肺动脉高压与肺间质性疾病
肺动脉高压, 或PH值, occurs when blood pressure within the lungs becomes abnormally elevated. 它可以由肺动脉壁增厚引起, 心脏衰竭、肺部疾病(如 间质性肺病),以及肺部血管内的血块. It's common for ILD patients to also develop pulmonary hypertension.
PH should not be confused with ordinary hypertension – sometimes called systemic hypertension or high blood pressure. PH可发生在有或无普通高血压的患者.
PH in ILD is also a different disease than primary pulmonary hypertension – also called idiopathic pulmonary arterial hypertension – a rare condition in which PH develops in the absence of lung disease or any other known cause.
症状
PH的症状与ILD相似. 病人 with PH often complain of shortness of breath with physical activity that gradually worsens over time.
患者还可能出现以下症状:
- 胸部疼痛
- 晕厥发作
- 平躺时呼吸困难
- 脚踝肿胀
ILD患者, pulmonary hypertension is usually suspected when these symptoms are out of proportion to the severity of the patient's ILD.
诊断
诊断PH值, your doctor will start by conducting a thorough medical history and physical examination. This will include discussing your other medical problems, which may be associated with PH. 回顾你的用药和暴露情况很重要, since certain diet pills and recreational drugs can increase the likelihood of having PH. 体格检查, which includes listening to your heart sounds and observing your neck veins, 也可能揭示PH值.
Your doctor may also conduct the following tests to make an accurate diagnosis:
- 超声心动图. This is an ultrasound study that provides your doctor with video images of your heart and how it is functioning. We can estimate the blood pressure within your lungs based on the speed at which blood flows through your heart.
- 血液测试. 某些血液检查, specifically for a polypeptide called B-type natriuretic peptide (BNP), 能帮助支持PH的诊断吗. BNP is released into the blood when the heart is under strain, which often occurs as a result of PH.
- 右心导管. 该程序用于明确诊断PH值. 短导管(细导管, 软管)被放置在你的脖子或腹股沟, 然后是一个较小的, 引导较长的导管穿过它. This technique is used to directly measure the blood pressure in your lungs.
- 计算机断层扫描(CT)血管造影. In patients with documented PH, CT angiography may be performed to look for potential causes. 这是一种特殊的 CT扫描 用来研究你肺部的血管. 让血管出现在扫描图上, contrast dye will need to be injected into a vein in your arm at the time of the CT.
- 通气-灌注扫描(V/Q扫描). In patients with documented PH, V/Q scanning may be performed to look for potential causes. A very small amount of radioactive tracer is injected and inhaled to determine whether blood is flowing appropriately to those areas of the lung that are receiving oxygen.
治疗
治疗 f或PH值 can be divided into two main categories: supportive therapy and PH-specific疗法. 支持性疗法 is used to treat the underlying factors which may worsen PH, 为了防止并发症. PH-specific therapies are designed to reduce the blood pressure within the lungs.
Several PH-specific therapies have recently been approved by the U.S. Food and Drug Administration (FDA) for use in patients with other forms of PH – specifically pulmonary arterial hypertension. The efficacy of these therapies in patients with PH and ILD remains uncertain. 在ILD患者中使用ph特异性治疗仍然存在争议, 所以你应该和你的十大赌博平台排行榜讨论一下好处和风险.
支持性疗法
- 氧气. Supplemental oxygen helps increase oxygen supply to the blood and helps prevent narrowing of the pulmonary blood vessels, 会使酸碱度恶化.
- 利尿剂. 利尿剂, 或者水丸, help prevent swelling and are sometimes useful in improving heart and lung function in PH patients.
- 抗凝剂(血液稀释剂). Anticoagulants are sometimes required if blood clots are detected in the lungs or if PH is severe.
- 肺康复. 肺康复 has been shown to improve outcomes in patients with ILD, 以及PH患者. Be sure to tell your pulmonary rehabilitation provider if you have been diagnosed with PH.
PH-specific疗法
- 类前列腺素(Flolan, treprostiil, Iloprost). 前列腺素类, 或环前列腺素, therapy is available as a continuous intravenous infusion or as an inhaled medication. 前列腺素类药物很少用于ILD患者的PH.
- 内皮素受体拮抗剂(波生坦,Ambrisentan). 内皮素受体拮抗剂口服. 病人 who take them must have their liver function monitored regularly.
- 磷酸二酯酶抑制剂(西地那非). 磷酸二酯酶抑制剂可口服. They should not be used in patients taking nitrates (for example, nitroglycerin) for heart disease.
预后
Survival in patients with ILD and PH is worse than in ILD patients without PH. Several research studies are now looking at the role of PH treatment in improving the prognosis for these patients. 如果你是一个很好的候选人 肺移植 ILD和PH逐渐恶化, your doctor may refer you to a specialist for a 肺移植 evaluation.
加州大学旧金山分校健康医学专家已经审查了这些信息. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.