This complication of an unrepaired congenital heart defect needs close monitoring. Learn how treatment can help manage symptoms and improve quality of life.
Eisenmenger (I-sun-meng-ur) syndrome is a long-term complication of an unrepaired heart problem present at birth (congenital heart defect). Eisenmenger syndrome is life-threatening.
In Eisenmenger syndrome, there is irregular blood flow in the heart and lungs. This causes the blood vessels in the lungs to become stiff and narrow. Blood pressure rises in the lungs' arteries (pulmonary arterial hypertension). Eisenmenger syndrome permanently damages the blood vessels in the lungs.
Early diagnosis and repair of congenital heart defects usually prevents Eisenmenger syndrome. If it does develop, treatment involves regular medical visits and medications to improve symptoms.
Eisenmenger syndrome is most often a complication of having a hole between two chambers of the heart. Here, a hole between the heart's main pumping chambers (ventricular septal defect) causes more blood to flow to the lungs' arteries. Eventually this causes these blood vessels to stiffen and narrow, increasing pressure in the lungs' arteries. The high pressure causes the walls of the right lower heart chamber (right ventricle) to thicken (hypertrophy).
Symptoms of Eisenmenger syndrome include:
If you have any symptoms of Eisenmenger syndrome, see your health care provider. Make an appointment even if you have never been diagnosed with a heart problem. Symptoms such as shortness of breath or chest pain require medical attention.
Eisenmenger syndrome is usually caused by an unrepaired hole (shunt) between the main blood vessels or chambers of the heart. A shunt is a heart problem present at birth (congenital heart defect).
To understand how Eisenmenger syndrome affects the heart and lungs, it's helpful to know how the heart typically works.
The heart is divided into chambers — two upper chambers (atria) and two lower chambers (ventricles).
Heart valves control the flow of blood into and out of the chambers of the heart. These valves open to allow blood to move to the next chamber or to one of the arteries, and then close to keep blood from flowing backward.
Heart problems present at birth (congenital heart defects) that can cause Eisenmenger syndrome include:
In any of these heart problems, blood flows in a way it usually doesn't. As a result, pressure rises in the pulmonary artery. Over time, the increased pressure damages the smaller blood vessels in the lungs. The damaged blood vessel walls make it difficult to pump blood to the lungs.
In Eisenmenger syndrome, there's increased blood pressure in the side of the heart that has oxygen-poor blood (blue blood). The blue blood goes through the hole (shunt) in the heart or blood vessels. Oxygen-rich and oxygen-poor blood now mix. This causes low blood oxygen levels.
A family history of congenital heart defects increases the risk of similar heart problems in a baby. If you've been diagnosed with Eisenmenger syndrome, talk to your health care provider about screening other family members for congenital heart defects.
Eisenmenger syndrome is a life-threatening condition. The outlook for people diagnosed with Eisenmenger syndrome depends on the specific cause and if there are other medical conditions.
Without proper treatment and monitoring, complications of Eisenmenger syndrome may include:
To diagnose Eisenmenger syndrome, your health care provider does a physical exam and asks medical history questions.
Tests to diagnose Eisenmenger syndrome may include:
The goals of Eisenmenger syndrome treatment are to control symptoms, improve quality of life and prevent serious complications.
If you are diagnosed with Eisenmenger syndrome, you'll be referred to a heart specialist (cardiologist). Regular health checkups — at least once a year — are an important part of Eisenmenger syndrome treatment. It's helpful to find a cardiologist who has experience treating people who have congenital heart defects.
Medications are the main treatment for Eisenmenger syndrome. They can't cure the condition, but they can help improve your quality of life. You need regular medical checkups when taking medications. Your provider checks for any changes in blood pressure, pulse rate and fluid levels.
Medications for Eisenmenger syndrome include:
Health care providers don't recommend surgery to repair the hole (shunt) in the heart once Eisenmenger syndrome has developed.
Surgeries or procedures that may be done to treat symptoms or complications of Eisenmenger include:
If you need a procedure or surgery for Eisenmenger, consider getting care at a medical center with providers who have experience in congenital heart diseases.
If you're diagnosed with Eisenmenger syndrome, proper treatment and precautions can help improve quality of life. Try these strategies:
If you have Eisenmenger syndrome, becoming pregnant poses serious health risks — and can be life-threatening — for you and the baby. It's critical that pregnancy be avoided if you have Eisenmenger syndrome.
Effective contraceptive methods include vasectomy, an intrauterine device (IUD) or a contraceptive hormonal implant such as Nexplanon. Tying of the fallopian tubes (tubal ligation) is a very effective form of contraception. But it's less often recommended due to the risks posed by having even minor surgery.
Birth control pills containing estrogen aren't recommended for those who have Eisenmenger syndrome. Estrogen increases the risk of developing blood clots that could potentially block an artery to the heart, brain or lungs. Using a barrier method alone, such as a condom or diaphragm, isn't recommended due to the risk of failure.
A diagnosis of Eisenmenger syndrome can be scary. Although treatments can help symptoms and improve prognosis, you may feel worried or anxious.
You may find that joining a support group is helpful. Some people find that talking with others who've been through similar situations brings comfort and encouragement. Ask your health care provider if there are any local support groups.
Children with a congenital heart defect or Eisenmenger syndrome may have emotional and physical difficulties that can affect home and school life. For example, a long recovery time from surgeries or procedures may lead to developmental delays. Talk to your health care provider about ways you can help your child. This may include support groups or a visit to a therapist or psychologist.
Eisenmenger syndrome is a complicated condition. There's often a lot to discuss at a medical appointment. So it's a good idea to be prepared. Here's some information to help you get ready for your appointment and know what to expect from your health care provider.
Your time with your health care provider may be limited. Prepare a list of questions to help you make the most of your time together. List your questions from most important to least important in case time runs out. For Eisenmenger syndrome, some basic questions to ask your provider include:
In addition to the questions you've prepared to ask your health care provider, don't hesitate to ask additional questions during your appointment.
Your health care provider is likely to ask you many questions. Being ready to answer them may save time to go over information you want to spend more time on. Your provider may ask the following questions: