Update on pulmonary arterial hypertension in children: management strategies and clinical utility of sildenafil

Pulmonary hypertension is an area of growing interest in cardiology, respiratory, and rheumatic diseases due to the increased treatment that is now available to ameliorate the disease process and the improved survival with such medication. The most common cause of pulmonary hypertension is secondary to congenital heart disease and the availability of a medication that is widely used and familiar in clinical practice enables pediatricians to manage this condition. However, there is much to be learned regarding the indications for this medication, for example, in chronic lung disease, and perioperatively in congenital heart disease. It is not yet clear when the therapy should commence, when the disease is mild or when it has progressed. This article summarizes the current state of knowledge of disease therapy concentrating on the use of sildenafil in the pediatric population.