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What is Eisenmenger syndrome?
Eisenmenger syndrome is a condition that affects blood flow from the heart to the lungs in some people who were born with structural problems of the heart.
What causes Eisenmenger syndrome?
Eisenmenger syndrome develops over time as a result of the effects of high blood pressure in the lungs. This high blood pressure, or pulmonary hypertension, occurs because of congenital heart defects that cause blood to flow from the left side of the heart to the right side of the heart (left-to-right shunt). Congenital heart defects of this type include:
Patent ductus arteriosus (PDA). A connection between the aorta and the pulmonary artery that allows oxygen-rich (red) blood that should go to the body to recirculate through the lungs.
Atrial septal defect (ASD). An opening in the atrial septum, or dividing wall between the two upper chambers of the heart known as the right and left atria.
Ventricular septal defect (VSD). An opening in the ventricular septum, or dividing wall between the two lower chambers of the heart known as the right and left ventricles.
Atrioventricular canal defect (AV canal). A heart problem that involves several abnormalities of structures inside the heart, including an ASD, VSD, and improperly formed mitral and/or tricuspid valves.
Symptoms of Eisenmenger syndrome
Eisenmenger syndrome signs and symptoms include:
- Bluish or grayish skin color (cyanosis)
- Large, rounded fingernails or toenails (clubbing)
- Easily tiring and shortness of breath with activity
- Shortness of breath while at rest
- Chest pain or tightness
- Skipped or racing heartbeats (palpitations)
- Fainting (syncope)
- Numbness or tingling in the fingers or toes
Eisenmenger syndrome Diagnosis
Chest x-ray and ECG
Echocardiography or cardiac catheterization
Diagnosis is suspected by history of uncorrected cardiac anomalies, supported by chest x-ray and ECG, and established by 2-dimensional echocardiography with color flow and Doppler studies. Cardiac catheterization is often done to measure pulmonary artery pressure, pulmonary vascular resistance, and response to pulmonary vasodilators.
Laboratory testing shows polycythemia with Hct > 55%. Increased RBC turnover may be reflected as an iron deficiency state (eg, microcythemia), hyperuricemia, and hyperbilirubinemia.
Chest x-ray usually shows prominent central pulmonary arteries, peripheral pulmonary vessel pruning, and right heart enlargement. ECG shows right ventricular hypertrophy, right axis deviation, and, occasionally, right atrial enlargement.
Treatment for Eisenmenger syndrome
At times, people with symptoms may have blood removed from the body (phlebotomy) to reduce the number of red blood cells, and then receive fluids to replace the lost blood (volume replacement).
Affected people may receive oxygen, although it is unclear if it helps to prevent the disease from getting worse. People with very severe symptoms may need a heart-lung transplant.