Pulmonary hypertension
Pulmonary hypertension
复制标题
DOI：
10.1001/jama.284.24.3160
复制
发表时间：
2000-12-27
影响因子：
120.7
通讯作者：
Gaine, S
中科院分区：
文献类型：
--
作者：
Gaine, S
临床上有用的基于治疗的肺动脉高压分类将疾病分为5个不同的类别：（1）与呼吸系统疾病和/或低氧血症相关的肺动脉高压;（2）肺静脉高压;（3）慢性血栓栓塞性疾病;（4）肺动脉高压;（5）肺动脉高压;（6）肺动脉高压;（7）肺动脉高压;（8）肺动脉高压;（9）肺动脉高压;（10）肺动脉高压;（11）肺动脉高压;（12）肺动脉高压;（13）肺动脉高压;（14）肺动脉高压;（15）肺动脉高压;（16）肺动脉高压;（17）肺动脉高压;（18）肺动脉高压;（19）肺动脉高压;（1和（5）由于直接影响肺脉管系统的疾病引起的肺动脉高压。肺动脉高压包括患有原发性肺动脉高压、先天性心脏病、结缔组织病和肝脏疾病。这些异质性疾病具有相似的特征性病理变化，包括原位血栓形成、平滑肌肥大和内膜增生。右心导管插入术对确诊、判断预后和分配治疗方案至关重要。少数患者对急性血管扩张剂试验有良好反应，并对钙通道阻滞剂治疗有长期获益。持续静脉注射依前列醇可改善晚期原发性肺动脉高压患者的症状和生存率，并对其他形式的肺动脉高压具有潜在获益。肺移植仍然是最大限度的药物治疗失败的个人的重要选择。最近发现了家族性原发性肺动脉高压的基因，正在进行临床试验的新药也在增加，这些都是令人鼓舞的进展。
A clinically useful, treatment-based classification of pulmonary hypertension divides the disease into 5 distinct categories: (1) pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia; (2) pulmonary venous hypertension; (3) chronic thromboembolic disease; (4) pulmonary arterial hypertension; and (5) pulmonary hypertension due to disorders directly affecting the pulmonary vasculature, Pulmonary arterial hypertension includes individuals with primary pulmonary hypertension, congenital heart disease, connective tissue disease, and liver disease. These heterogeneous diseases have similar characteristic pathological changes, including in situ thrombosis, smooth muscle hypertrophy, and intimal proliferation. Right heart catheterization is essential to confirm diagnosis, determine prognosis, and assign therapy. A minority of patients have a favorable response to an acute vasodilator trial and long-term benefit with calcium channel blocker therapy. Continuous intravenous epoprostenol improves symptoms and survival in patients with advanced primary pulmonary hypertension and has potential benefit in other forms of pulmonary arterial hypertension. Lung transplantation remains an important option for individuals in whom maximal medical therapy fails. The recent discovery of the gene for familial primary pulmonary hypertension and the increase in new drugs undergoing clinical trials are encouraging developments.