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Eisenmenger Syndrome

New Treatment Modalities for Pulmonary Hypertension

For children born with congenital heart defects, such as large holes or large connections between the great arteries, they are at risk for developing a progressive blood vessel disease in their lungs if their heart defects are not repaired early in life. These types of congenital heart defects allow excessive blood flow to the lungs, and over the first 7 years of life these blood vessels can become very restrictive to flow in response to this abnormal influx of blood flow. If this restriction of blood flow progresses to the peripheral blood vessels in the lungs, it is called pulmonary vascular disease. This usually marks the onset of "permanent" pulmonary hypertension (or pulmonary high blood pressure), and this type of blood vessel disease is not known to be reversible. For this reason, these large defects usually are repaired in the first 5-7 years of life, to avoid the life-long effects of pulmonary hypertension.

Once pulmonary hypertension occurs, we term the combination of a heart defect and pulmonary hypertension as "Eisenmenger syndrome". In the setting of Eisenmenger syndrome, closing the heart defect can be potentially dangerous rather than beneficial, so children with Eisenmenger syndrome confront the lifelong effects of this disease, without a surgical repair option. The symptoms that accompany this problem include breathlessness with activity, bluish discoloration of the skin (particularly nose, lips, gums, and fingers), and several other blood and organ changes that occur with aging in the setting of low oxygen levels. The low oxygen levels are the result of blood passing across the defect in the heart without first picking up oxygen in the lungs.  

Historically, it has been reported that patients with Eisenmenger syndrome confront early mortality, with 77% survival at 15 years of age and 42% survival at 25 years of age.  The most common cause of death in these patients is sudden heart rhythm problems, heart failure, or lung hemorrhage. With advances in the recognition of risks confronted by adults with Eisenmenger syndrome, and evolving modalities to minimize the risks of bleeding, and minimize the evolution of heart failure, many of these adults are living well into their 40's, 50's, and even into their 60's. 

In the past, it was believed that the use of medications to reduce pulmonary blood pressure would be too risky in patients with Eisenmenger syndrome, since they would be susceptible to a decrease in their body's blood pressure which could make their oxygen levels drop dangerously. However, studies over the past decade have dispelled this myth and have led to wide-spread use of new drugs focused at reducing pulmonary blood pressure. Current studies have shown that these advanced therapies both improve quality of life, and may have a beneficial impact on survival.

The specialized drugs that are used to treat pulmonary hypertension fall into three main categories and may be used separately or in combination: 

  1. Phosphodiesterase-5 enzyme inhibitors, such as revatio or adcirca.  
  2. Endothelin antagonists, such as tracleer or letairis.  
  3. Prostanoids, which comes in an intravenous form called flolan, a skin delivery system called remodulin, and an inhaled version such as ventavis or tyvaso.


The Ahmanson/UCLA Adult Congenital Heart Disease Center is beginning a research study to evaluate the impact of inhaled ventavis on exercise capacity in our Eisenmenger syndrome patients. This is a 3 month trial, in which the medication and all associated testing is provided free of cost. If you are interested in learning more about these advanced therapies, or participating in the ventavis study, please call or email  (310-794-9629 or achdc@mednet.ucla.edu).