日本小児循環器学会雑誌 第26巻 第3号（206-218） 2010年
Dunbar Ivy1)，Ben T Saji2)
1)Department of Pediatrics, University of Colorado Denver School of Medicine, Denver, Colorado, USA and 2)Department of Pediatrics, Toho University, Medical Center, Tokyo, Japan
Pulmonary arterial hypertension (PAH) is a life-threatening disease whose prognosis has changed dramatically over the past decade since the introduction of new therapeutic agents as well as the off-label application of adult pulmonary hypertension specific therapies to children. Nevertheless, PAH still has no cure and the aim of treatment is to prolong survival by improving quality of life, symptoms, exercise capacity and hemodynamics. The selection of appropriate therapies for PH is complex and must be carefully chosen according to the etiology and pulmonary vasoreactivity. As insight advances into mechanisms responsible for the development of PAH, the introduction of novel therapeutic agents will hopefully further improve the outcome of this incurable disease.
pulmonary hypertension，children，endothelin receptor antagonists，phosphodiesterase-5，prostacyclin
Dunbar Ivy, MD Chief and Selby’s Chair of Pediatric Cardiology, Director, Pediatric Pulmonary Hypertension Program, University of Colorado Denver School of Medicine, The Children’s Hospital 13123 East 16th Avenue, B100, Aurora, Colorado 80045, USA E-mail: email@example.com