Pulmonary Vascular Disease Program, Yale University School of Medicine, 15 York Street, LCI 105-C, New Haven, CT 06510, USA.
Ther Adv Respir Dis. 2012 Jun;6(3):147-59. doi: 10.1177/1753465812441849. Epub 2012 Mar 21.
Pulmonary arterial hypertension is a progressive and incurable disease. Over the past two decades, significant advances have been made in understanding and thus managing this disease. Multiple therapeutic options are currently available and optimizing the treatment of pulmonary arterial hypertension has become complex. Patients who meet the American College of Chest Physicians criteria for vasoresponsiveness can be safely and effectively treated with high-dose calcium channel blockers but require close follow up to assure durability of response. Patients with World Health Organization (WHO) functional class IV status and those with determinants of high risk for progression and death should be treated with an infused prostanoid agent without delay. These patients should also be referred early after stabilization for transplant evaluation. Patients with WHO functional class II status benefit from early initiation of oral therapies. Those with WHO functional class III status and lower determinants of risk for progression may receive treatment with one or more oral or inhaled agents, though many experts would advise early use of infused prostanoids for these patients as well.
肺动脉高压是一种进行性且无法治愈的疾病。在过去的二十年中，人们在理解和治疗这种疾病方面取得了重大进展。目前有多种治疗选择，优化肺动脉高压的治疗变得复杂。符合美国胸科医师学会（ACCP）血管反应性标准的患者可以安全有效地使用高剂量钙通道阻滞剂进行治疗，但需要密切随访以确保反应的持久性。世界卫生组织（WHO）功能分级为 IV 级的患者和有进展和死亡高风险因素的患者应立即使用输注前列环素药物进行治疗。这些患者在稳定后也应尽早转介进行移植评估。WHO 功能分级为 II 级的患者从早期开始口服治疗中获益。那些 WHO 功能分级为 III 级且进展风险较低的患者可以接受一种或多种口服或吸入药物治疗，但许多专家会建议这些患者也尽早使用输注前列环素药物。
