eisenmenger syndrome课件

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;18:533-47 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,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,Eisenmenger Syndrome Open lung biopsy,Pathological results Cardovasc Pathol. 2002 11(4): 221-8 20 cases with or without corrective surgery Fatal outcome is frequently associated with fibrinoid necrosis of small pulmonary arteries Kyobu Geka. 2001; 54(5): 374-8 Single case report: 10 m/o, Down, VSD, Eisenmenger Lung biopsy: Grade 2 Heath Edwards classificationResult: Survived Conclusion: evidence is weak! High grade lesions may be missed in a random biopsy Progression of structural changes can occur even in the absence of high grade changes Risk for biopsy?,Pulmonary Hypertension: Differential diagnosis,Pulmonary thrombo-embolism (a. or v.) Congenital heart diseases mitral or aortic valve diseases, LV dysfunction, systemic HTN Pulmonary airway disease Hypoxic pulmonary hypertension Interstitial lung disease Collagen-vascular disease Parasitic disease (ex. schistosomiasis) Peripheral pulmonary artery stenosis (ex. Takayasu) Cirrhosis with portal hypertension Sickle cell disease Primary pulmonary hypertension,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1922,Eisenmenger Syndrome Interventions,Principles: To avoid any factors that may destabilize the delicately balanced physiology An approach of nonintervention is recommended. Indications for intervention Preventing complications Flu vaccines, IE prophylaxis Restore the physiological balance Iron deficiency anemia, anti-arrhythmic management, digoxin and diuretics for right-side heart failure,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1614 1616 Ann Intern Med 1998; 128: 745-755,Eisenmenger Syndrome Interventions,Medical managements under investigations Nifedipine Oxygen therapy Prostacyclin Nitric oxide Managements of complications Corrective or palliative surgery Lung or heart-lung transplantation,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1614 1616 Ann Intern Med 1998; 128: 745-755,Eisenmenger Syndrome: corrective or palliative surgery Surgery : How to do?,Pulmonary banding Arq Bras Cardiol. 1997 69(5): 369-72 Pulmonary banding in one patient with biopsy-proven irreversible pulmonary vascular changes led to regression of pulmonary vascular changes, which made surgical closure of the defects possible.,Eisenmenger Syndrome: Medical treatment Nifedipine,Cardiovascular Drugs and Therapy 1992; 6(2): 183-6 10 children, 3-12 years of age, VSD, AVSD, Eisenmenger Nifedipine had a relaxing effect on the pulmonary bed, especially in the younger child with Eisenmenger;s mechanism. Clinical Cardiology 1991;14(12):957-61 4 patients, age? VSD, Eisenmenger Increase right to left shunt, without compromise SpO2 Chronic (4 weeks) nifedipine therapy increases SaO2 on exercise and improves maximal exercise capacity in patients with Eisenmenger syndrome. Ann Intern Med 1998; 128: 745-755 Do not suggest: due to worries of syncope and sudden death Braunwald: Heart Disease 6th: 1614-1616 Should be keep in investigation,Eisenmenger Syndrome: Medical treatment Oxygen Therapy,Br Heart J 1986; 55:385-90 15 childrens with pulmonary disease, not randomized Treatment: long term domiciliary oxygen for a minimum of 12 hours a day for up to 5 years Results: nine treated children survived. Sixed untreated children died. AJRCCM 2001 164(9): 1682-7 23 patients (mean age: 32 +/- 6) with post-tricuspid congenital heart defects (VSD: 10, PDA: 13) and Eisenmenger. Severity: pulmonary hypertension, erythrocytosis, limited exercise endurance Randomized trial. 2 years follow up Treatment: nocturnal oxygen therapy, at least 8 hrs a day Results: it did not modify the natural history of patients with advanced Eisenmenger Syndrome Conclusions Evidence: small case size, nonrandomized vs randomized, controversial Good for children, not good for adults?,Eisenmenger Syndrome: Medical treatment Inhaled Nitric Oxide,Inhaled NO in pulmonary hypertension secondary to congenital heart diseases Heart 2001;86:553558 23 patients enrolled. Mean Qp/Qs: 1.1 (SD: 0.9) Mean SBP / PAP: 89 (22) / 72 (30) ASD, VSD, PDA, TGA, TOF, DORV, truncus arteriosis Inhaled NO, at 20 ppm and 80 ppm Results What determines “effective”? Total pulmonary vascular resistance was reduced by more than 20% 20 ppm:18%, 95% CI 2% to 34% 80 ppm: 29%, 95% CI 10% to 38% Qp/Qs 1: remained unchanged!,Eisenmenger Syndrome: Medical treatment Inhaled Nitric Oxide & Pregnancy,Only 2 cases was reported Am J Obstet Gynecol. 1999 Jan;180(1 Pt 1):64-7. Am J Obstet Gynecol. 1999 Aug;181(2):419-23. Inhaled NO during labor 2 cases delivered successfully, but died thereafter (21 days and 2 days postpartum),Eisenmenger Syndrome: Medical treatment Inhaled Nitric Oxide,Inhaled NO is effective in primary pulmonary hypertension (NEJM 1997;336:111-117) and neonatal PPHN (NEJM 1997;336:605-610) Inhaled NO may not be effective in patients Qp/Qs 1 Inhaled NO may be helpful for pregnant woman in delivery,Eisenmenger Syndrome Complications,Ann Intern Med 1998; 128: 745-755,Eisenmenger Syndrome: Complications Hemostatic problems,Common Problems Thrombocytopenia Prolonged bleeding time, PT/PTT Deficiency in Vit. K dependent clotting factors Increased fibrinolytic activity Common Manifestations Mucocutaneous bleeding Epistaxis, hemoptysis, massive bleeding Pathogenesis Not fully understood Management Blood component therapy Avoidance of NSAIDs, heparin,Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine, P 1617-1618 Ann Intern Med 1998; 128: 745-755,Eisenmenger Syndrome: Complications Hyperviscosity Syndrome,Manifestations Headaches, altered mentation, visual disturbances, tinnitus, paresthesis, fatigue, dizziness, myalgia Hct 65%, or Hct 65% with iron deficiency or microcytic anemia Treatment Correct dehydration Iron supplement Phlebotomy if symptomatic Prophylactic phlebotomy is not suggested 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,Hyperviscosity thrombosis, emboli Abnormal hemostasis hemorrhage Right to left shunt brain abscess, paradoxical emboli 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,More than 1/3 of patients Manifestations Proteinuria, elevated serum creatinine, diminish GFR, hyperuricemia, renal failure 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,Rare Pathophysiology ? Increase resorption of uric acid Increase production of uric acid and impaired excretion 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,Mechanism: Megakaryocytes bypass the lung (due to right to left shunt) induce PDRF in peripheral promoting local cell proliferati