Helgeson Scott A, Enderby Cher Y, Moss John E, Gass Jennifer M, Zeiger Tonya K, Burger Charles D
Department of Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, FL.
Department of Pharmacy, Mayo Clinic, Jacksonville, FL.
Mayo Clin Proc Innov Qual Outcomes. 2019 Jul 19;3(3):376-379. doi: 10.1016/j.mayocpiqo.2019.06.002. eCollection 2019 Sep.
Pulmonary arterial hypertension is a progressive vascular disease with a high mortality rate without proper therapy. Identification of the appropriate treatment for each patient is critical in regard to adverse effects, health care costs, ease of treatment, and the potential for prognostication. Treatment strategies typically begin with acute vasoreactivity testing, which is performed during a right heart catherization. If positive, a calcium channel blocker may work; however, another pulmonary arterial hypertension-specific medication is necessary when testing is negative. Acute vasoreactivity testing is currently recommended to be performed only in certain subgroups of pulmonary arterial hypertension, but not when related to connective tissue disease. In this report, we describe a patient who had systemic sclerosis-related pulmonary arterial hypertension with a positive acute vasoreactivity test result. The patient was placed on calcium channel blocker monotherapy that has been well tolerated for 12 years, resulting in improved symptoms and exercise capacity. The long-term response to calcium channel blocker therapy in systemic sclerosis-associated pulmonary arterial hypertension has not been previously described. In addition, pulmonary artery pressures have been well controlled. The absence of genetic smooth muscle variants prevalent in vasoresponsive idiopathic pulmonary arterial hypertension is also unique.
肺动脉高压是一种进行性血管疾病，若未得到恰当治疗，死亡率很高。就不良反应、医疗费用、治疗便利性及预后可能性而言，为每位患者确定合适的治疗方法至关重要。治疗策略通常始于急性血管反应性测试，该测试在右心导管插入术期间进行。如果测试结果为阳性，钙通道阻滞剂可能有效；然而，测试结果为阴性时，则需要使用另一种肺动脉高压特异性药物。目前建议仅在某些肺动脉高压亚组中进行急性血管反应性测试，但与结缔组织病相关时不建议进行。在本报告中，我们描述了一名患有系统性硬化症相关肺动脉高压且急性血管反应性测试结果为阳性的患者。该患者接受了钙通道阻滞剂单一疗法，12年来耐受性良好，症状和运动能力均有所改善。此前尚未描述过系统性硬化症相关肺动脉高压患者对钙通道阻滞剂治疗的长期反应。此外，肺动脉压力得到了很好的控制。血管反应性特发性肺动脉高压中普遍存在的遗传性平滑肌变异的缺失也很独特。
