Eisenmenger Syndrome - Symptoms, Causes, Diagnosis, and Treatment

Introduction

A long-term untreated congenital heart problem causes Eisenmenger syndrome. It can prove to be a life-threatening condition. This condition leads to irregular blood flow in the heart and lungs, which in turn causes narrowing and stiffness of the blood vessels in the lungs. Finally, all this leads to increased blood pressure in the lung’s arteries. Eisenmenger syndrome is known to damage the blood vessels in the lungs permanently.

What Are the Symptoms?

Symptoms of Eisenmenger syndrome have been mentioned below:

• Pain or tightness in the chest.

• Cyanosis (bluish or grayish skin discoloration due to decreased oxygen levels).

• Coughing up blood.

• Dizziness or fainting.

• Headaches.

• Tiredness.

• Shortness of breath with activity.

• Clubbing of nails (large, rounded nails in the finger or toe).

• Palpitations (skipped or racing heartbeats).

• Numbness or tingling in fingers or toes.

• Shortness of breath while at rest.

How Is It Caused?

The most common cause of Eisenmenger syndrome is an unrepaired hole between the heart chambers or the heart's main blood vessels. A shunt is a congenital heart defect; that is, it is present at birth.

There are various conditions of the heart that can cause Eisenmenger syndrome, which are as follows:

• Ventricular Septal Defect: It is the most common cause of this condition. In this, there is a hole between the ventricles of the heart.

• Atrioventricular Canal Defect: In this, there is a hole between the upper and lower chambers of the heart.

• Atrial Septal Defect: In this, there is a hole between the heart's upper chambers.

• Patent Ductus Arteriosus: In this, there is an opening between the aorta and the pulmonary artery.

The above four heart defects change the normal pathway of blood flow in the heart and, thus, the body. Hence, there is an increase in blood pressure within the pulmonary vessels. After some time, these changes damage the smaller blood vessels in the lungs. These damaged blood vessels make it difficult to transport blood to the lungs.

Are There Any Risk Factors?

Yes, a few factors put a person at risk of developing this condition. If they have a family history of congenital heart defects, the baby has an increased risk of developing similar heart problems. If they have been diagnosed with Eisenmenger syndrome, they should consult the physician to screen other family members for congenital heart defects.

How Can It Be Diagnosed?

Initially, a person will be asked questions about the signs and symptoms he is facing along with the family history and medical and personal histories. Then, a physical examination will be conducted. The physician will require him to undergo various tests to confirm the diagnosis.

They are as follows:

• Blood Tests: The most common test is the complete blood count test. There can be increased Red Blood Cells. Also, this can be used to test kidney and liver function and iron levels.

• Electrocardiogram (ECG): This helps in assessing the electrical activity of the heart.

• Echocardiogram: This is the ultrasound of the heart. It helps to check the blood flow through the heart.

• Chest X-ray.

• Computerized Tomography (CT) Scan: This test utilizes X-rays to form cross-sectional images of specific body parts. Thus, a detailed study of the heart and lungs can be done.

• Walking Test: It is a six-minute walking test to assess an individual's tolerating level while performing a mild level of exercise.

• Magnetic Resonance Imaging (MRI): This test utilizes magnetic fields and radio waves to form detailed images of the blood vessels present in the lungs.

• Cardiac Catheterization: This test uses a catheter and a dye to check for the blood flow in the heart.

How Can It Be Treated?

The main objectives of the management of Eisenmenger syndrome are to manage symptoms, prevent serious complications, and improve the quality of life. It can be done using either medications or surgery.

1. Medications

Medications are the commonly used mainstream treatment for Eisenmenger syndrome. They cannot help in curing the condition, but they can be used to improve the quality of life. The patient should have regular check-ups while taking medications. The physician will check for any changes in the blood pressure, fluid levels, and pulse rate during follow-ups.

Various medications that can be prescribed for the management of Eisenmenger syndrome are as follows:

• Blood-thinning medications or Aspirin.

• Heart rhythm drugs.

• Iron supplements.

• Antibiotics.

• Drugs for pulmonary arterial hypertension.

• Sildenafil and Tadalafil.

2. Surgery

Physicians or specialists do not recommend surgical procedures to close the shunt in the heart after the development of Eisenmenger syndrome.

Various surgeries or procedures that can be done to manage symptoms or complications of this condition have been mentioned below:

• Phlebotomy or Blood Withdrawal: This procedure will be recommended if the red blood cell count becomes extremely high and causes symptoms like headaches, vision, and concentration issues. This procedure should not be done regularly and requires consent from a heart specialist.

• Transplant: If none of the treatments works, the patient may require a heart transplant.

Are There Any Complications?

As mentioned earlier, Eisenmenger syndrome can prove to be a life-threatening condition. The prognosis can vary among individuals depending on the condition's cause and other medical issues.

Following are the complications that can develop if this condition is not managed and monitored:

• Decreased levels of blood oxygen.

• Arrhythmias (irregular heartbeats).

• Heart failure.

• Sudden cardiac arrest.

• Stroke.

• Bleeding in the lungs.

• Infection in the heart

• Kidney problems.

• Gout.

• Pregnancy risks.

Will Lifestyle Changes And Home Remedies Work?

Proper treatment and precautions can help improve the quality of life in an individual diagnosed with Eisenmenger syndrome.

The following can be done:

• Regular exercising. One should talk to their physician regarding the type of exercise suitable for them and the restricted exercises.

• Avoid dehydration.

• Avoid going to high altitudes.

• Get a flu vaccine.

• Avoid conditions that can cause decreased blood pressure.

• Use medications and supplements only after talking to the specialist.

• Avoid smoking (both active and passive) and tobacco consumption.

Conclusion

Eisenmenger syndrome is caused as a complication of untreated congenital heart defects, which can be life-threatening. It should be managed as early as possible to improve the prognosis. A patient can talk to a specialist online with the help of online medical platforms to know more about this condition.