Eisenmenger Syndrome

A persistent side effect of an untreated cardiac condition that was present at birth is Eisenmenger syndrome (congenital heart defect). Eisenmenger syndrome poses a serious risk to life.

The heart and lungs experience erratic blood flow in Eisenmenger syndrome. As a result, the blood arteries in the lungs stiffen and constrict. Lung artery blood pressure increases (pulmonary arterial hypertension). The lungs' blood arteries are irreversibly affected by Eisenmenger syndrome.

Eisenmenger syndrome is often avoided through early detection and treatment of congenital cardiac abnormalities. If it develops, regular doctor visits and drugs to address the symptoms are part of the treatment.

Eisenmenger Syndrome: Causes

Eisenmenger syndrome is typically brought on by an unrepaired hole (shunt) between the heart's chambers or major blood arteries. A shunt is a cardiac condition that exists from birth (congenital heart defect).

Mechanics of the Heart

It is necessary to understand how the heart generally functions to comprehend how Eisenmenger syndrome affects the heart and lungs.

  • Two top chambers (atria) and two lower chambers make up the heart's chamber structure (ventricles).

  • Blood veins on the right side of the heart transport blood to the lungs (pulmonary arteries).

  • Blood absorbs oxygen in the lungs before returning to the left side of the heart through the pulmonary veins.

  • The blood is then pumped from the left side of the heart to the rest of the body through the aorta.

  • The movement of blood into and out of the heart chambers is regulated by heart valves. These valves open to let blood pass to the next chamber or an artery, and then close to stop blood from flowing in the opposite direction.

The Progression of Eisenmenger Syndrome

Eisenmenger syndrome may result from congenital heart abnormalities, which are cardiac conditions present at birth.

  • Defect of the ventricle septum. The most frequent reason for Eisenmenger syndrome is this. The tissue wall between the right and left sides of the heart's primary pumping chambers has a hole in it (a shunt).

  • Defect in the atrioventricular canal. The walls separating the heart's upper and lower chambers, or atria and ventricles, meet in this significant opening in the core of the organ. Moreover, some cardiac valves may not function adequately.

  • Septal defect in the heart. This is a gap in the tissue that separates the right and left sides of the heart's upper chambers (atria).

  • Arteriosus ductus patent. The pulmonary artery, which transports blood depleted in oxygen to the lungs, and the artery, which transports blood abundant in oxygen to the rest of the body, meet here (aorta).

Every one of these cardiac conditions causes abnormal blood flow. Pressure in the pulmonary artery increases as a result.

The smaller blood arteries in the lungs are damaged over time by increasing pressure. Pumping blood to the lungs is challenging because of the blood vessel walls' deterioration.

In Eisenmenger syndrome, the side of the heart with oxygen-poor blood has elevated blood pressure (blue blood). The hole (shunt) in the heart or blood arteries is where the blue blood exits. Blood with and without oxygen is now mixed. Low blood oxygen levels result from this.

Eisenmenger Syndrome: Symptoms

The patient with Eisenmenger syndrome mainly presents with the following symptoms that include −

  • Low oxygen levels can cause blue or grey skin tones (cyanosis)

  • Stiffness or discomfort in the chest

  • Exhaling blood

  • Fainting or vertigo

  • Shortness of breath and easily worn out with exercise

  • Headaches

  • Big, rounded toenails or fingernails (clubbing)

  • Tingling or numbness in the fingers or toes

  • Breathing difficulty when at rest

  • Racing or skipped heartbeats (palpitations)

When to Visit a Doctor?

See your doctor if you have any Eisenmenger syndrome symptoms. Even if you have never had a cardiac condition officially diagnosed, schedule a consultation. Medical treatment is necessary for symptoms including chest discomfort or shortness of breath.

Eisenmenger Syndrome: Risk Factors

The likelihood of a newborn developing congenital heart abnormality increases if there is a family history of the condition. If you have Eisenmenger syndrome, talk to your doctor about having your family members checked for congenital cardiac problems.

Eisenmenger Syndrome: Diagnosis

The diagnosis of Eisenmenger syndrome is mainly done based on history and some of the tests may be required for confirmation and to rule out underlying causes

  • A blood test. It's common to do a total blood cell count. Eisenmenger syndrome may have a high red blood cell count.

  • Electrocardiogram (ECG). The electrical activity of the heart is measured by this rapid and painless examination.

  • A chest X-ray. An X-ray of the chest can help reveal the health of the heart and lungs.

  • Echocardiogram. An echocardiogram is a name for a heart ultrasound. In this procedure, sound waves are used to provide precise photographs of the beating heart.

  • Lung magnetic resonance imaging (MRI) scan. This examination produces finely detailed pictures of the blood arteries in the lungs using magnetic fields and radio waves.

  • Catheterization of the heart. The heart is reached by inserting a long, thin, flexible tube (catheter) into a blood artery, typically in the groin or wrist.

  • Exercising test. This six-minute walking test is used to assess your tolerance for light exercise.

Eisenmenger Syndrome: Treatment

The treatment is based on the severity of the symptoms. Your doctor may advise conservative or surgical treatment.

Conservative Treatment

Conservative treatment includes −

  • Meds for heart rhythm. Anti-arrhythmic drugs assist regulate the heart's rhythm and stop irregular heartbeats.

  • Supplementing iron. If your iron levels are too low, your doctor could advise you to take them. Never start taking iron supplements without first consulting your doctor.

  • Either aspirin or drugs that thin the blood. Your doctor could advise aspirin or a blood thinner like warfarin if you've had a stroke, blood clot, or certain forms of irregular heartbeats.

Surgical Treatment

Surgical treatment may be required in severe cases which include −

  • Blood being drawn (phlebotomy). Your doctor could advise this surgery if your red blood cell count rises to an abnormal level and starts to cause symptoms like headaches, attention problems, or vision issues.

  • Transplant. If alternative therapies are unsuccessful, some Eisenmenger syndrome sufferers may require surgery to replace their heart or lungs.

Eisenmenger Syndrome: Prevention

Eisenmenger syndrome may only be avoided by correcting congenital cardiac problems as early as feasible in childhood.


An inter professional team composed of a cardiologist, pulmonologist, cardiac surgeon, internist, thoracic surgeon, and intensivist is the best option for managing Eisenmenger syndrome. The goal is to address the underlying condition, which has several origins. Once Eisenmenger syndrome manifests, it has a terrible prognosis.

Although heart and lung transplants are an option, there remains a donor shortage. The effectiveness of drugs that widen the pulmonary vasculature is neither predictable nor constant. The majority of patients pass within 12 to 24 months following their diagnosis.

Dr. Durgesh Kumar Sinha
Dr. Durgesh Kumar Sinha


Updated on: 28-Apr-2023


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