Univentricular Congenital Heart Defects and the Fontan Circulation

The Univentricular Heart: past, present and future.- Part I Anatomy & epidemiology of the functionally univentricular heart.- The anatomical substrates of the univentricular heart.- Epidemiology of univentricular hearts.- Part II Management of the fetus and neonate with a univentricular heart.- Prenatal Diagnosis of the functionally univentricular heart.- Postnatal management and pre-operative assessment of the univentricular patient.- Imaging to aid decision making in the neonate.- Univentricular Heart: Decision Making in the Management of the Neonate.- Catheter Interventions in the Initial Palliation of Univentricular Hearts.- Comfort Care in Patients with a Single Ventricle.- Part III Surgical management.- History of the Fontan surgical procedure.- Early modifications of Fontan surgery and evolution of the total cavo-pulmonary connection (TCPC).- Staged approach to total cavo-pulmonary connection.- Classical Norwood Stage I and Modifications.- Hybrid strategies for hypoplastic left heart syndrome and comprehensive stage II repair.- Part IV The Fontan physiology.- Determinants of cardiac output and exercise tolerance in the Fontan circulation.- Part V Follow up in childhood of Fontan patients.- Neuro-developmental outcomes of Fontan patients.- Follow-up in childhood of Fontan patients - Quality of life.- Anticoagulation strategies in the single ventricle.- Electrophysiology Considerations after the Fontan Operation.- Managing the pulmonary circulation.- Fontan Failure in Children and Cardiac Transplantation.- Part VI Transition, education and lifestyle.- Transition to adult life.- Part VII Follow up in adult life.- Considerations when Developing a Multidisciplinary Adult Fontan Clinic.- Echocardiography and adult Fontan patient.- Cardiac MRI imaging in the Fontan patient.- CT in patients with univentricular hearts and a Fontan circulation.- Physiological testing and basics of cardiac catheterisation in Fontan

Dr Clift and Prof Dimopoulos are senior cardiologists with expertise in congenital heart disease (CHD) and pulmonary hypertension (PH). They work in two of the biggest tertiary centres for congenital heart disease in the UK (Birmingham and London) and have cared for thousands of patients with simple and complex congenital heart disease. They are accomplished researchers and educators in the field of CHD. Between them, they have published over 300 papers, addressing important clinical questions and educating the readers in topic such as the Fontan circulation, PH in ACHD, the effects of cyanosis and prognostication in CHD.

 Prof. Angelini is a full professor of Pathology at Padua University with expertise in pathology of fetal, paediatric and adult congenital heart disease. She works in the largest tertiary center for Cardiovascular Pathology and congenital heart disease in Italy. She was trained in CHD at the Brompton hospital. She is involved in many educational activities in CHD around Europe. She has published more than 300 papers in the field of cardiovascular pathology, addressing relevant clinico-morphological correlations.