Surgery in congenital heart defects: current developments and a few case examples

Congenital heart surgery includes the palliative treatment and surgical complete repair of cardiac malformations in newborns, children and adolescents. Palliative surgery allows early or long-term survival, depending on the primary malformation and the condition of the patient. Confection of a systemic-to-pulmonary shunt (the modified Blalock-Taussig shunt) allows in general recovery from severe cyanosis and leads to development of the hypoplastic pulmonary vascular tree in newborns with severe pulmonary stenosis. Longterm palliation can be applied to all patients in whom establishment of a biventricular heart can not be realized. The total cavo-pulmonary connection represents one possibility to bypass a single ventricle in these complex cases. Total repair allows the restitution of a completely normal anatomy and physiology after surgery; usually, this type of surgery is followed by a normalization of life expectancy with minimal pharmacotherapy. Preoperative diagnosis of congenital heart disease is reasonably performed by transthoracic echocardiography in the majority of cases. Cardiac catheterism is reserved for complex cases and those in which full hemodynamic evaluation is required for proper planning of surgery. Continuous improvement has been realized in the fields of cardiac anesthesiology and pediatric intensive care during the last decade; hence more and more complex cases have been accepted without any negative effect on the operative mortality. Additionally there has been a number of improvements in surgical and perfusion techniques, thus allowing open heart surgery in newborns with a minimal weight of 2000 g. Intraoperative transesophageal echocardiography is performed routinely and allows the proper control of surgical repair; furthermore this examination may be helpful during the weaning period from the extracorporeal circulation. There is a number of interesting topics that will take importance or will be developed in the near future: the role of interventional cardiology in pediatric patients gains more and more importance. There is a potential for minimally-invasive surgery and the number of potential candidates for heart transplantation may increase, due to the fate of long-term survivors after palliative surgery.