Cardiovascular Research Center, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, 149 13th Street, Charlestown, MA 02129, USA.
Heart Fail Clin. 2010 Apr;6(2):215-22. doi: 10.1016/j.hfc.2009.11.007.
When pulmonary hypertension (PH) and right ventricular dysfunction accompany heart failure, the impact on functional capacity and prognosis are ominous. Newer clinical strategies to preferentially lower pulmonary pressures and pulmonary vascular tone improve functional performance and symptoms of heart failure by targeting the nitric oxide signal transduction pathways, as with PDE5 inhibition. Additional studies are needed to determine if these therapies will impact long-term patient outcomes and elucidate the specific mechanisms whereby these treatments are effective. Furthermore, the recent finding that mutations in BMPR2 cause familial forms of pulmonary arterial hypertension and that BMPR2 expression is decreased in secondary forms of PH strongly implicate BMP signaling in the underlying pathophysiology of PH. Translation of emerging basic science insights in the vascular biology of PH and BMP signaling will provide novel therapeutic strategies for the spectrum of pulmonary hypertensive diseases.
当肺动脉高压 (PH) 和右心室功能障碍伴随心力衰竭时，对功能能力和预后的影响是不祥的。新的临床策略优先降低肺动脉压和肺血管张力，通过靶向一氧化氮信号转导途径改善心力衰竭的功能表现和症状，如 PDE5 抑制。需要进一步的研究来确定这些治疗方法是否会影响患者的长期预后，并阐明这些治疗方法有效的具体机制。此外，最近的研究发现 BMPR2 突变导致家族性肺动脉高压，而在继发性 PH 中 BMPR2 的表达降低，这强烈提示 BMP 信号在 PH 的潜在病理生理学中起作用。PH 的血管生物学和 BMP 信号的新兴基础科学研究成果的转化将为各种肺动脉高压疾病提供新的治疗策略。
