Eisenmenger Syndrome课件.ppt

Eisenmenger Syndrome Presented by: Ri 高祥豐 Outline of Presentation Eisenmenger syndrome: introduction Definition, manifestation, natural courses, diagnosis, differential, diagnostic tests Prognostic factors, especially for corrective surgery Intervention: medical and surgical Management of complications Eisenmenger Syndrome Definition: Pulmonary vascular obstructive disease that develops as a consequence of a large preexisting left-to-right shunt that pulmonary artery pressures approach systemic levels and the direction of the flow becomes bidirectional or right to left. Eisenmenger SyndromePathophysiology Eisenmenger Syndrome Precipitating congenital heart diseases Ventricular septal defect Atrial septal defect Patent ductus arteriosis Atrio-ventricular septal defect Truncus arteriosus Aortopulmonary window Univentricular heart D-transposition of the great vessels Surgically created aorto-pulmonary connections Eisenmenger SyndromeClinical Manifestations Right to left shunt: Cyanosis (general or differential) Low cardiac output, congestive heart failure Exertional dyspnea, fatigue, syncope, orthopnea, PND, peripheral edema Neurologic symptoms: (hyperviscosity) Headache, dizziness, Congestive heart failure Others Hemoptysis, arthralgia, gout, renal dysfunctions Eisenmenger SyndromeNatural course and prognosis Age: second or third decade of age Factors determining the likelihoods of Eisenmenger syndrome Size and location of heart defects PDA, VSD: early onset (80% in childhood) ASD: delay onset (80% in adulthood) The rate of survival 10 years: 80% 15 years: 77% 25 years: 42% Poor prognostic factors High pulmonary artery resistance Syncope Elevated right heart filling pressure Severe hypoxemia NOT influenced by types of heart defects Eisenmenger SyndromeCauses of death Sudden death (30%) Congestive heart failure (25%) Hemoptysis (15%) Pregnancy Perioperative mortality of non-cardiac surgery Infectious diseases Eisenmenger SyndromeDiagnostic Testing Goals For t