Eisenmenger Syndrome

Eisenmenger Syndrome Definition

Eisenmenger syndrome is the symptoms in which congenital structural anomaly in the heart leads to affect pulmonary circulation. Trouble in pulmonary circulation affects blood stream from heart to the lungs.

In Eisenmenger syndrome most commonly found abnormality is a hole formed in between cardiac chambers. This hole creates trouble to maintain the blood stream in a proper direction. The maximum amount of blood returns to the lungs instead to a whole body circulatory system. This overload creates excess pressure in the pulmonary blood vessels. This causes stiffing and narrowing of the affected blood vessels and gradually damage whole pulmonary circulation.

Eisenmenger syndrome is developed when excessive pressure in the lungs due to the surplus supply of the blood causes reverse blood flow through the shunt and cause de-oxygenated blood transmit to left ventricles from the right chambers of the heart. The consequence of this causes deoxygenated blood pump out from the heart to different organs and creates an oxygen crisis in the physiological system. This leads to fatal condition. Immediate medical assistance minimize the danger. ^[1,3]

Symptoms

Eisenmenger syndrome signs and symptoms include:

• Cyanosis (Skin color becomes bluish or grayish)
• Clubbing (Nails of the fingers and toes become large and rounded)
• Tingling sensation or numbness in the fingers and toe
• Easily physical exhaustion
• Little physical effort and even in resting position affected individuals experience shortness of breathing
• Pain and tightness in the chest cavity
• Irregular heartbeat or palpitations
• Frequent attack of syncope or fainting
• Bleeding occurs during coughing (hemoptysis)
• vertigo
• Headaches
• Abdominal pain and swelling due to cholelithiasis.

The symptoms associated with Eisenmenger Syndrome becomes prominent before puberty and the condition become worsen in the early young age.

In later stages of life hemoptysis occurs and associated signs include brain abscess, cerebral embolic phenomena, or endocarditis may develop.

Transient ischemic attacks,  slurred speech, neurologic symptoms, headaches, visual problems, increased fatigue and signs of thromboembolism may develop due to polycythemia progression as a secondary symptom. ^[2,3,4]

Physiology

The heart is a major circulatory organ and result of a birth defect in heart anatomy causes Eisenmenger syndrome. The clinical findings mentioned that congenial shunt or hole in the ventricular septal present in between the two lower hear chambers (ventricles) is most common anatomical defect in the Eisenmenger syndrome.

This anatomical defects alters physiology of the circulatory system. Normally from right ventricles blood flow from heart to lungs for purification. Oxygenated blood transmits from lungs to heart and store in the left ventricle and pump out to every organ, tissues and cells to supply energy.

Defect in anatomy in Eisenmenger syndrome leads to reverse back blood to the lungs instead of other organs after purification. Presence of hole allows to carry forward deoxygenated blood from right ventricle to left ventricle.

Other anatomical abnormalities which can cause Eisenmenger syndrome are as follows:

• Atrial septal defect (Hole present in between atria)
• Atrioventricular canal defect
• Cyanotic heart disease (Defective pulmonary circulation)
• Patent ductus arteriosus (The faulty shutting in the ducts , resultant defective pulmonary circulation)
• Truncus arteriosus

In Eisenmenger syndrome, gradually lungs have excessive blood pressure and do not allow . Deoxygenated blood  to come up in the lungs for purification. Hole present between the septal allow to balance blood content in the two lower chambers. Thus deoxygenated blood reached to the left ventricle and circulate different organs. ^[3,4]

Diagnosis

The diagnosis of Eisenmenger Syndrome is conducted due to an unmanageable cardiac problem. Doctors usually recommended following diagnostic tests:

• Chest x-ray
• ECG
• 2-dimensional echocardiography with color flow
• Doppler studies

Cardiac catheterization assists to measure pulmonary vascular resistance, pulmonary artery pressure, and response to pulmonary vasodilators.

Blood test showed Hct (hematocrit) level more than 55 indicates the risk of onset of polycythemia and Lowered number of RBCs indicates iron deficiency anemia.[2, 4]

Treatment

Eisenmenger Syndrome is a non curable disease and the aim of the treatment provides better quality of life by minimizing the symptomatic attacks and prevents associated complications. It is always advisable to take expert’s consultation to manage the Eisenmenger syndrome and it is also noticed that after the development of the Eisenmenger syndrome,  doctor does not recommend cardiac surgery to repair the hole in heart, as any surgical intervention may become fatal for patients.

Close monitoring

After development of the Eisenmenger syndrome close monitoring is required to observe all the symptomatic approaches are coming out by physical exam, and blood tests and depends on patient’s usual complains.

Medications

Different cardiac protective and cardio tonics are recommended to manage the Eisenmenger syndrome. But but after prescribing the medications doctors usually closely monitor the pulse rate, blood pressure and plasma volume. The usual prescribed medicines are:

• Anti-arrhythmic drugs
• Iron suppliments
• Anticoagulants (e.g. warfarin)
• blood-thinning medications (e.g. Aspirin)
• Endothelin receptor antagonists to prevent narrowing and stiffening of the blood vessels
• Bosentan is recommended with caution of liver damage to prevent resistance development in the pulmonary arteries.
• It is suggested not to take self medication by consuming OTC drugs, including NSAIDs (ibuprofen) or (naproxen).
• Sildenafil and tadalafil are recommended for controlling excessive pressure in the lungs. These drugs able to open the blood vessels in the lungs and maintain the natural blood flow of the circulatory system. Some included side effects are stomach upset, vertigo and disturbed vision.
• Antibiotics recommended for any onset of infection or before conducting any medical procedure to control the infection spread to heart (endocarditis).

Phlebotomy

phlebotomy is recommended for balancing the red blood cell count and then receive fluid assist with volume replacement. This helps to replace the fluid loss.

Heart-lung transplantation

The transplantation of the lungs and heart is recommended in case of other treatment failure.

Birth control and Pregnancy

Eisenmenger syndrome may become fatal in case of conceiving a child. Birth control and avoid pregnancy are very important suggestive advise for females having Eisenmenger syndrome. ^[4]

Prognosis

The prognosis of Eisenmenger Syndrome depends upon co-morbidity and how early (at which age) lung pressure become increases.  ^[2]

Complications

Eisenmenger syndrome has several complications, which can be averted by proper treatment and monitoring. The included complications are

• Low oxygen levels in blood (cyanosis)
• High red blood cell count (erythrocytosis)
• Arrhythmias
• Sudden cardiac arrest
• Heart failure
• Blood come up during coughing (hemoptysis)
• Stroke
• Kidney problems
• Risks of conceiving a child ^[3]

Life expectancy

Eisenmenger syndrome is a fatal disorder. Maximum life span for individuals having Eisenmenger syndrome is 50 to 60 years. ^[4]

References:

1. Mikhael F El-Chami (2014); Eisenmenger Syndrome; Retrieve from: http://emedicine.medscape.com/article/154555-overview
2. Kurt R. Schumacher (2014); Eisenmenger Syndrome; Retrieve from: https://medlineplus.gov/ency/article/007317.htm
3. Mayo Clinic Staff (2016); Eisenmenger Syndrome; Retrieve from:
4. http://www.mayoclinic.org/diseases-conditions/eisenmenger-syndrome/symptoms-causes/dxc-20177663
5. Jeanne Marie Baffa; Eisenmenger Syndrome; Retrieve from: http://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/eisenmenger-syndrome