Eisenmenger Syndrome

Form of pulmonary hypertension

Eisenmenger syndrome develops in individuals with significant heart defects. It may also occur in individuals who have surgically-created shunts that divert blood from the left side of the heart to the right side. Either way, the extra blood on the right side of the heart creates increased blood flow to the lungs. Blood pressure increases in the lungs (pulmonary hypertension). This is called Eisenmenger syndrome.

Usually, Eisenmenger syndrome develops while individuals with heart defects are still children, but it may occur in adolescence or young adulthood. Infants born with a large hole between the ventricles of the heart (ventricular septal defect) or whose ductus arteriosus fails to close after birth (patent ductus arteriosus) are at high risk for developing Eisenmenger syndrome. The syndrome affects both males and females.

Symptoms

The symptoms of Eisenmenger syndrome are the same as those of pulmonary hypertension:

• Shortness of breath, especially with activity
• Extreme tiredness, lethargy
• Fainting, dizziness

Individuals with Eisenmenger syndrome usually have other symptoms, as well. These are caused by complications of their heart defects and can lead to heart failure, bleeding and clotting issues, and gallbladder or kidney problems.

Diagnosis

Eisenmenger syndrome is first suspected when an individual begins to show symptoms of pulmonary hypertension. An echocardiogram (ultrasound of the heart, also known as an ECG) can help confirm the diagnosis, as can chest x-ray. Those with the syndrome will almost always have abnormal ECG results, so a cardiac catheterization is often used to detect the severity of the pulmonary hypertension.

Treatment

Individuals with Eisenmenger syndrome not only need treatment for their pulmonary hypertension, but also for their underlying heart defect. Individuals should not participate in competitive sports or any intense athletic activities, since their hearts are not up to the physical challenge. Those with Eisenmenger syndrome are also at risk for heart damage caused by infection (bacterial endocarditis), and need to take antibiotics before dental or surgical procedures.

Women with Eisenmenger syndrome should avoid becoming pregnant because pregnancy puts both their own lives and the lives of their unborn babies at risk. If a woman with Eisenmenger syndrome becomes pregnant, she faces a greater than 50% risk of death during labor or immediately after delivery. Her fetus faces a 28% risk of death and an 85% chance of being born prematurely.

It is possible for an individual with Eisenmenger syndrome to receive a heart-lung transplant. Once Eisenmenger syndrome begins, in most cases, the underlying heart defect can no longer be repaired. Survival rates after heart-lung transplant have improved over the years, but are still about 68% one year after surgery and 43% after 5 years.

_Source:

"Detailed Description of Eisenmenger's Syndrome." Mayo Clinic. Mayo Foundation for Medical Education and Research. 3 Aug 2007

"Eisenmenger's Syndrome." Congenital Heart Disease. 8 May 2007. UCSF Medical Center. 3 Aug 2007