The Mayo Clinic defines Eisenmenger Syndrome as a long-term complication of an unrepaired heart defect that someone was born with (congenital). Eisenmenger syndrome is characterized by congenital heart defects that cause blood to circulate abnormally in your heart and lungs. When blood flow is disrupted, the blood vessels in your lungs stiffen and narrowed increasing the pressure in your lungs' arteries (pulmonary arterial hypertension). The blood vessels in your lungs are permanently damaged as a result of this. This potentially fatal condition can usually be avoided with early detection and repair of congenital heart defects.
How does the heart function? The human heart is divided into four chambers, two on the right and two on the left, according to the Mayo Clinic. The right-side transports blood to vessels that lead to the lungs. The oxygen in your lungs enriches your blood, which then circulates to the left side of your heart. The left side of your heart pumps blood into the aorta, a large vessel that circulates blood throughout your body. Valves regulate the flow of blood into and out of your heart's chambers. These valves open to allow blood to flow to the next chamber or an artery, then close to prevent blood from flowing backward.
People with Eisenmenger syndrome are born with a hole between the two pumping chambers of the heart, the left and right ventricles (ventricular septal defect), according to Medline Plus. The hole allows blood that has already picked up oxygen from the lungs to return to the lungs rather than the rest of the body. Increased blood flow can damage the small blood vessels in the lungs over time. This results in high pulmonary blood pressure. As a result, blood flow reverses through the gap between the two pumping chambers. This allows oxygen-depleted blood to reach the rest of the body. Furthermore, Eisenmenger syndrome can develop before a child reaches puberty. It can, however, develop in young adulthood and progress throughout young adulthood.
What are the manifestations of Eisenmenger syndrome? Eisenmenger syndrome symptoms vary depending on the heart defect and affected organs, according to the Cleveland Clinic. They usually do not appear until people are in their teens, twenties, or thirties. The symptoms worsen gradually. Shortness of breath during exertion, cyanosis (bluish skin), clubbing (abnormally large and rounded fingernails and toenails), heart palpitations (abnormal or fast heartbeat), headaches, dizziness or fainting, coughing up blood, and leg swelling are the most common signs and symptoms of the condition.
A family history of heart defects increases the likelihood of a baby developing a congenital heart defect, including Eisenmenger syndrome. If you've been diagnosed with a heart defect or Eisenmenger syndrome, talk to your doctor about screening other family members for heart defects. According to Mayo Clinic, Eisenmenger syndrome complications may include low oxygen levels in your blood (cyanosis), a high red blood cell count, an irregular heart rhythm, enlargement and thickening of the heart walls, along with low oxygen levels, sudden cardiac arrest, heart failure, stroke, kidney problems, an increased risk of infection, and pregnancy risks.
According to the Cleveland Clinic, doctors use different laboratory tests to diagnose Eisenmenger syndrome. Your doctor may use an electrocardiogram, echocardiogram or cardiac catheterization, MRI scan, and blood tests in addition to a physical exam. Eisenmenger syndrome is a potentially fatal condition. The prognosis for people with Eisenmenger syndrome is determined by the type of congenital heart defect as well as other medical conditions. Some people with Eisenmenger syndrome lives into their 50s, 60s, or even beyond.
PNFP Marie-Claire Gaas, RND
References:
1. Eisenmenger syndrome
- https://www.mayoclinic.org/diseases-conditions/eisenmenger-syndrome/symptoms-causes/syc-20350580#:~:text=Eisenmenger%20syndrome%20causes%20increased%20blood,and%20oxygen%2Dpoor%20blood%20mix.
- https://my.clevelandclinic.org/health/diseases/17921-eisenmenger-syndrome
- https://medlineplus.gov/ency/article/007317.htm
