Guest Editor's Memo

This issue of Advances in Pulmonary Hypertension focuses on the management of patients with congenital heart disease and associated pulmonary hypertension. More than a million adults in the United States have congenital heart defects, and adults now outnumber children with congenital heart defects. Many of these patients present complex cases with unique anatomical defects and very complicated interplay between pulmonary blood flow and pulmonary vascular resistance. Up to 40% of congenital heart patients are at risk for developing pulmonary hypertension and up to 10% actually develop it. Half of these can progress to Eisenmenger syndrome, a condition resulting in profound cyanosis from venous to systemic blood flow, when shunt lesions go unrecognized and untreated.

The goal of the following articles is to provide a broad overview of the congenital heart lesions most likely to result in pulmonary vascular disease, so the pulmonary hypertension specialist can become aware of the presenting features and the unique management strategies required. A multitude of treatments are now available for these patients, including medical therapies targeting the pulmonary vasculature, percutaneous devices that can be used to close abnormal intracardiac and vascular communications, balloons and stents that can be used to increase blood flow when the circulation is com promised, and even catheter-based valve prostheses that can be implanted without requiring surgery. A variety of surgical procedures also exist that can target the many different heart defects and valve abnormalities and dramatically alter the natural history of these disorders. Often what is utilized is a hybrid technique with several different specialists working together to improve the quantity and quality of life for this challenging patient population. It is indeed an exciting time to provide care for this constantly expanding group of patients!