呼吸急促（气促）, 头晕, swelling of the legs and chest pain or pressure are symptoms that could indicate a variety of medical conditions, 包括肺动脉高压. 

This is different from the hypertension people monitor with blood pressure cuffs squeezing their arms. Hypertension is high blood pressure affecting the vessels of the body. Pulmonary hypertension involves just the vessels that carry blood to the lungs.

“Early diagnosis and more aggressive treatment up front increases the survival,上州肺病专家说 Birendra Sah, MD. “肺动脉高压是无法治愈的, but if we diagnose it early and if we treat them with proper drugs, 这可以提高生存率.”

 Pulmonary hypertension develops when blood vessels in the lungs become thickened, 缩小, 阻塞或破坏.

Krithika Ramachandran, MBBS， is a colleague of Sah’s, specializing in critical care and pulmonary diseases. “通常我向病人解释的方式是, 我让他们想象一个气球，气球的末端系着一根吸管. If the diameter of the straw just keeps getting smaller and smaller, that balloon has to be squeezed harder and harder to send flow through the straw.

“That’s essentially what’s happening with the right side of the heart in this situation.”

诊断

A diagnosis can be challenging because the first symptoms are generally nonspecific. 一个人可能会感到呼吸急促或疲倦. As the disease progresses, legs may swell, or the belly may swell as fluid is retained.

Pulmonary hypertension is dangerous because untreated, it leads to congestive heart failure. The chambers of the heart dilate, and their ability to push blood into the lungs diminishes. This reduces the supply of oxygen to the brain and the rest of the body.

适当治疗肺动脉高压, health providers begin by identifying what type of pulmonary hypertension a patient has.

One type, pulmonary arterial hypertension, occurs when the walls of the artery become stiff. 这可能是由于一种基因通过家庭遗传而产生的, 先天性心脏病, 使用某些药物或非法物质, 结缔组织紊乱，如狼疮或硬皮病, 肝脏疾病或人类免疫缺陷病毒感染. Some people develop this condition without ever learning its cause. 

Sah says pulmonary arterial hypertension is typically treated with vasodilator medications, 哪些有助于血管放松. 除了, 病人可能需要补充氧气, 或者用药物稀释血液, 或者是帮助身体排出多余水分的药物. 另一种类型, 慢性血栓栓塞性肺动脉高压, which occurs due to longstanding blood clots in the lung blood vessels, can be treated with surgery to remove the blood clots in select patients, or with vasodilators in patients who are not candidates for surgery.

The other types of pulmonary hypertension are generally treated with supportive care, he says. 这意味着一些病人可能需要补充氧气. 有些人可能需要血液稀释剂. 有些可能需要吸入血管扩张剂. Most benefit from careful measurement of their fluid and salt intake and body weight.

其他问题

People with pulmonary hypertension are managing a disease that can complicate their lives in multiple ways.

“It’s a very precarious line they toe when they’re trying to keep themselves healthy. So anything and everything needs to be evaluated as a risk-benefit ratio before taking it,拉马钱德兰说.

Most patients are taking a medication to help maintain their body’s salt and fluid balance, so they have to be careful about the salt content of their foods and about taking other medications.

如果他们感冒了, they may not be   able to take a decongestant because it can increase pressure in the lungs and make it harder for the heart to work properly. 和 the pain reliever ibuprofen can affect liver function and kidney function.

大多数治疗心房颤动的药物, 一种常见的心律紊乱, 通过减慢心率来锻炼. This can be a particular concern for someone with pulmonary hypertension because it can depress the heart’s already-impaired ability to pump blood.

锻炼也必须谨慎对待. Lifting weights above the head is a no-no because of the stress it puts on the heart. Traveling to high altitudes, or on an airplane, may require the use of supplemental oxygen.

多种原因

The cardiovascular system in a woman with pulmonary hypertension cannot handle the stress of pregnancy and can be life-threatening to her. Also, many of the medications used to treat the disease can cause fetal defects. So women with pulmonary hypertension are advised not to become pregnant.

Pulmonary hypertension caused by left-sided heart disease can be the result of mitral valve or aortic valve disease or failure of the  left ventricle because of a heart attack or another disease.

Pulmonary hypertension caused by lung disease can be the result of chronic obstructive pulmonary diseases including emphysema and bronchitis, 肺纤维化或阻塞性睡眠呼吸暂停. 

Other health conditions — including inflammatory diseases such as sarcoidosis and vasculitis, 血液疾病, 代谢紊乱, 肾脏疾病和其他疾病-可引发肺动脉高压. 和, people with clotting disorders or pulmonary emboli are at risk for developing pulmonary hypertension caused by chronic blood clots.

“The landscape for treating this disease has changed dramatically in the last 10 to 15 years,拉马钱德兰说. 在20世纪90年代, 肺动脉高压的诊断就像是被判了死刑, 超过一半的病人在三年内死亡. “But now, it can be one of those diseases that you die with instead of die from.”

She explains that multiple medications are available today to help dilate the blood vessels and reduce the pressure, 所以心脏可以将血液泵入肺部.

It’s a complicated disease that requires careful medical management.

Sah says patients with pulmonary hypertension keep in close contact with their pulmonologists, 定期进行检查. 

本文发表在 《推荐最近最火的赌博软件》杂志2023年春季号.