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.,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1614 - 1616,Eisenmenger Syndrome Pathophysiology,Ann Intern Med 1998; 128: 745-755,Heath-Edwards Classifications Circulation 1958;18:533-47Grade I: Arteriolar medial hypertrophy Grade II: Intimal proliferation Grade III: intimal fibrosis, occlusion Grade IV: Plexiform lesions Grade V: Hemosiderin-filled macrophage Grade VI: Necrotizing arteritis,NEJM 2000; 342(5); 334-342,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,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1614 1616 Ann Intern Med 1998; 128: 745-755,Eisenmenger Syndrome Clinical 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,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1614 1616 Ann Intern Med 1998; 128: 745-755,Eisenmenger Syndrome Natural 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,Ann Intern Med 1998; 128: 745-755 NEJM 1993; 329:864-872,Eisenmenger Syndrome Causes of death,Sudden death (30%) Congestive heart failure (25%) Hemoptysis (15%) Pregnancy Perioperative mortality of non-cardiac surgery Infectious diseases,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1614 1616 Ann Intern Med 1998; 128: 745-755,Eisenmenger Syndrome Diagnostic Testing,Goals For the diagnosis of heart defect For evaluating the severity For stratification, predictable prognostic factors? For surgery? Choices Electrocardiography RAE, RVH, right axis deviation, arrhythmia Chest X ray Cardiomegaly, dilated pulmonary arteries, pulmonary artery calcification Echocardiography: TEE is preferred Heart defect, direction of shunting, pulmonary hypertension Cardiac catheterization Open lung biopsy,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1614 1616 Ann Intern Med 1998; 128: 745-755,Eisenmenger Syndrome Cardiac catheterization,Goals: to detect, localize, and quantitate intracardiac shunting and to determine the severity of pulmonary vascular disease What can we measure? Qp / Qs Rp / Rs Pulmonary arteriolar vasodilator in cardiac catheterization What we want to know? reversibility Agents: 100% oxygen, nitric oxide, tolazoline, adenosine triphosphate, prostacyclin 100% O2: Circulation 1959;20:66-73 / NEJM 1993; 329(12):864-872 A fall to less than 80% of base line: 80% survival after surgery Remain higher than 80%: 27% survival after surgery Nitric oxide: Am J Cardiol. 1996;77:532-5 ATP: Circulation. 1994;90:1287-93 NO and ATP: Cut point? Prognosis? Difficult to intervention Contrast media should be avoided It may cause hypotension, which could be lethal in these patients,Eisenmenger Syndrome: Cardiac catheterization,Cautions: NEJM 1993; 329(12):864-872 Oxygen consumption should be measured directly rather than assumed Agitated patient systemic blood pressure higher than before polycythemia can independently elevate resistance due to an increase in viscosity In infant, Hct 50% - 40%: viscosity decrease 30% Measure the blood pressure and partial pressure of oxygen completely, before and after the administration of vesodilators.,Eisenmenger Syndrome Surgical Criteria: ASD,Qp/Qs 1.5 Rp/Rs 2/3, but Qp/Qs 1.5, significant left-to-right shunt, reversibility(+), lung biopsy? Pulmonary vascular resistance 92%: 92% of long-term survival 92%: 50% of long-term survival,Braunwald E. Heart Disease 6th, 1526 1527, 1594 - 1595 Park: Pediatric Cardiology for Practitioners, 4th ed. P132 NEJM 1993; 329(12):864-872,