Granton John T, Rabinovitch Marlene
Department of Medicine, University of Toronto, Division of Respirology and Critical Care Medicine Programme, University Health Network, 10 EN-220, 200 Elizabeth Street, Toronto, Ontario, Canada M5G 2C4.
Cardiol Clin. 2002 Aug;20(3):441-57, vii. doi: 10.1016/s0733-8651(02)00017-6.
Pulmonary arterial hypertension (PAH) is a recognized complication of congenital systemic to pulmonary arterial cardiac shunts. The prognosis of PAH in this situation is better than primary or other secondary forms of PAH. Our knowledge of the pathophysiology of PAH complicating congenital heart disease has evolved over the past decade. Despite differences in etiology and pathobiology, therapies that have proven successful for primary PAH may benefit this group of patients.
肺动脉高压（PAH）是先天性体循环至肺循环心脏分流的一种公认并发症。在这种情况下，PAH的预后优于原发性或其他继发性PAH形式。在过去十年中，我们对先天性心脏病合并PAH的病理生理学的认识有所发展。尽管病因和病理生物学存在差异，但已被证明对原发性PAH有效的治疗方法可能对这组患者有益。
