Eisenmenger Syndrome: Causes, Symptoms, Complication and Treatment

Eisenmenger Syndrome, a complex yet critical condition, arises as a consequence of untreated congenital heart defects. It manifests as a reversal of blood flow due to pulmonary hypertension, leading to life-threatening complications. 

Understanding its aetiology, symptoms, and management is pivotal for healthcare professionals and patients alike. In this blog, we delve into the intricacies of Eisenmenger Syndrome, shedding light on its nuances and treatment modalities.

What is Eisenmenger Syndrome?

Eisenmenger syndrome arises from an excessive volume of blood coursing through the arteries that transport blood from the heart to the lungs. This condition induces pulmonary hypertension, characterised by elevated blood pressure within the lung’s blood vessels and the right cardiac chamber. Additionally, it prompts blood diversion between the heart’s left and right sides, indicating an anomalous connection causing blood to flow from the left heart chamber to the right one.

Typically stemming from one or multiple untreated congenital heart anomalies present since birth, Eisenmenger syndrome progressively results in irreversible lung impairment and associated complications.

What are the Stages of Eisenmenger Syndrome?

Eisenmenger Syndrome progresses through several stages, which are:

• Initial Shunt: Begins with congenital heart defects like VSD (ventricular septal defect), ASD (atrial septal defect), or PDA (patent ductus arteriosus).
• Increased Pulmonary Blood Flow: Shunt causes excess blood flow to the lungs, leading to pulmonary hypertension.
• Pulmonary Vascular Disease: Elevated pulmonary artery pressure leads to narrowing and stiffening of arteries.
• Right-to-Left Shunting: Pressure in the right heart surpasses the left, reversing the shunt’s direction.
• Cyanosis and Complications: Deoxygenated blood enters systemic circulation, causing blueness, fatigue, and other symptoms.
• Eisenmenger Syndrome: Irreversible stage marked by poor prognosis, managed symptomatically with lifestyle adjustments and, in severe cases, transplantation.

What are the types of Eisenmenger Syndrome?

Eisenmenger Syndrome manifests in three types, each linked to distinct congenital heart defects. These types are:

• Type 1: Arises from ventricular septal defect (VSD), where the shunting occurs between ventricles.
• Type 2: Associated with atrial septal defect (ASD), featuring shunting between the atria.
• Type 3: Correlates with other heart anomalies like patent ductus arteriosus (PDA).

In all types, increased pulmonary pressure prompts shunt reversal, causing cyanosis. Over time, this influx of deoxygenated blood leads to complications such as polycythemia and thromboembolism. Such classifications aid in tailored management strategies for patients with Eisenmenger Syndrome.

What are the Symptoms of Eisenmenger Syndrome?

Symptoms of Eisenmenger syndrome can vary based on the specific heart defect and organs involved. Typically, they do not manifest until puberty or early adulthood, gradually worsening over time.

Common indicators of the condition include:

• Chest pain (angina)
• Irregular heartbeats (arrhythmias)
• Enlarged and curved fingernails and toenails (clubbing)
• Coughing up blood
• Bluish or greyish skin colouration (cyanosis)
• Episodes of dizziness or fainting
• Swelling in the legs, ankles, feet, or abdomen (oedema)
• Rapid, pounding, or irregular heartbeats (heart palpitations)
• Fatigue and diminished energy levels (lethargy)
• Difficulty breathing (dyspnea)

What are the Causes of Eisenmenger Syndrome?

Understanding the blood circulation in the heart is crucial for grasping Eisenmenger syndrome. In a normally functioning heart:

• The body sends blood that has lost oxygen to the right atrium, which then pumps it to the right ventricle.
• The right ventricle then propels the deoxygenated blood to the lungs for oxygenation.
• Oxygenated blood is received by the left atrium from the lungs and is pumped to the left ventricle.
• The left ventricle subsequently sends the oxygenated blood out to the body.

However, if there’s an opening in the wall between the right and left ventricles or any other structural abnormalities in the heart, oxygen-rich and oxygen-poor blood may mix. This leads to an excess flow of blood to the lungs, causing pulmonary hypertension and a deficiency of oxygen-rich blood reaching the body.

What are the Complications of Eisenmenger Syndrome?

