About eisenmenger complex

What is eisenmenger complex?

General Discussion

Eisenmenger syndrome is a rare progressive heart condition that develops in some individuals with structural malformations of the heart that are present from birth (congenital heart defects). The disorder is characterized by increased blood pressure in the main blood vessel (pulmonary artery) connecting the heart to the lungs (pulmonary hypertension) and improper blood flow within the heart.

The normal heart has four chambers. The two upper chambers, known as atria, are separated from each other by a fibrous partition known as the atrial septum. The two lower chambers are known as ventricles and are separated from each other by the ventricular septum. Valves connect the atria (left and right) to their respective ventricles. The valves allow for blood to be pumped through the chambers. Blood travels from the right ventricle through the pulmonary artery to the lungs where it receives oxygen. The blood returns to the heart through pulmonary veins and enters the left ventricle. The left ventricle sends the now oxygen-filled blood into the main artery of the body (aorta). The aorta sends the blood throughout the body.

The most common congenital heart defect associated with Eisenmenger syndrome is a ventricular septal defect (VSD) or a "hole in the heart" between the two lower chambers of the heart (left ventricle and right ventricle. This defect allows blood to flow from the left ventricle into the right ventricle (left-to-right shunt). The shunt causes increased blood flow into the lungs eventually resulting in pulmonary hypertension, which causes progressive damage to the small blood vessels in lungs (pulmonary vascular disease). As the damage continues, pulmonary hypertension increases and the small blood vessels become thickened or blocked hampering the flow of blood. Ultimately, blood flow is reversed back through the shunt so that blood flows from the right ventricle into the left ventricle (right-to-left shunt) bypassing the lungs completely. A variety of symptoms including life-threatening complications may occur.

Eisenmenger syndrome specifically refers to the combination of pulmonary hypertension and right-to-left shunting of the blood within the heart.

What are the symptoms for eisenmenger complex?

Eisenmenger syndrome signs and symptoms include:


What are the causes for eisenmenger complex?

Specific genes that cause Eisenmenger syndrome have not been identified and the condition is not thought to be inherited.

Eisenmenger syndrome is caused by a defect in the structure of the heart, more specifically a ventricular septal defect (VSD) or other shunt. A VSD is a hole in the heart in the region that connects the left ventricle and the right ventricle. At birth, this hole allows large amounts of blood to flow between the two chambers, increase the volume of blood traveling to the lungs and increasing lung pressures, damaging the small blood vessels in the lungs. The rise in lung pressures due to often irreversible changes in the lung vessels (pulmonary vascular disease) is called pulmonary arterial hypertension.

Without early surgical correction of the underlying defect, the damage in the small arteries within the lung leads to increasing resistance to blood flow. The increasing pressure in the right ventricle can surpass that of the left ventricle and, when this happens, “blue” blood flow from the right to the left ventricle through the VSD (bidirectional, reversed or right-to-left shunt), leading to insufficient oxygen supply to the body (hypoxia), bluish discoloration of the skin and mucous membranes (cyanosis), elevated levels of circulating red blood cells, and other findings characteristic of Eisenmenger syndrome.

What are the treatments for eisenmenger complex?

Treatment The treatment of Eisenmenger syndrome should be managed by a medical team with expertise in both congenital heart disease (cardiologist) and pulmonary hypertension (cardiologist or pulmonologist), with help from other specialists, e.g. hematologists, radiologists, anesthetists etc. The goal of treatment is to minimize symptoms, and the treatment often aligns with patients who are being treated for other types of pulmonary arterial hypertension.

Currently, Eisenmenger patients are treated with pulmonary arterial hypertension therapies aimed at reducing the lung resistances and increase the amount of blood flowing through the lungs, hence delivering more oxygen to the body and reducing the load to the heart. Additional medications used include diuretics that reduce the amount of fluid in the body and, at times, medication to prevent blood clots. Beta blockers and especially calcium channel blockers are avoided, because they have a negative effect on the right ventricle. Iron supplementation may be necessary for individuals with iron deficiency anemia.

Affected individuals may have an increased risk of developing bacterial infections of the heart lining and valves (bacterial endocarditis); therefore, disease management includes the administration of appropriate antibiotics (antibiotic prophylaxis) prior to dental visits, for certain oral procedures. In addition, anesthesia and sedation carry significant risks and should be avoided. Careful monitoring during anesthesia is essential for any patients undergoing essential unavoidable surgical procedures, which should be performed in expert centers. Pregnancy should be avoided in women with pulmonary hypertension and Eisenmenger syndrome since it carries significant risks for both the mother and the developing fetus. Thus, it is essential that affected women have a thorough understanding of such risks and receive information, support, and guidance from their physicians and other members of their healthcare team concerning appropriate options to prevent pregnancy. Oxygen therapy has also been met with controversial reviews. There is no data to support its use as a mean for increasing exercise capacity or survival in adult patients. However, it has been seen to help patients with advanced disease in need of a heart-lung transplant or for nocturnal support.

Individuals with Eisenmenger syndrome should avoid dehydration, high altitudes, and activities that could cause a sudden drop in blood pressure such as saunas, steam rooms or hot tubs. Extreme physical exercise should also be limited. In severely affected patients with physical deterioration, a heart-lung transplant may be necessary.

What are the risk factors for eisenmenger complex?

A family history of heart defects also increases the risk of a baby developing a congenital heart defect, including the possibility of developing Eisenmenger syndrome. Talk to your doctor about screening other family members for heart defects if you've been diagnosed with a heart defect or Eisenmenger syndrome.

Is there a cure/medications for eisenmenger complex?

Eisenmenger syndrome affects some people who have structural heart defects from birth, more specifically, a ventricular septal defect (VSD) or another shunt. A VSD is a hole in the heart that connects the left and right ventricles. It interferes with the flow of blood from the heart to the lungs, which results in abnormal blood circulation. People with this condition are born with a hole between the two pumping chambers of their hearts.

  • Although there's no cure, Eisenmenger syndrome is primarily treated with medications that may help you feel better, improve your quality of life and prevent serious complications.
  • Diagnosis: Cardiac catheterization and echocardiography are primarily performed to check the pressure in the blood.
  • Medications can help you control your irregular heartbeats and bring them in line with a normal heart rhythm.
  • If your red blood cell count rises to a level that causes headaches, vision problems, or other symptoms, phlebotomy is done. This should only be done after consultation with a congenital expert.
  • Blood thinners are also prescribed to patients, but only if they have experienced a stroke earlier in their life. Blood thinners should only be used under proper supervision because they can have serious side effects.
  • Endothelin receptor antagonists are prescription drugs that help you feel more energetic by lowering the resistance in your lung arteries. preventing endothelin's impact
    Cyanosis,Rounding of the tips of the fingers and toes (clubbing),Shortness of breath,Fatigue,Lethargy, or arrhythmias,Reduced oxygen in the blood,High pressure in the lungs
    Shortness of breath while moving around or at rest,Tightness or pain in the chest,Skipping or racing heartbeats (palpitations),Dizziness,Fainting

Video related to eisenmenger complex