ROLE OF PHARMACOLOGICAL TESTS IN THE TREATMENT OF PRIMARY PULMONARY-HYPERTENSION
ROLE OF PHARMACOLOGICAL TESTS IN THE TREATMENT OF PRIMARY PULMONARY-HYPERTENSION
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DOI：
10.1016/s0002-9149(99)80384-1
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发表时间：
1995-01-19
影响因子：
2.8
通讯作者：
MAGNANI, B
中科院分区：
文献类型：
--
作者：
GALIE, N;USSIA, G;MAGNANI, B
原发性肺动脉高压（PPH）是一种病因不明的罕见疾病，其特征是不断向右心室衰竭和死亡发展。血管收缩是PPH患者肺血管阻力（PVR）和肺动脉压（PAP）升高的病理生理因素之一。因此，血管扩张剂治疗被认为是治疗这种疾病的合乎逻辑的方法之一。在心导管插入术期间，推荐使用短效、可滴定的血管扩张剂进行急性药物挑战，以选择最有可能对长期治疗有反应的患者。需要遵循准确的方法指南，以尽量减少PAP和肺动脉阻力的自发变异性。药理学试验的病理生理学解释需要分析右心室液压蟾蜍的两个组成部分，即肺血管床的阻力和顺应性。只有当PVR使用临界开放压力评估或与PAP同时降低相关时，计算出的PVR的降低才可被认为是肺血管舒张的证明。只有那些PVR下降大于或等于20%与PAP下降大于或等于20%相关的患者才应被认为是急性试验的“应答者”。在临床研究中，只有20-30%的患者有短期反应。研究最深入的短效药物是前列环素，但也使用了其他药物，如乙酰胆碱、腺苷和一氧化氮。前列环素已被证明可预测肺血管扩张剂反应
Primary pulmonary hypertension (PPH) is a rare disease of unknown etiology characterized by a constant progression toward right ventricular failure and death. Vasoconstriction is 1 of the pathophysiologic factors responsible for the increase of pulmonary vascular resistance (PVR) and pulmonary artery pressure (PAP) in patients with PPH. Thus vasodilator treatment is considered 1 of the logical approaches to medical therapy of such a condition. Acute drug challenge with a short-acting, titratable vasodilator during heart catheterization is recommended to select patients who are most likely to respond to longterm treatment. Accurate methodologic guidelines need to be followed to minimize the spontaneous variability of PAP and pulmonary arteriolar resistance. Pathophysiologic interpretation of pharmacologic trials requires analysis of the 2 components of the right ventricular hydraulic toad, i.e., resistance and compliance of the pulmonary vascular bed. Reduction of the calculated PVR may be considered as a demonstration of pulmonary vasodilation only if PVR is assessed using the critical opening pressure or if it is associated with a simultaneous reduction of PAP. Only those patients in whom a reduction of PVR of greater than or equal to 20% is associated with a decrease in PAP of greater than or equal to 20% should be considered as ''responders'' to the acute tests. In clinical studies only 20-30% of the patients are short-term responders. The most intensively studied short-acting drug for shortterm challenge is prostacyclin, but other agents such as acetylcholine, adenosine, and nitric oxide have been utilized. Prostacyclin has been shown to predict pulmonary vasodilator response