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Everything You Need to Know about Eisenmenger Syndrome


Eisenmenger syndrome is a long-term complication of one or more unrepaired congenital heart defects. People with less access to healthcare and heart screening services may have heart defects that go undetected and untreated. Undiagnosed and untreated heart defects may cause the blood to circulate abnormally through the heart, lungs, and body, damaging the heart and blood vessels over time.

Healthy heart and vascular function

In a healthy heart, the two chambers on the left side of the heart work together to pump oxygen-rich blood it has just received from the lungs out into the body. Next, the blood returns to the right side of the heart, where the two chambers work together to pump the oxygen-depleted blood into the lungs to receive fresh oxygen. Finally, the blood returns to the left side of the heart, and the cycle begins again.

The oxygen-rich blood and oxygen-depleted blood never mix.

Congenital heart defects

Some congenital heart defects consist of one or more holes between the heart chambers or blood vessels. These holes allow oxygen-rich blood and oxygen-depleted blood to flow back and forth between the heart chambers or the blood vessels and mix. This flowing and mixing changes the pressures inside the chambers and vessels and allows blood without oxygen to go out to the body.

Over time, this scenario can lead to heart and blood vessel damage due to pressure changes and damage to the body due to a lack of oxygen reaching the cells.

The syndrome

If an infant is born with certain congenital heart defects (CHD) that are not detected and diagnosed early, the unusual blood flow creates extra work for the heart and blood vessels. Over time, the damage may lead to health problems.

Any CHDs that cause high blood pressure in the lungs can cause Eisenmenger syndrome, but the most common are holes in the walls between the heart chambers or blood vessels, including:

  • Atrial septal defects.
  • Ventricular septal defects.
  • Atrioventricular canal defects.
  • Patent ductus arteriosus.

These holes lead to:

  • Pulmonary hypertension (high blood pressure in the lungs): blood mixes between the heart chambers. The higher pressure in the left side of the heart, which usually pumps blood throughout the body, pushes more blood than usual into the right side of the heart and blood vessels carrying blood to the lungs.
  • Hypoxemia (low oxygen in the blood): Oxygen-rich and oxygen-depleted blood mix through the hole in the heart. Deoxygenated blood flows to the body, delivering less oxygen than usual to the body's cells.

Causes

Eisenmenger syndrome occurs when untreated congenital heart defects (CHDs) put so much strain on the heart and blood vessels that irreversible damage occurs.

Congenital heart defects are heart defects that are present at birth. If the infant does not show signs of a heart defect or they do not receive screening, the defect may go unnoticed and untreated until the symptoms of Eisenmenger syndrome start to manifest later in life.

Risk factors

Risk factors for Eisenmenger syndrome include a family history of congenital heart defects. If someone in your family has a congenital heart defect, you also have an increased risk of CHD.

Early screening, detection, and treatment of CHDs are the keys to avoiding Eisenmenger syndrome.

People in low-income situations have an increased risk of developing this condition because they do not have regular access to healthcare and heart screening for their infants. Even if diagnosed, a mild CHD may go untreated if not considered an urgent medical concern.

Symptoms

Eisenmenger syndrome symptoms often develop around puberty and usually between two and 65 years of age, depending on the location and size of the heart defect or defects.

The most prominent symptom is cyanosis, a bluish hue to the lips or nail beds due to the decreased oxygen available to the body's cells. Other symptoms may include:

  • Clubbing of the fingers and toes (the tips thicken, and the nail begins to slope down).
  • Numbness or tingling in the fingers and toes.
  • Heart palpitations or irregular heart rate.
  • Sores on the feet and legs.
  • Chest pain or tightness.
  • Shortness of breath.
  • Coughing up blood.
  • Organ damage.
  • Dizziness.
  • Lethargy.
  • Swelling.
  • Fainting.
  • Fatigue.

In response to the lower oxygen levels, your body will increase red blood cell production to carry more oxygen to the cells. More red blood cells cause thickening of the blood and, over time, can cause:

  • Changes in vision.
  • Organ damage.
  • Headaches.
  • Dizziness.
  • Stroke.

Complications

Complications of Eisenmenger syndrome include

  • Low oxygen levels, which may not respond to oxygen.
  • Liver and gallbladder congestion.
  • Increased risk of infections.
  • Reduced life expectancy.
  • High-risk pregnancies.
  • Sudden cardiac arrest.
  • Kidney disease.
  • Heart failure.
  • Stroke.

Diagnosis

If you have symptoms of Eisenmenger syndrome, your doctor will ask you questions about your personal and family medical history. Then, they will perform a physical exam and may order tests and scans to confirm the diagnosis.

Tests and scans may include:

  • EKG.
  • Blood tests.
  • Chest x-ray.
  • Cardiac MRI.
  • Echocardiogram.
  • Cardiac catheterization
  • Pulmonary function tests.

Treatment

The treatment for Eisenmenger syndrome is mainly supportive and may include medications to treat the symptoms, such as:

  • Diuretics to remove excess fluid.
  • Antiarrhythmics to control the heart's rate and rhythm.
  • Anticoagulants to prevent blood clots.
  • Antihypertensives to prevent or control high blood pressure.

Surgery to fix the heart defects is not usually beneficial since the damage is already done. However, a heart and lung transplant may be necessary in very severe cases.

Prevention

Early diagnosis and treatment of the heart can usually prevent Eisenmenger syndrome. Heart screening can pick up on heart disease early, sometimes before an infant is born. Therefore, early diagnosis and treatment can prevent the syndrome from developing.

If you have already developed Eisenmenger's syndrome, there are a few ways you can prevent further complications:

  • Avoid spending long periods in higher altitudes.
  • Avoid becoming dehydrated.
  • Avoid isometric exercises.
  • Avoid things that can cause rapid blood pressure drops, such as hot tubs or saunas.
  • Talk to your doctor if you are pregnant or plan to become pregnant.

You may also need antibiotics before specific dental procedures to prevent infection.

Conclusion

Eisenmenger's syndrome is a life-threatening medical condition. Talk to your doctor if you suspect your symptoms may be related to a heart defect.

Key takeaways

Eisenmenger syndrome is a life-threatening medical condition. Talk to your doctor if you suspect your symptoms may be related to a heart defect.

Be sure to keep all your scheduled appointments with your doctor and follow their advice regarding diet, exercise, and activities to avoid.

Call 9-1-1 if you or someone around you has chest pain, difficulty breathing, or if they become unresponsive.

Resources:

Basit, H., et al. (2022). Eisenmenger Syndrome. StatPearls - NCBI Bookshelf.

Cotts, T.B. (2022). Review of Eisenmenger Syndrome. American College of Cardiology.

GARD. (2021). Eisenmenger syndrome - About the Disease. Genetic and Rare Diseases Information Center.

Mayo Clinic. (2020). Eisenmenger syndrome - Symptoms and causes.

National Organization for Rare Disorders. (2019). Eisenmenger Syndrome.

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