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
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 syndrome can occur due to a structural irregularity in the heart at birth that may be undiagnosed or uncorrected. It’s usually a ventricular septal defect (VSD), which is a hole in the heart.
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 to pulmonary 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:
- atrial septal defect
- atrioventricular septal defects
- patent ductus arteriosus
- unrepaired tetralogy of Fallot
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.
Who’s at risk of Eisenmenger syndrome?
Men and women experience Eisenmenger syndrome in equal numbers, and an estimated 25 percent to 50 percent of people with Down syndrome have Eisenmenger syndrome.
Can Eisenmenger syndrome be prevented?
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
Eisenmenger syndrome can cause several symptoms in people. It’s not unusual if your symptoms don’t resemble those of someone else with the same diagnosis.
The main symptom is usually related to cyanosis, a lack of oxygen in the body, and high pressure in the lungs. Some manifestations of this include:
- bluish discoloration to the skin, such as the lips, fingers, and toes
- clubbing, or a rounded appearance of the fingertips or toes
- irregular heart rhythms
- swelling in the abdomen or lower legs
- unusual shortness of breath
Some people with Eisenmenger syndrome may have complications resulting from their condition. These include an increased chance of:
People with Eisenmenger syndrome can also experience
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
According to a 2016 review, deaths during pregnancies in people with Eisenmenger syndrome range from 30 to 50 percent, and can be as high as 65 percent in people who require cesarean delivery. The most common causes of death related to pregnancy and Eisenmenger syndrome are:
- heart failure
- 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 can help you manage your condition. You may want to consider including the following types of medical professionals:
If you have Eisenmenger syndrome, it’s important to talk with your doctor about
There’s no definitive test yet to diagnose someone with Eisenmenger syndrome. Doctors may use multiple tools to see if a person has the condition. Examples of these tests include:
- cardiac catheterization, a procedure that involves using a catheter in the groin or wrist to measure the pressure and blood flow in the heart
- cardiac magnetic resonance imaging to help doctors identify anatomical abnormalities
- chest X-ray
- lab testing, including complete blood counts and iron level testing
- pulmonary function tests
- pulse oximetry, a noninvasive measurement of oxygenation in the blood
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.
Doctors may prescribe a variety of medication types to treat Eisenmenger syndrome. These
- antiarrhythmics to maintain heart rate
- blood thinners to reduce the risk of blood clots
- diuretics, commonly known as water pills
- iron supplementation
In severe instances, people with Eisenmenger syndrome may be candidates for heart-lung transplants.
Sometimes, doctors recommend using
Doctors typically advise people with Eisenmenger syndrome to avoid extreme physical exercise, or exercise that places significant challenges on the heart. In addition, it’s important to avoid:
- heavy alcohol consumption
- 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
- pregnancy complications
- ventricular failure
But this doesn’t mean all people with Eisenmenger syndrome will die at a younger age. Some live longer lives. A 2017 study showed that taking medications to manage the condition had the biggest impact on life expectancy.
If you or a loved one has Eisenmenger syndrome, it’s important to connect with a doctor regularly. A doctor can monitor your heart function and discuss the latest therapies that could benefit your condition.
Taking the above steps and maintaining a healthy lifestyle can help you maintain your heart function whenever possible.