McGoon M D, Vlietstra R E
Mayo Clin Proc. 1984 Oct;59(10):672-7. doi: 10.1016/s0025-6196(12)62055-2.
Primary pulmonary hypertension is an uncommon but serious disease that often results in debilitating symptoms and early death. One approach to treatment has been to attempt reduction of pulmonary arterial pressure and right ventricular afterload by using vasodilator agents to decrease pulmonary arteriolar resistance. Use of a variety of vasodilators has yielded only limited and infrequent success. Although an occasional patient may respond to vasodilator therapy with improved pulmonary hemodynamics and symptomatic status, most patients do not. Reasons for lack of success include the presence of predominantly fixed vascular obstructive disease rather than active vasoconstriction, inexorable progression of disease, and adverse nonpulmonary vascular effects of vasodilators, such as systemic hypotension. Because of occasional beneficial responses, a trial of vasodilators is warranted in patients with primary pulmonary hypertension. Initiation of drug therapy should be undertaken during hemodynamic monitoring in order to allow assessment of response, identification of adverse effects, and comparison of different agents. No vasodilator is clearly superior in primary pulmonary hypertension.
原发性肺动脉高压是一种罕见但严重的疾病，常导致使人衰弱的症状和早期死亡。一种治疗方法是尝试通过使用血管扩张剂降低肺小动脉阻力，以降低肺动脉压和右心室后负荷。使用各种血管扩张剂仅取得了有限且不常见的成功。尽管偶尔有患者可能对血管扩张剂治疗有反应，肺血流动力学和症状状态有所改善，但大多数患者并非如此。治疗失败的原因包括主要存在固定性血管阻塞性疾病而非活动性血管收缩、疾病的不可阻挡进展以及血管扩张剂的不良非肺血管效应，如系统性低血压。由于偶尔有有益反应，对于原发性肺动脉高压患者，进行血管扩张剂试验是有必要的。药物治疗应在血流动力学监测期间开始，以便评估反应、识别不良反应并比较不同药物。在原发性肺动脉高压中，没有一种血管扩张剂明显更具优势。
