What is Eisenmenger Syndrome?

Eisenmenger syndrome is a heart and lung condition that typically occurs when certain heart defects go unnoticed and unrepaired. In a sense, it is a complication of these heart defects, in most cases, rather than a primary symptom appearing at birth. The failure for accurate diagnosis of heart defects that can damage the way the heart and lungs work together can create serious complications.

The typical heart defects indicated in Eisenmenger syndrome are ventricle septal defects or holes between the two bottom chambers of the heart, holes in the atria or upper two chambers of the heart, persistence of the patent ductus (a blood vessel that usually closes right after birth), or a mix of heart defects. In each case, initially, blood shunts from the left to the right side of the heart. This means oxygenated blood heads back to the right ventricle where it is pumped to the lungs.

This extra amount of blood poses a significant risk to the lungs and over time the lungs develop high pressure or pulmonary hypertension. As Eisenmenger syndrome progresses, pulmonary circulation becomes inefficient and some of vessels leading to the heart may become so rigid that they no longer accept blood flow. Blood can back up and in the heart it may begin to shunt or flow to the opposite side (right to left). This means the left side of the heart is now getting unoxygenated blood to send to the tissues, which can result in significant tissue failure. Moreover, the stiffness of the pulmonary vessels is a condition called pulmonary vascular disease, and it can be managed in some patients but it cannot be cured.

Symptoms that occur as pulmonary hypertension or pulmonary vascular disease develop include blue tone to skin especially at the extremities; this is also called cyanosis. People may have trouble breathing after minimal activity, could feel tired all of the time, may experience chest pain, and might have unusual heart rhythms. Sometimes the first symptoms of this condition is heart attack or stroke, or some people have coughing fits where they cough up blood due to the congestion in the lungs.

The main treatment of Eisenmenger syndrome is to address heart defects before they cause it. Early surgeries to prevent pulmonary vascular disease are highly desirable. Yet some people may show no signs of having heart defects, especially if those defects aren’t interfering with function in any way, or with things like early growth and development. If defects aren’t diagnosed until a later point, one of the things doctors usually do before determining repair is to determine pressure in the pulmonary circulation and to decide if pulmonary vascular disease is present. Repair may not be indicated if pressure is too high.

Treatment of Eisenmenger syndrome varies as to patient and seriousness of the condition. Some people can be well managed with different medications that improve circulation, and others benefit from things like treatment with oxygen. The most severe cases may require heart/lung transplant.