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. Patients in underdeveloped countries are more likely to present late with uncorrected congenital cardiac lesions and a markedly elevated pulmonary vascular resistance (PVR). They are more likely to be inoperable secondary to Eisenmenger physiology. Patients with Eisenmenger syndrome usually do not survive beyond the second or third decade. Long-term survival depends on the patient’s age at the onset of pulmonary hypertension and the coexistence of additional adverse features, such as Down syndrome. Survival predominantly depends on right ventricular function. The mortality rate in pregnant patients with Eisenmenger syndrome is reported to be approximately 50%, although it may be higher. The most frequent terminal event in this syndrome is a combination of hypoxemia and arrhythmia in the setting of rapid increases in pulmonary vascular resistance or decreases in systemic vascular resistance (SVR). Death also commonly results from congestive heart failure, massive hemoptysis, or thromboembolism. A study by Diller et al indicated that survival rates for untreated patients with Eisenmenger syndrome may have been overestimated in previous studies and that these rates have not improved since the 1970s. The report involved a literature review of 12 studies published between 1971 and 2013 (1131 patients total), along with an analysis of 219 contemporary, treatment-naïve patients at the investigators’ own institution.
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. Some of the treatment medications for Eisenmenger syndrome include Medications, to control arrhythmias. If you have an arrhythmia, you may receive medications to control your heart rhythms. 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). However, people who have Eisenmenger syndrome are also at increased risk of bleeding when taking these medications, so don't take any blood thinners unless your doctor tells you to do so. You shouldn't take over-the-counter pain medications, such as ibuprofen (Advil, Motrin IB, others) or naproxen (Aleve), without talking to your doctor first. Endothelin receptor antagonists: These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. One of these medications, bosentan (Tracleer), may improve your energy level and symptoms by lowering the resistance in your lung arteries. If you take bosentan, you'll need monthly liver monitoring because the drug can damage your liver. Sildenafil and tadalafil: Sildenafil (Revatio, Viagra) and tadalafil (Cialis, Adcirca) are sometimes used to treat high blood pressure in your pulmonary arteries caused by Eisenmenger syndrome. These drugs work by opening the blood vessels in the lungs to allow blood to flow through more easily. Side effects include upset stomach, dizziness and vision problems. Antibiotics: Depending on your condition, you may need to take antibiotics before having certain dental and medical procedures. These procedures may allow bacteria to enter your bloodstream. Antibiotics taken before these procedures can help destroy or control the harmful bacteria that may lead to an infection of your heart's tissues (endocarditis). Antibiotics are recommended only before certain dental procedures (those that cut your gum tissue or part of the teeth) and procedures involving the respiratory tract, infected skin or tissue that connects muscle to bone. Some of the other advanced methods are: Oxygen Therapy, Pulmonary Vasodilator Therapy, Endocarditis, Erythrocytosis, Anticoagulation, Contraception, Pregnancy, and Genetic Counseling, Transplantation and Corrective Surgery.