- People with Eisenmenger Syndrome are born with a hole between the the left and right ventricles of the heart (ventricular septal defect) [1]. This hole allows oxygenated blood to flow back into the lungs instead of going out to the rest of the body like it should. With this condition, pressure is higher in the pulmonary artery (pulmonary hypertension) [2]. The result is that blood backs up and does not go into the lungs to pick up oxygen. It instead travels directly from the right side of the heart to the left side, and oxygen-poor blood is pumped out to the body [1].
- Eisenmenger Syndrome is high risk for pregnant women, and in fact pregnancy is not recommended for women with this condition. This is because of the high risk of maternal mortality during pregnancy– a range of 30-50%. If a woman does want to continue with a pregnancy, it is advised that she restrict physical activity, use continuous oxygen for at least the third trimester, and use pulmonary vasodilating drugs. The most critical time for the mother is during labor and delivery and the first week of postpartum. During this period, the use of anticoagulants is also recommended [3].
Symptoms [1]:
- Cyanosis
- Clubbing of fingernails and toenails
- Numbness and tingling of fingers and toes
- Chest pain
- Coughing up blood
- Dizziness
- Syncope (fainting)
- Fatigue
- Shortness of breath
- Palpitations
- Stroke
- Gout
Treatment [4]:
- Phlebotomy (having blood drawn) may be recommended if a person’s red blood cell count becomes too high and causes symptoms. Phlebotomy should not be done routinely however, and should only be performed after consulting with a doctor. During this procedure, it is important to also obtain IV fluids.
- If no other treatments prove effective, the patient may eventually need a heart and lung transplant or a repair of the hole in the heart.
- Medications that relax and open up blood vessels (vasodilators) can also be used
Sources:
- Eisenmenger syndrome. NIH Web site. https://medlineplus.gov/ency/article/007317.htm. Accessed July, 2017.
- Eisenmenger syndrome. NIH Web site. https://rarediseases.info.nih.gov/diseases/6323/eisenmenger-syndrome. Updated October, 2010. Accessed July, 2017.
- Naderi, S, Raymond R. Pregnancy and heart disease. Dis manag. 2014. http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/cardiology/pregnancy-and-heart-disease/.
- Eisenmenger syndrome. Mayo Clinic Web site. http://www.mayoclinic.org/diseases-conditions/eisenmenger-syndrome/diagnosis-treatment/treatment/txc-20179044. Accessed July, 2017.
By: Stephanie Kramer