How is Eisenmenger syndrome treated medically?

  Patients with cardiac insufficiency need to be treated with digoxin cardiotonic therapy. If the patient does not have significant cyanosis, diuretics can be used to reduce the cardiac load; however, if the patient has significant cyanosis and high blood viscosity, diuretics may cause thrombosis or embolism in these patients, so diuretics are not recommended for patients with significant cyanosis. In addition, in patients with Eisenmenger’s syndrome, thrombosis/embolism of the pulmonary vessels and coughing up blood can occur simultaneously. Anticoagulants can prevent thrombosis/embolism of the pulmonary vessels, but can aggravate the patient’s coughing up blood, so anticoagulants are not recommended for patients with Eisenmenger’s syndrome who have symptoms such as coughing up blood. Long-term home oxygen therapy can improve the patient’s symptoms and quality of survival, and patients with this condition can receive this treatment. Again, it is important to point out that drugs for the treatment of hypertension in the body circulation, such as calcium antagonists, should not be easily used for pulmonary hypertension in precardiac disease because they can cause a decrease in pressure in the patient’s body circulation, increase the right-to-left shunt flow at the site of the defect, and the patient experiences a marked increase in cyanosis, causing a decrease in activity tolerance or even sudden death.  The method of taking prostacyclin analogs for the treatment of pulmonary hypertension is divided into oral, intravenous and nebulized inhalation. There are two types of prostacyclin analogs available in China: inhaled iloprost and oral beprost sodium, which are used to reduce pulmonary vascular pressure and resistance, improve exercise tolerance, and increase oxygen saturation by preventing intravascular thrombosis and diastolic pathways in the pulmonary vasculature. Beprostol sodium is available in oral dosage form and is relatively inexpensive. Iloprost can be administered by both nebulized inhalation and intravenous pumping. Among them, intravenous application of Iloprost has the best therapeutic effect, but it needs to be used with more weighing of the pros and cons of this treatment method because of the need to apply deep vein placement, which can increase the occurrence of cerebrovascular adverse events.