Reichenberger F, Voswinckel R
Abteilung Pneumologie, Medizinische Klink II, Universitätsklinikum Gießen Marburg GmbH, Gießen.
Internist (Berl). 2011 Apr;52(4):405-14; quiz 415. doi: 10.1007/s00108-011-2810-y.
Pulmonary arterial hypertension is a chronic disorder of the pulmonary circulation characterized by an increased pulmonary artery pressure and an impaired cardiac function. It can present as idiopathic form or associated with several systemic diseases. Typical symptoms include progressive dyspnoea and exercise intolerance. When pulmonary arterial hypertension is suspected, echocardiography is the key investigation for noninvasive assessment of the disease. The diagnosis requires an invasive measurement of pulmonary hemodynamics by right heart catheterization. Treatment is based on general measures and application of pulmonary vasoactive compounds, such as prostanoids, endothelin receptor blockers and phosphodiesterase 5 inhibitors. These treatment options can be combined at progression of the disease. Lung transplantation might be an option in patients refractory to medical therapy.
肺动脉高压是一种肺循环的慢性疾病，其特征为肺动脉压力升高和心功能受损。它可表现为特发性形式或与多种全身性疾病相关。典型症状包括进行性呼吸困难和运动不耐受。当怀疑有肺动脉高压时，超声心动图是对该疾病进行无创评估的关键检查。诊断需要通过右心导管插入术对肺血流动力学进行有创测量。治疗基于一般措施以及应用肺血管活性化合物，如前列环素、内皮素受体拮抗剂和磷酸二酯酶5抑制剂。在疾病进展时，这些治疗选择可以联合使用。对于药物治疗无效的患者，肺移植可能是一种选择。
