Eisenmenger Syndrome- Your Ultimate Guide

Cardiology | by Dr. Shuvo Dutta | Published on 29/08/2023

Introduction

If you have been experiencing symptoms like shortness of breath, heart palpitations, or dizziness, then it can be an indication of Eisenmenger syndrome. It is one of the conditions affecting the lung’s blood vessels and the heart’s right side. It is usually present at birth and requires on-time treatment. If it is left untreated, then it can lead to the risk of ventricular septal defect and pulmonary hypertension. 

In this blog, we will discuss comprehensively this condition, including its symptoms, causes, diagnosis, and treatment options. However, kindly note that the information is for your reading purposes and doesn’t interrupt the consultation of a healthcare professional. You can book your appointment with a leading cardiologist in Kolkata at the BM Birla Heart Research Centre in Kolkata, the only cardiology hospital in the Eastern region. You will find the most experienced and highly qualified doctors here. 

What is Eisenmenger?

Eisenmenger syndrome is among the life-threatening conditions of an unrepaired heart issue present at birth, also known as congenital heart defect. In this condition, the patient experiences irregularity in the blood flow in the heart and lungs. It leads to stiffness and narrowness in the lung's blood vessels and blood pressure keeps increasing in the lungs’ arteries, also known as pulmonary arterial hypertension. Eisenmenger causes permanent damage to the lung’s blood vessels. 

To prevent Eisenmenger syndrome, the only way is early diagnosis and congenital heart defects repair. There might be a need for frequent visits and medications when it comes to treatment if even after early diagnosis Eisenmenger develops. The treatment options help in improving the symptoms. 

What are Eisenmenger's symptoms?

Eisenmenger syndrome symptoms might vary depending on the affected organs and heart condition. Symptoms don’t happen until the baby has hit puberty or are observed in adulthood. Later, they begin to get worse gradually, once they are noticed. Here are some of the often-experienced symptoms of Eisenmenger syndrome:

• Easily getting tired 
• Shortness of breath even with a small activity 
• Coughing up blood 
• Fainting 
• Dizziness
• Chest pain or tightness 
• Bluish or grayish skin color due to cyanosis or low oxygen levels 
• Regular headaches 
• Losing breath while at rest 
• Large and rounded fingernails or toenails
• Heart palpitations 
• A feeling of numbness or tingling in toes and fingers

What are Eisenmenger's causes?

To understand the causes of Eisenmenger’s syndrome, it is important to understand how blood flows through the heart. A healthy heart is discussed below:

• Right atrium: It pumps blood to the right ventricle after the deoxygenated blood is received.
• Right ventricle: It picks up additional oxygen by pumping deoxygenated blood to the lungs.
• Left atrium: It pumps oxygenated blood from the lungs to the left ventricle.
• Left ventricle: It involves pumping the oxygenated blood out of the body.

However, if there is a hole in the wall between heart ventricles or any structural defects, then there can be a mix-up of blood that is rich in oxygen and poor blood. As an outcome, additional blood flow to the lungs leads to pulmonary hypertension. Also. it will lead to blood flow to the body that is not rich in oxygen.

How is Eisenmenger diagnosed?

The healthcare provider will involve physical evaluation and questions about the medical history of the patient to confirm Eisenmenger’s syndrome diagnosis. Here are the tests often recommended to the patients:

• Blood tests: A complete blood cell count test is ordered as the count of red blood cells is often high in Eisenmenger syndrome. These tests are performed to identify the functioning of the kidney and liver, including iron levels. 
• Electrocardiogram (ECG): It is a fast and painless method to identify the heart’s electrical activity. During this test, electrodes or sensors are connected to the patient’s chest and arms or legs at times. The test benefits in knowing whether the heart is beating fast or slow. 
• Chest X-ray: This test is ordered to understand the lungs and heart conditions.
• Echocardiogram: Heart’s ultrasound is referred to as an echocardiogram. This test involves using sound waves to create detailed heart pictures in motion. It also displays the blood flow to the heart. 
• Computerized tomography (CT) scan: It is ordered for creating cross-sectional pictures of particular body parts. It is performed to obtain more information on the lungs and arteries. The dye is used as it helps in showing the images more clearly. It offers more comprehensive details than the traditional method of X-rays.
• Magnetic resonance imaging (MRI) scan: This test involves using magnetic fields and radio waves to create detailed lung blood vessel pictures. 
• Cardiac catheterization: This test helps in measuring the heart; 's chambers and blood pressure in the blood vessels. It also benefits in identifying the hole sizes in the heart and blood flow throughout the area. The use of dye helps in obtaining clear X-ray pictures and videos. 
• A walking test.:This test is ordered to identify how well the patient can involve himself/herself during a moderate level of exercise.

What are the options for Eisenmenger treatment?

The options for Eisenmenger syndrome treatment focus on improving the symptoms and averting any serious harm to the lungs or heart. Here are some of the options:

• Antibiotics are recommended to avert the risk of bacterial infections in the lungs or heart. 
• Anticoagulants are given for the prevention of blood clotting risk. 
• Diuretic medications are recommended as they help in reducing fluid accumulation in the body. 
• Dual endothelin receptor antagonists are prescribed as they benefit in lowering the blood pressure in the lungs. 
• Iron supplements are given to patients with iron deficiency or anemia. 
• Supplemental oxygen therapy is prescribed so that the patient can breathe better. 
• People with serious symptoms and organ harm might require a heart or lung transplantation. 
• Surgery might be recommended for people with serious symptoms of Eisenmenger syndrome complications. Phlebotomy is often recommended to treat and manage the symptoms. 

Conclusion

Eisenmenger syndrome is a condition that can be hazardous as it affects the lung’s blood vessels and the heart’s right side. Since, it is present at birth, untreated congenital heart defects can lead to high blood pressure. Symptoms often begin to hit the person during puberty or adulthood. It is important to be aware of the symptoms like exercise intolerance, heart palpitations, cyanosis, etc. Getting in touch with the best cardiologist can go a long way. Taking professional help on time can relieve the symptoms. Make sure not to take the condition lightly and take the necessary steps recommended by your doctor.

FAQ -

How long does it take for Eisenmenger syndrome to develop?

Eisenmenger syndrome’s symptoms usually develop during puberty or early adulthood.

Is Eisenmenger syndrome hereditary?

No, Eisenmenger syndrome is not hereditary and doesn’t run in families. 

Is Eisenmenger syndrome complex?

Yes, Eisenmenger syndrome is a complex condition and should be treated on time. If left untreated, it can cause ventricular septal defect and pulmonary hypertension.