Pathophysiology: Eisenmenger syndrome is a complication of an uncorrected high-flow, high-pressure congenital heart anomaly leading to chronic pulmonary arterial hypertensionand shunt reversal. 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. 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 orpreeclampsia. 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 signs and symptoms include: • Bluish or grayish skin color (cyanosis) • Large, rounded fingernails or toenails (clubbing) • Easily tiring and shortness of breath with activity • Shortness of breath while at rest • Chest pain or tightness • Skipped or racing heartbeats (palpitations) • Fainting (syncope) • Dizziness • Numbness or tingling in the fingers or toes
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. Treatment: reatment for Eisenmenger phenomenon has historically been palliative, and closure of the underlying shunt is contra-indicated (remember that right to left shunts have been used to palliative patients with severe pulmonary arterial hypertension) 2. More recently some pharmacological agents show the prospect of being beneficial in pulmonary arterial hypertension, including Eisenmenger syndrome (see PAH article). In selected cases combined heart and lung transplantation can be performed.