What Is Eisenmenger Syndrome?

Overview

Eisenmenger syndrome is a rare and serious heart condition characterised by the reversal or shunting of blood flow within the heart due to a defect in its structure. This condition typically arises as a complication of certain congenital (present from birth) heart defects, resulting in increased pressure in the pulmonary arteries (which carry blood from the heart to the lungs) and eventual damage to the blood vessels in the lungs.

In this article, we will delve into the details of Eisenmenger syndrome, exploring its causes, symptoms, diagnosis, treatment options, and much more.

Causes of eisenmenger syndrome

To understand Eisenmenger syndrome, it is necessary to know how our heart operates. Physiologically, our heart is like a powerful pump that keeps our bodies working properly. It has four parts called chambers: two on the top called atria and two on the bottom called ventricles. 

The heart works through a complex process involving rhythmic contractions and relaxation. It starts with the contraction of the atria, which allows blood to flow into the ventricles. The atrioventricular (AV) valves inhibit the backflow of blood into the atria when the ventricles contract. During a ventricular squeeze, the AV valves get closed, enabling another set of valves called semilunar valves to open and pump blood into the arteries. 

The left side of the heart receives oxygenated blood from the lungs and pumps it to the rest of the body, delivering oxygen and nutrients to the body's tissues. On the other hand, the right side of the heart receives deoxygenated blood from the body and delivers it to the lungs for oxygenation. This continuous cycle of contraction and relaxation ensures the efficient circulation of blood, providing oxygen and nutrients to all parts of the body.

Eisenmenger syndrome usually develops as a consequence of a specific type of congenital heart defect known as a left-to-right shunt. These defects cause an abnormal connection between the heart chambers, leading to increased pressure in the lungs. The three major defects are the ventricular septal defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA).¹ 

In these conditions, abnormal connections between the heart chambers or blood vessels cause oxygen-rich and deoxygenated blood to mix. Over time, the increased pressure in the pulmonary arteries causes the direction of blood flow to reverse, leading to the development of Eisenmenger syndrome. Moreover, this increased pressure sometimes causes the blood vessels in the lungs to become thickened, narrowed, and scarred, making it difficult for blood to flow through. As a result, the heart has to work harder to pump blood, eventually leading to heart failure.

Risk factors of eisenmenger syndrome

The risk factors for Eisenmenger syndrome include a history of congenital heart disease, specifically ventricular septal defect or atrial septal defect. Some other important risk factors may include:

• High blood pressure
• Infective endocarditis
• Smoking
• Family history of pulmonary hypertension

Signs and symptoms of eisenmenger syndrome

The symptoms of Eisenmenger Syndrome can vary depending on the severity of the disease. The following are the most common symptoms:

• Shortness of breath
• Fatigue
• Chest pain
• Heart palpitations
• Oedema (swelling in the lower extremities of the body)
• Blue or purple discolouration of  the skin (cyanosis)
• Clubbing (rounding of the tips) of toes and fingers
• Coughing blood

In severe cases, patients may experience syncope (fainting) or even sudden death. It is important to note that these symptoms can be similar to those of other medical conditions, so it is advised to seek medical attention if you experience any of these symptoms.

Diagnosis of eisenmenger syndrome

Diagnosing Eisenmenger syndrome can be challenging, as the symptoms can be similar to those of other medical conditions. A thorough medical history and physical examination are usually the first steps in diagnosing the disease. Some of the additional diagnostic testing methods may include:

• An echocardiogram
• Electrocardiogram (ECG)
• Pulmonary function tests
• A chest X-ray
• Blood tests to assess oxygen levels in the body
• In some cases, cardiac catheterization may be necessary to measure the pulmonary vascular resistance

Management and treatment for eisenmenger syndrome

There is currently no cure for Eisenmenger Syndrome, but there are treatments available to help manage the symptoms and prevent complications.

Pulmonary vasodilators, such as sildenafil and bosentan, can help to dilate the blood vessels in the lungs, reducing pulmonary vascular resistance. 

Oxygen therapy can also be helpful in increasing oxygen saturation levels. Certain medications, such as diuretics and blood thinners may be prescribed to alleviate symptoms and prevent complications.

In some cases, surgical intervention like a lung or heart transplant may be necessary. However, these options are reserved for severe cases and evaluated depending on the patient's symptoms, reports, and circumstances.

Lifestyle changes, including avoiding strenuous activities, maintaining a healthy weight, and quitting smoking are crucial in managing the condition.

Furthermore, it is vital for patients with Eisenmenger syndrome to receive regular medical care and to avoid situations that could worsen their symptoms, such as high altitudes or pregnancy.

Complications of eisenmenger syndrome

The complications of Eisenmenger syndrome can be serious and life-threatening. Heart failure is a common complication, as the heart has to work harder to pump blood through the narrowed blood vessels in the lungs. Blood clots can also form in the blood vessels, increasing the risk of stroke or pulmonary embolism. Infective endocarditis, a bacterial infection of the heart valves, is also a risk for patients with Eisenmenger Syndrome.

Here are some other complications of this condition:

• Low blood oxygen levels in the body
• Kidney issues
• Irregular heart rhythm
• Bleeding in the lungs and airways
• Pregnancy risks
• Gout

FAQs

How can I prevent eisenmenger syndrome?

There is no way to prevent Eisenmenger Syndrome, as it is usually caused by a congenital heart defect. However, it is important for patients with a history of congenital heart disease to receive regular medical care and avoid situations that could worsen their symptoms.

How common is eisenmenger syndrome?

Eisenmenger Syndrome is a rare medical condition, affecting only a small percentage of people with congenital heart disease. However, the condition can be life-threatening, and prompt diagnosis and treatment are essential.

When should I see a doctor?

If you or a loved one experience any symptoms of Eisenmenger syndrome, such as shortness of breath, fatigue, chest pain, or cyanosis then it is recommended to seek medical attention right away. Early diagnosis and treatment can help to prevent complications and improve outcomes.

Summary

Eisenmenger Syndrome is a rare and complex disease that affects the heart and lungs. It is caused by a congenital heart defect, specifically a ventricular septal defect or atrial septal defect, and can lead to serious complications, including heart failure, blood clots, and infective endocarditis. 

While there is no cure for Eisenmenger Syndrome, there are treatments available to help manage the symptoms and prevent complications. It is important for patients with this condition to receive regular medical care and to avoid situations that could worsen their symptoms.