Götting M, Addicks J Ph, Uibel St, Jensen A-M, Groneberg D A, Quarcoo D
Aus dem Institut für Arbeitsmedizin, Charité--Universitätsmedizin Berlin.
Versicherungsmedizin. 2010 Mar 1;62(1):9-15.
Pulmonary hypertension (PH) is a disease which is characterised by an increase in the mean pulmonary arterial pressure (mPAP) in the lung circulation of over 25 mmHg in rest and over 30 mmHg in movement. Due to the chronic overload of the right ventricle, the heart is always affected by a PH and often develops a so-called cor pulmonale chronicum which can lead to right-heart failure. There are five groups in the clinical WHO Venice classification which are arranged according to pathogenetical, clinical and therapeutical criteria. In addition, an adjusted NYHA classification helps to grade the significance of the disease stages. Principally, one classifies a mostly isolated form of the pulmonary arterial hypertension (PAH) and other secondary forms of the PH which develop on the grounds of existing problems such as left-heart diseases, hypoxic lung diseases, pulmonary embolism and infections. The pathophysiological reasons for a PH are just as various as the different manifestations. Yet there are generally four main alterations in the walls of the pulmonary vessels. This includes vasoconstriction, rarefaction of vessels, vascular remodelling and the occlusion of vascular lumen by a thrombus with subsequent structural remodelling of the vascular and mounted extracellular matrix. The diagnostic procedure should be algorithm-oriented and includes anamnesis, physical examination, electrocardiogram (ECG), thoracic x-ray and echocardiography. To confirm the diagnosis and for a better measuring of the prognosis, an examination with a right-heart flow-directed balloon-tipped catheter is favourable. Because of the change in the pathophysiological concepts of the PH from a vasoconstrictive to a vasoproliterative genesis, additional pharmacological targets are developed for therapeutic treatment. Today the former regime of therapy with high-dosed calcium-channel blockers such as vasodilatators only finds application after pharmacological testing at so-called responders. The current scheme of therapy is focused on the synergic effects of different drugs, such as prostacyclines, endothelial-receptor blockers and phosphodiesterase-5 inhibitors. After the failure of pharmacological treatments, the endarteriectomy remains as the last therapy option, although it is accompanied by poor survival rates.
肺动脉高压（PH）是一种疾病，其特征是肺循环中平均肺动脉压（mPAP）在静息时超过25 mmHg，运动时超过30 mmHg。由于右心室长期负荷过重，心脏总是受到肺动脉高压的影响，并常常发展为所谓的慢性肺源性心脏病，进而可能导致右心衰竭。世界卫生组织（WHO）威尼斯临床分类中有五个组，它们是根据发病机制、临床和治疗标准进行排列的。此外，修订后的纽约心脏协会（NYHA）分类有助于对疾病阶段的严重程度进行分级。原则上，可将肺动脉高压（PAH）的一种主要为孤立形式以及基于诸如左心疾病、低氧性肺疾病、肺栓塞和感染等现有问题而发展的其他继发性肺动脉高压形式区分开来。肺动脉高压的病理生理原因与不同表现形式一样多种多样。然而，肺血管壁通常存在四种主要改变。这包括血管收缩、血管稀疏、血管重塑以及血栓导致的血管腔阻塞，随后是血管和附着的细胞外基质的结构重塑。诊断程序应以算法为导向，包括病史采集、体格检查、心电图（ECG）、胸部X线检查和超声心动图检查。为了确诊并更好地评估预后，采用右心漂浮导管进行检查是有利的。由于肺动脉高压的病理生理概念已从血管收缩性起源转变为血管增殖性起源，因此开发了额外的药物靶点用于治疗。如今，以前使用高剂量钙通道阻滞剂（如血管扩张剂）的治疗方案仅在对所谓的反应者进行药物测试后才会应用。当前的治疗方案侧重于不同药物（如前列环素、内皮受体阻滞剂和磷酸二酯酶-5抑制剂）的协同作用。在药物治疗失败后，尽管内膜剥脱术的生存率较低，但它仍是最后的治疗选择。
