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高血压

Overview

肺动脉高压 is a rare and very serious blood vessel disorder of the lung. Primary pulmonary hypertension (PPH) – which is pulmonary hypertension that isn't a side effect of (or secondary to) another condition such as 肺气肿 or lupus – strikes only between one and two people per million.

Managing this illness and its complications, 包括右心衰, requires expertise and access to sophisticated treatment such as oral and continuous intravenous medication, 肺移植. 治疗 for pulmonary hypertension can be highly complex and require intensive ongoing follow-up, all of which are available through our 肺 高血压 Program.

Our approach to pulmonary hypertension

UCSF has been at the forefront of evaluating and treating pulmonary hypertension (high blood pressure in the lungs) for more than 30 years. We care for patients with either primary pulmonary hypertension, 自行发生的情况, 或者继发性肺动脉高压, which is a side effect of another disease and is often misdiagnosed. Our hospital is one of only three medical centers on the West Coast designated a comprehensive center of care by the 肺 高血压 Association.

Managing this rare illness and its complications requires the expertise and sophisticated care that UCSF can provide. Our patients have access to the full range of treatments, 包括口语, inhaled and catheter-based therapies, as well as experimental approaches being evaluated in clinic trials.

In some cases, the best option may be a lung transplant. UCSF performs 40 to 50 lung transplants each year and leads the nation in higher-than-expected rates of patient survival and transplant function.

奖 & 识别

  • usnews-neurology

    Among the top hospitals in the nation

  • usnews-pulmonology

    No. 肺病学全国排名第八 & 肺部手术

  • One of the nation's best in cardiology and heart & 血管手术

  • PHA-2x

    Accredited center of comprehensive care (肺 高血压 Association)

迹象 & 症状

肺动脉高压 is classified into five different types:

  • 肺 arterial hypertension (PAH). This form affects blood vessels in the lungs that carry blood from the heart to the lungs where it picks up oxygen. This category is divided into two types:
    • Primary pulmonary hypertension (PPH) is very rare and can occur for no known reason. In about 10 percent of cases, it's inherited. It most often occurs in young adults and is more than twice as common in women as men.
    • PAH related to exposure to toxins including diet drugs such as fenphen; street drugs including cocaine and methamphetamine; HIV; collagen vascular diseases including scleroderma, lupus and rheumatoid arthritis; chronic liver disease; and congenital heart diseases.
  • 肺静脉高压(PVH). This form is caused by diseases of the left side of the heart, such as heart failure or mitral valve disease. This can increase pulmonary artery blood pressure but usually doesn't become severe PAH.
  • 呼吸系统. 肺动脉高压 can be associated with diseases of the respiratory system including interstitial lung disease, 肺气肿, 哮喘支气管炎, sleep apnea and chronic exposure to high altitude.
  • 慢性血栓. 血凝块 in the lung blood vessels
  • 血管疾病. 肺动脉高压 due to disorders directly affecting the blood vessels in the lungs such as parasites, or inflammation of the blood vessels.

There are no specific signs and 症状 in the early stages of the disease. The 症状 that may occur – such as shortness of breath, fatigue and chest pain – also are common to many other conditions. Other 症状 that may occur include dizziness, 脚踝和腿部肿胀, fainting and a bluish cast to lips and skin.

随着时间的推移, pulmonary arterial hypertension can damage your heart to the point of danger and result in complications that can interfere with your daily life. Complications from pulmonary hypertension include:

  • 右心室增大
  • 血凝块

诊断

肺动脉高压 in its early stages is difficult to recognize on routine physical exam. It may be two or three years after onset that 症状 become severe enough to be noticed.

肺动脉高压 is diagnosed primarily with an echocardiogram, which is an ultrasound examination of the heart. The echocardiogram measures the heart's size and shape by using sound waves to create an image of the heart and can estimate the pulmonary artery pressure.

  • Electrocardiogram (ECG or EKG) to measure your heart's electrical function
  • Right heart catheterization to measure blood pressure inside the heart and blood vessels of the lungs and measure blood flow. This test is performed in all patients to confirm the diagnosis and to help guide therapy.
  • 六分钟步行测验
  • Assessment of how well you accomplish daily living tasks

If your doctor determines that you have pulmonary hypertension, the next task is to find out what is causing it. The tests to determine the cause include:

  • 电脑断层摄影术, 叫做CT或CAT, scan to see if there is a blood clot or other disease in your lungs
  • 肺 function test to rule out obstructive lung disease
  • Sleep study to see if you have sleep apnea, a condition in which you may stop breathing during sleep
  • Lab tests to rule out hepatitis, collagen disease, HIV or other conditions

If no discernible cause for the pulmonary hypertension is found, then the diagnosis of primary pulmonary hypertension is made. If a cause is elucidated, then the diagnosis is secondary pulmonary hypertension.

The New York 心 Association and the World Health Organization (WHO) use the following system to classify pulmonary arterial hypertension.

肺动脉高压 is divided into four classifications:

  • 类1. You've been diagnosed as having the disease but show no 症状. Ordinary physical activity doesn't cause undue discomfort.
  • 二班. Your 症状 occur only with activity but not when you're at rest
  • 3班. There are no 症状 at rest but they occur with even slight exertion
  • 第4类. 你休息的时候也有症状

治疗

The treatment f或者继发性肺动脉高压 usually begins with treating the underlying cause. 例如, 如果是血凝块, 或者肺栓塞, 导致高血压, the clot may need to be surgically removed.

Several treatments are used to manage primary pulmonary hypertension, although they don't cure the condition. 这些包括:

  • 钙通道阻断药物. These help the heart pump better by relaxing muscles in the walls of blood vessels.
  • Endothelin receptor antagonists (ERAs). These medications reverse the effects of endothelin, a substance in blood vessels that causes constriction.
  • 持续输注丙烯醇. This drug dilates and reverses thickening of the blood vessels of the lungs, and helps prevent platelets from clotting the blood. It has to be administered continuously via an implanted catheter and a portable, 电池驱动泵. The drug lasts less than six minutes, which is why it must be infused continuously into the bloodstream.
  • 抗凝血剂. These drugs help keep blood from clotting, which makes it flow better.
  • 利尿剂. Prescribed to minimize water retention, a condition that makes the heart work harder.
  • 地高辛. This drug can help the right side of the heart pump better.
  • 生活方式的改变. Adequate rest, a healthy diet, exercise and stress reduction can help control the disease. People with pulmonary hypertension should avoid smoking, 怀孕, birth control pills and high altitudes.
  • 补充氧气. This is used for patients with low oxygen level in the blood.
  • 肺或心肺移植. These procedures are employed for patients with severe pulmonary hypertension and extremely poor quality of life. 这是最后的手段.

加州大学旧金山分校健康 medical specialists have reviewed this information. 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.

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