Pulmonary Hypertension in Pediatric and Adult Congenital Heart Disease: Deciding When and When Not To Repair. An Overall Perspective of Latin America and the Caribbean Countries

Congenital heart defects are very prevalent in the world, affecting 1 out of 100 live births. In Latin America and the Caribbean, more than 57,000 children are born with a congenital heart defect each year, 45% of which are moderately and highly complex. Globally, 6-8% of congenital heart defects may develop pulmonary hypertension and, of these, 50% will develop the most extreme form, Eisenmenger syndrome. These consequences depend on the type of congenital heart defect and the timing of surgical and/or hemodynamic repair, and are more prevalent in less developed countries. Latin America and the Caribbean are estimated to have more than 200,000 cases of Eisenmenger syndrome. Pulmonary arterial hypertension is the common and final outcome of various congenital heart defects. Timely diagnosis, hemodynamic analysis and specialized treatment help in deciding on medical treatment and surgical repair. This article reviews the definition, classification pathophysiology and hemodynamic diagnostic approach to pulmonary hypertension related to congenital heart defects. It provides a narrative description of the situation in Latin America and the Caribbean. Finally, it provides a general overview of medical treatment concepts and a brief description of Eisenmenger syndrome.