with only 16 ml priming volume which has proven to be very effective in both lung replacement (ECMO mode) and pulmonary and circulatory replacement (ECLS mode). We were the first to treat children with this device, and we subsequently conducted and published a retrospective mutlicenter study, which enabled us to identify risk factors such as bleeding and thrombosis and to modify therapy recommendations appropriately (Fleck et al., 2013; Stiller et al., 2017).
Further research applications for the development of ECLS treatment into a pulsatile mode to improve end organ perfusion in childhood are in progress (ethical approval obtained).
Second, at the same time, the demand for ventricular assist devices (VAD) for long-term support rose due to the scarcity of donor organs for transplantation. The only long-term support system approved for infants and small children with terminal heart failure is the paracorporeal pneumatically driven pulsatile BerlinHeart EXCOR device (Berlin, Germany), which was developed in the 1990s. Over the last 30 years, we have been involved in the further development of this device both in Berlin and in Freiburg (see Publications).
Our data for health related quality of life and for bleeding problems due to AvWF have part of the improvement for these terminally ill children. AvWS is characterized by the loss of high molecular-weight multimers of von Willebrand factor (vWF), which was be shear stress-induced, ultimately impairing vWF’s function. We found that AvWS is characterized by significant variation in the severity of bleeding propensity among pediatric patients during MCS. With detailed clinical and biochemical phenotyping work on the risk-stratification of patients during MCS workup.