pediatrics
Eisenmenger Syndrome
Symptoms and Signs of Eisenmenger Syndrome
Symptoms of Eisenmenger syndrome develop at different ages depending on the cause. Patients with pre-tricuspid left-to-right shunts (ASD, partial anomalous pulmonary venous return) usually do not develop symptoms until later in life (age 20 to 40 years). However, patients with unrepaired post-tricuspid shunts (VSD, PDA, or more complex congenital heart disease) may develop irreversible, symptomatic pulmonary vascular disease within the first few years of life. Symptoms include cyanosis, syncope, dyspnea during exertion, fatigue, chest pain, and palpitations. Some patients die suddenly. Hemoptysis is a late symptom. Manifestations of Ischemic Stroke , Secondary Erythrocytosis commonly causes symptoms (eg, transient ischemic attacks with slurred speech or other neurologic symptoms, vision problems, headaches, increased fatigue, signs of thromboembolism). Abdominal pain may result from Physical examination detects central cyanosis and digital clubbing. Signs of Heart failure (eg, hepatomegaly, peripheral edema, jugular venous distention) may be present. Palpation may reveal a right ventricular heave. A holosystolic murmur of tricuspid regurgitation may be present at the lower left sternal border. An early diastolic, decrescendo, high-pitched murmur of pulmonic regurgitation may be audible along the left sternal border. A loud, single second heart sound (S2) is a constant finding; a pulmonary ejection sound (click) is common. An unusual finding of clubbing and cyanosis of only the lower extremities indicates the cause of the cyanosis is right-to-left shunting through an unrepaired large PDA directed primarily to the descending aorta. 