Pulmonary Hypertension in Children: The Twenty-First Century

Understanding of idiopathic pulmonary hypertension, and pulmonary hypertension in general, has progressed over the years. A more succinct understanding of the pathophysiology of this disease has elucidated its mechanisms and facilitated a rational approach to its management. This approach has provided hope for patients who had a disease that was associated with significant morbidity and mortality shortly after diagnosis. Medications continue to evolve, providing this group of patients an improved outlook. Newer medications, especially in oral form, will continue to be developed and future studies will include combination medical therapy. Finally, the genomic approach to the study of pulmonary hypertension, including the identification of candidate genes, may allow manipulation of selected genes, further the understanding of the pathobiology of pulmonary hypertension, and allow this devastating disease to someday be prevented. Although recent advances have provided more options, further work is necessary to understand the role of the new treatment options and devise a rational approach to this disease entity.