高血压英文ppt精品课件eisenmenger

Eisenmenger Syndrome Presented by: Ri 高祥豐 Outline of Presentation n Eisenmenger syndrome: introduction Definition, manifestation, natural courses, diagnosis, differential, diagnostic tests n Prognostic factors, especially for corrective surgery n Intervention: medical and surgical n Management of complications Eisenmenger Syndrome n 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;18:533-47 Grade 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 n 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 n Right to left shunt: Cyanosis (general or differential) n Low cardiac output, congestive heart failure Exertional dyspnea, fatigue, syncope, orthopnea, PND, peripheral edema n Neurologic symptoms: (hyperviscosity) Headache, dizziness, Congestive heart failure n 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 n Age: second or third decade of age n 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) n The rate of survival 10 years: 80% 15 years: 77% 25 years: 42% n 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 n Sudden death (30%) n Congestive heart failure (25%) n Hemoptysis (15%) n Pregnancy n Perioperative mortality of non-cardiac surgery n 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 n Goals For the diagnosis of heart defect For evaluating the severity For stratification, predictable prognostic factors? For surgery? n Choices Electrocardiography n RAE, RVH, right axis deviation, arrhythmia Chest X ray n Cardiomegaly, dilated pulmonary arteries, pulmonary artery calcification Echocardiography: TEE is preferred n 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 n Goals: to detect, localize, and quantitate intracardiac shunting and to determine the severity of pulmonary vascular disease n What can we measure? Qp / Qs Rp / Rs n Pulmonary arteriolar vasodilator in cardiac catheterization What we want to know? reversibility Agents: 100% oxygen, nitric oxide, tolazoline, adenosine triphosphate, prostacyclin n 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 n Nitric oxide: Am J Cardiol. 1996;77:532-5 n ATP: Circulation. 1994;90:1287-93 n NO and ATP: Cut point? Prognosis? Difficult to intervention n Contrast media should be avoided It may cause hypotension, which could be lethal in these patients Eisenmenger Syndrome: Cardiac catheterization n 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 n 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 n Qp/Qs 1.5 n Rp/Rs 2/3, but Qp/Qs 1.5, significant left-to-right shunt, reversibility(+), lung biopsy? n Pulmonary vascular resistance 92%: 92% of long-term survival 65%, or Hct 65% with iron deficiency or microcytic anemia n Treatment Correct dehydration Iron supplement Phlebotomy if symptomatic n Prophylactic phlebotomy is not suggested n May cause iron deficiency anemia Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1617-1618 Ann Intern Med 1998; 128: 745-755 Eisenmenger Syndrome Phlebotomy Ann Intern Med 1998; 128: 745-755 Eisenmenger Syndrome: Complications Cerebrovascular events n Hyperviscosity thrombosis, emboli n Abnormal hemostasis hemorrhage n Right to left shunt brain abscess, paradoxical emboli n Prevention Phlebotomy has no rule in prevention Correct microcytic anemia Avoid air in peripheral IV intervention Control atrial fibrillation Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1617-1618 Ann Intern Med 1998; 128: 745-755 Eisenmenger Syndrome Hemoptysis: cause and therapy Ann Intern Med 1998; 128: 745-755 Eisenmenger Syndrome: Complications Renal dysfunctions n More than 1/3 of patients n Manifestations Proteinuria, elevated serum creatinine, diminish GFR, hyperuricemia, renal failure n Preventions: avoid nephrotoxic drugs Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1617-1618 Ann Intern Med 1998; 128: 745-755 Eisenmenger Syndrome: Complications Gout n Rare n Pathophysiology ? Increase resorption of uric acid Increase production of uric acid and impaired excretion n Treatment Colchicine Avoid NSAIDs Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1617-1618 Ann Intern Med 1998; 128: 745-755 Eisenmenger Syndrome: Complications Hypertrophic Osteoarthropathy n Mechanism: Megakaryocytes bypass the lung (due to right to left shunt) induce PDRF in peripheral promoting local cell proliferation osseous formation bone pain, arthralgia n Management: Salsalate: nonacetylated analog of aspirin Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1617-1618 Ann Intern Med 1998; 128: 745-755 Eisenmenger Syndrome: Complications Cholethiasis n Elevated unconjugated bilirubin in bile secretions as a results of the increased erythrocyte mass increasing risk for developing gall stone. n Management: Asymptomatic cholelithiasis: conservative Symptomatic cholelithiasis: not known Cholecystitis: surgery Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1617-