Eisenmenger syndrome may lead to various health issues, including:

1. Brain haemorrhage.
2. Brain abscess from infection.
3. Insufficient blood supply to the brain (cerebral vascular insufficiency).
4. Gout.
5. Myocardial infarction.
6. Cardiac insufficiency.
7. Endocarditis or other heart infections.
8. Thickened blood causing hyperviscosity syndrome.
9. Renal failure.
10. Complications during pregnancy.
11. Cerebrovascular accident (stroke).
12. Abrupt demise.

How is Eisenmenger Syndrome Diagnosed?

Eisenmenger syndrome is often detected by a healthcare provider during a physical assessment. To confirm the diagnosis, they may request the following examinations:

1. Blood oxygen level assessment.
2. Cardiac catheterization.
3. Chest X-ray.
4. Complete blood count (CBC).
5. Echocardiogram (echo).
6. Electrocardiogram (EKG).
7. Heart MRI.
8. Pulmonary function testing.

What is the Treatment for Eisenmenger Syndrome?

Treatment for Eisenmenger syndrome focuses on alleviating symptoms and preventing severe heart or lung damage. Potential therapies include:

• Antibiotics: To prevent bacterial infections in the heart and lungs.
• Anticoagulants: To prevent blood clots.
• Diuretics: To lessen the body’s accumulation of fluid.
• Dual Endothelin Receptor Antagonists: To lower high blood pressure in the lungs.
• Iron Supplements: To address iron-deficiency anaemia.
• Supplemental Oxygen: To improve breathing.

By the time Eisenmenger syndrome develops, surgical repair of the underlying heart defect is usually no longer an option. However, individuals with severe symptoms and organ damage may require a heart or lung transplant.

How can you Prevent Eisenmenger Syndrome?

Preventing Eisenmenger Syndrome involves early detection and management of congenital heart defects (CHDs). Key strategies include:

Early Diagnosis – Prenatal and newborn screening to detect CHDs early.

Timely Treatment

• Surgical correction or catheter-based interventions for CHDs.
• Medical management for those not eligible for surgery.

Regular follow-up – Continuous monitoring by a cardiologist.

Preventing Secondary Complications

• Use of prophylactic antibiotics to prevent infections.
• Maintaining a heart-healthy lifestyle.

Education and Awareness – Educating patients and families about symptoms and treatment adherence.

These measures can prevent the progression of Eisenmenger Syndrome.

Conclusion

Understanding Eisenmenger Syndrome is crucial for managing its impact. Early diagnosis and appropriate treatment can significantly enhance the quality of life, highlighting the importance of awareness and medical advancements. It is always advisable to seek medical help from a cardiologist. Timely care and help can ensure an appropriate diagnosis and treatment of your condition. 

At the CK Birla Hospital, we ensure patients get holistic medical support which includes treatment in a compassionate environment. This patient-centric approach not only helps patients heal better but also ensures they are aware of the preventive measures as well. In case you need to consult a cardiologist, reach out to us, or book a direct appointment with cardiologist at the CK Birla Hospital. 

FAQs About Eisenmenger Syndrome

Can Eisenmenger Syndrome be Cured?

Eisenmenger syndrome is not curable. It’s a complex condition where abnormal blood flow in the heart leads to serious complications, managed with medications and sometimes surgery.

What is the Life Expectancy of Someone with Eisenmenger Syndrome?

Life expectancy varies but is generally reduced. With proper management, including medications, oxygen therapy, and sometimes heart/lung transplant, individuals may live into adulthood, but prognosis depends on individual factors.

Is Eisenmenger Syndrome Hereditary?

Eisenmenger syndrome is not directly hereditary, but it can be associated with congenital heart defects that may have a genetic component. However, it primarily arises from untreated or poorly managed heart conditions.

Can Individuals with Eisenmenger Syndrome Lead a Normal Life?

Individuals with Eisenmenger syndrome often face limitations due to their condition, but with proper management and lifestyle adjustments, they can lead fulfilling lives, although their activities may be restricted.

What are the Risks Associated with Pregnancy in Eisenmenger Syndrome?

Pregnancy poses significant risks for both the mother and foetus in Eisenmenger syndrome, including increased strain on the heart, higher risk of complications like heart failure, and maternal mortality.

Can Eisenmenger Syndrome be Treated with Medication Alone?

Medication can help manage symptoms and improve the quality of life in Eisenmenger syndrome, but it’s often not enough on its own. Additional interventions like oxygen therapy or surgery may be necessary.