New Applications of Cardiopulmonary Exercise Testing and Training in Paediatric Heart Disease

Congenital heart defects (CHD) have an incidence of 4-8/1000 live births and encompass a broad spectrum of disorders. Due to advances in cardiac surgery and cardiology care, most children born with CHD are now surviving into adulthood and there are currently more adults affected than children. Exercise capacity is reduced across the spectrum of patients with CHD, both in natural history and after surgical and interventional treatment. The aim of this project was to better understand exercise limitations and safety/usefulness of training in young patients with heart disease across a broad spectrum of disorders and in particular we focused on left-to-right shunts, systemic right ventricular physiology, univentricular physiology with Fontan palliation and dilated cardiomyopathy. Future perspective include exploring new ways of engaging teenagers with univentricular palliation in systemic and respiratory muscle training. Furthermore, the collaboration with biomedical engineers will allow us to gain in depth understanding of univentricular physiology. Similarities exisit in the physiological changes observed during exercise and pregnancy and the final goal is to implement the model with these variables to achieve better clinical outcome in this growing population of young adults with complex lesions and limited exercise and child-bearing potential. Exercise testing and training are becoming more and more relevant to guide therapy and management but also to assess the ability in daily activities that play an important role in many aspects of life that have not been addressed specifically until now. The increasing data available enable physicians to give adequate counseling regarding vocational or professional choices, suitable leisure activities and family planning according to the levels of activity considered safe and sustainable in the specific physiology. Further studies will warrant deeper understanding of issues that are specific to univentricular physiology and will help us to target interventions to improve quantity and quality of life.