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肺动脉高压(PAH)是许多先天性心脏病常见并发症之一。PAH是否存在及肺血管病理状况很大程度决定原发疾病的临床表现、疾病过程以及外科手术治疗的可能性。在过去40年,尽管人们对肺循环有了较多了解,但对似乎具有相同程度的PAH患儿却出现不同的治疗结果仍感到困惑。70年代血管扩张药物的临床使用使人们受到很大鼓舞,但后来发现口服钙通道阻滞剂并不总是有效。自90年代以来,由于前列环素和一氧化氮(NO)应用于临床,使口服钙通道阻滞剂治疗无效的PAH患儿又看到生存的希望。
Pulmonary hypertension (PAH) is one of the common complications of many congenital heart diseases. The presence or absence of PAH and the pathophysiology of pulmonary vascular disease determine the clinical presentation of the primary disease, the course of the disease, and the likelihood of surgical treatment. In the past 40 years, despite the increased understanding of pulmonary circulation, it remains puzzling to find different treatment outcomes for children with PAH that appear to have the same degree. People are greatly encouraged by the clinical use of vasodilators in the 1970s, but it was later discovered that oral calcium channel blockers were not always effective. Since the 1990s, prostacyclin and nitric oxide (NO) have been used clinically to see the hope of survival in children with PAH who have failed oral calcium channel blocker therapy.