Houtchens Jeanne, Martin Douglas, Klinger James R
Division of Pulmonary, Sleep and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, RI 02903, USA.
Pulm Med. 2011;2011:845864. doi: 10.1155/2011/845864. Epub 2011 Sep 20.
Pulmonary arterial hypertension is a rare disease, which requires a high index of suspicion to diagnose when patients initially present. Initial symptoms can be nonspecific and include complaints such as fatigue and mild dyspnea. Once the disease is suspected, echocardiography is used to estimate the pulmonary arterial (PA) pressure and to exclude secondary causes of elevated PA pressures such as left heart disease. Right heart catheterization with vasodilator challenge is critical to the proper assessment of pulmonary hemodynamics and to determine whether patients are likely to benefit from vasodilator therapy. Pathologically, the disease is characterized by deleterious remodeling of the distal pulmonary arterial and arteriolar circulation, which results in increased pulmonary vascular resistance. In the last fifteen years, medications from three different classes have been approved for the treatment of pulmonary arterial hypertension. These include the prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors.
肺动脉高压是一种罕见疾病，患者初诊时需要高度怀疑才能确诊。初始症状可能不具特异性，包括疲劳和轻度呼吸困难等主诉。一旦怀疑患有该病，会使用超声心动图来评估肺动脉（PA）压力，并排除PA压力升高的继发性原因，如左心疾病。进行血管扩张剂激发试验的右心导管检查对于正确评估肺血流动力学以及确定患者是否可能从血管扩张剂治疗中获益至关重要。在病理上，该病的特征是远端肺动脉和小动脉循环的有害重塑，这会导致肺血管阻力增加。在过去十五年中，三类不同的药物已被批准用于治疗肺动脉高压。这些药物包括前列环素类、内皮素受体拮抗剂和磷酸二酯酶-5抑制剂。
