Eisenmenger Syndrome Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

Definition of Eisenmenger Syndrome First used by Paul Wood, is defined as pulmonary vascular obstructive disease that develop as a consequence of a large preexisting left-to-right shunt such that pulmonary artery pressures approach systemic levels that direction of flow becomes bidirectional or right-to-left shunt The high pulmonary vascular resistance is usually established in infancy, and can sometimes be present from birth

Natural History Usually healthy childhood, and gradually become progressively cyanotic with each succeeding decade. Complications tend to occur from 3 rd decade onward, and congestive heart failure, the most common, usually occurs after 40 years Most survive to adulthood with 77% & 42% survival rate at 15 and 25 years age. Common modes of death are sudden death(30%), CHF(25%), hemoptysis(15%), pregnancy, following surgery, and infectious causes (brain abscess, endocarditis) account for most remainder.

Complications Congestive heart failure Arrhythmias (atrial fibrillation/flutter) Bleeding disorders Brain abscess Erythrocytosis Hyperuricemia/gout Infectious endocarditis Paradoxical emboli Pulmonary artery calcification & aneurysm Progressive valvular disease Renal dysfunction Sudden death Stroke or transient ischemic attack

Indications for Intervention  Avoid factors that may destabilize the delicately balanced physiology, in general, an approach of nonintervention is recommended.  Main interventions are directed toward preventing complications or to restore physiological balance Isovolumic phlebotomy unless iron deficiency anemia Hypovolemia be avoided Noncardiac surgery only when necessary without general anesthesia Hemoptysis commonly due to bronchial vessel or pulmonary infarction Influenza shots, antiarrhythmic management, salt restriction Endocarditis prophylaxis is recommended  Transplantation

Risks for Eisenmenger Syndrome Pregnancy (contraindicated) General anesthesia Dehydration Hemorrhage Cardiac and noncardiac surgery Drugs (vasodilator, diuretics,oral pill, nonsteroidal anti-inflammatory drugs) Anemia commonly due to iron deficiency Intravenous lines (air embolism,infection) Altitute exposure Pulmonary infection

Interventional Options  Phlebotomy with fluid replacement in symptomatic from erythrocytosis  Supplement oxygen at home,although not routine  Transplantation  Investigational therapy. Calcium channel blockers; nifedipine. ACE inhibitor; controversal. Prostacyclin. Pulmonary artery banding