Eisenmenger syndrome is a heart condition that can occur as a long-term side effect of an uncorrected heart defect at birth. According to the Adult Congenital Heart Association ，这种情况的名称来自确定情况的Victor Eisenmenger博士。

Eisenmenger syndrome can have serious symptoms and cause an increase in deaths, especially due to a higher chance of stroke. Keep reading to find out more about this condition and its treatments.

Eisenmenger综合征可能是由于出生时心脏的结构不规则性而发生的，可能未被诊断或未矫正。它通常是心室间隔缺陷（VSD），它是心脏中的一个孔。

Blood usually flows from the right side of your heart to the left. The VSD allows blood to flow from the left side of the heart to the right. This backward flow prevents the heart from working as effectively and can lead topulmonary artery hypertension.

A VSD is a type of “shunt,” which indicates that blood isn’t going where you expect it to. Other known shunts can cause Eisenmenger syndrome. These include:

A person can have one or multiple heart defects. Some of these defects can be so small they’re very difficult for a doctor to detect at an early age, when the defects are often most treatable.

Currently, doctors don’t know what causes the heart defect that leads to Eisenmenger syndrome. Researchers haven’t identified any responsible inherited component or gene mutations.

谁有艾森曼格综合症的风险？

Men and women experience Eisenmenger syndrome in equal numbers, and an estimated25 percent to 50 percentof people with Down syndrome have Eisenmenger syndrome.

可以防止Eisenmenger综合征吗？

Often, a heart defect can be most treatable at an early time of diagnosis. This allows medical professionals to repair the heart defect at a young age before it causes complications. Because of enhanced detection and repair, the occurrence of Eisenmenger syndrome decreased from 8％至4％ between the 1950s to the 2000s.

Eisenmenger综合征会引起人们的几种症状。如果您的症状与其他诊断相同的人的症状不像其他人的症状并不罕见。

主要症状通常与cyanosis, a lack of oxygen in the body, and high pressure in the lungs. Some manifestations of this include:

有些患有Eisenmenger综合征的人可能由于病情而引起并发症。这些include增加的机会：

Eisenmenger综合征的人也可以体验 organ damage if their organs do not get enough oxygen.

Doctors do not recommend that people with Eisenmenger syndrome get pregnant. Pregnancy places considerable demands on the heart and lungs. If a birthing parent has pulmonary artery hypertension and congestive heart disease because of Eisenmenger syndrome, they could face a high chance of death during pregnancy.

According to a2016 review，Eisenmenger综合征患者怀孕期间的死亡率从30％到50％不等，需要剖宫产的人高达65％。与怀孕和艾森曼格综合症有关的最常见死亡原因是：

  • 心脏衰竭
  • endocarditis
  • blood clots that can lead to stroke

It’s possible to get pregnant without knowing you have Eisenmenger syndrome, or perhaps you truly desire to become pregnant. In either case,finding a multidisciplinary care team可以帮助您管理状况。您可能需要考虑包括以下类型的医疗专业人员：

  • obstetrician
  • 肺科医生
  • 心脏病专家
  • neonatologist

If you have Eisenmenger syndrome, it’s important to talk with your doctor about birth control options . Some birth control methods, such as pills that contain estrogen, may increase your chance of blood clots. So, be sure to avoid those.

尚无确切的测试来诊断Eisenmenger综合征。医生可以使用多种工具来查看一个人是否有病情。这些测试的示例包括：

If a doctor identifies the VSD and higher pressure in the lungs, this may lead to a diagnosis. The condition can be similar to other medical conditions, including:

It is important to know that once high pressure in the lungs starts to cause symptoms, the effects are usually difficult to reverse. Currently, the only cure for Eisenmenger syndrome is a heart and lung transplant. But doctors reserve this intervention for the most severe cases.

In many cases, doctors focus on managing the condition and preventing it from getting worse.

药物

Doctors may prescribe a variety of medication types to treat Eisenmenger syndrome. These include :

Researchers are also examining the benefits of medications that can dilate or widen blood vessels. An example is sildenafil , which healthcare professionals sell under the brand nameViagra.

Surgery

在严重的情况下，Eisenmenger综合征患者可能是心肺移植物的候选者。

Home management

Sometimes, doctors recommend using supplemental oxygen at home to reduce Eisenmenger syndrome effects. But oxygen therapy might not always help, as the shunt may still limit the amount of oxygen-rich blood that reaches the body’s tissues.

Lifestyle changes

医生通常会建议患有Eisenmenger综合征的人避免极端的体育锻炼，或者锻炼在心脏上面临重大挑战。此外，避免：

  • dehydration
  • 大量饮酒
  • 抽烟
  • traveling to high altitudes
  • using hot tubs, saunas, or steam rooms as these can cause a sudden drop in blood pressure

Also, consider taking good care of your dental health, since you can have a higher chance of endocarditis or heart valve infections. Some doctors may recommend taking antibiotics before going to the dentist to prevent infections.

Eisenmenger syndrome is associated with a shortened life expectancy. People with the condition may be more likely to die in their 20s and 30s than people without.

The most common causes of death for individuals with Eisenmenger syndrome are:

  • hemoptysis
  • pregnancy complications
  • stroke
  • 心室衰竭

But this doesn’t mean all people with Eisenmenger syndrome will die at a younger age. Some live longer lives. A2017 studyshowed that taking medications to manage the condition had the biggest impact on life expectancy.

如果您或亲人患有Eisenmenger综合症，那么与医生定期联系很重要。医生可以监控您的心脏功能，并讨论可能有益于您病情的最新疗法。

Taking the above steps and maintaining a healthy lifestyle can help you maintain your heart function whenever possible.