Eisenmenger's syndrome (or ES, Eisenmenger's reaction or tardive cyanosis) is defined as the process in which a left to right shunt caused by a congenital heart defect in the fetal heart causes increased flow through the pulmonary vasculature, causing pulmonary hypertension, which in turn causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. Eisenmenger syndrome is a cyanotic heart defect characterized by a long-standing intracardiac shunt (caused by ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) that eventually reverses to a right-to-left shunt. This syndrome is less frequent today because of medical screening with echocardiography early in life.
Signs and symptoms of Eisenmenger syndrome include the following: Cyanosis (a blue tinge to the skin resulting from lack of oxygen), High red blood cell count, Swollen or clubbed finger tips (clubbing), Fainting (also known as syncope), Heart failure, Abnormal heart rhythms, Bleeding disorders, Coughing up blood, Iron deficiency, Infections (endocarditis and pneumonia), Kidney problems, Stroke, Gout (rarely) due to increased uric acid resorption and production with impaired excretion, Gallstones, Cyanosis (pale blue or grayish skin due to decreased oxygen in the blood), Dyspnea, Shortness of breath at rest, Fatigue, Chest pain or chest tightness, Heart palpitations, Headache, Dizziness or syncope, Paresthesias, Blurred vision. Eisenmenger's syndrome can cause serious complications in pregnancy, though successful delivery has been reported.
Maternal mortality ranges from 30% to 60%, and may be attributed to fainting spells, thromboembolism, hypovolemia, hemoptysis or preeclampsia. Most deaths occur either during or within the first weeks after delivery. Pregnant women with Eisenmenger syndrome should be hospitalized after the 20th week of pregnancy - or earlier if clinical deterioration occurs. Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood.
Eisenmenger syndrome treatment is aimed at controlling your or your child's symptoms and managing the condition. Although there's no cure, medications may help you feel better, improve your quality of life and prevent serious complications. Doctors don't recommend surgery to repair the hole in your heart once Eisenmenger syndrome has developed, and any surgery may be life-threatening. It's important that you're treated by a doctor who has expertise in Eisenmenger syndrome. Iron supplements: Your doctor may prescribe iron supplements if he or she finds your iron level is too low. Don't start taking iron supplements without talking to your doctor first. Aspirin or other blood-thinning medications: If you have had a stroke, blood clot or certain types of irregular heart rhythms, your doctor may recommend aspirin or other blood thinners such as warfarin (Coumadin, Jantoven). Endothelin receptor antagonists: These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. Some of the other advanced methods are: Oxygen Therapy, Pulmonary Vasodilator Therapy, Endocarditis, Erythrocytosis, Anticoagulation, Contraception, Pregnancy, and Genetic Counseling, Transplantation and Corrective Surgery.