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EISENMENGER SYNDROME

EISENMENGER SYNDROME. DR SANDEEP R SR CARDIO 70 SLIDES. FIRST DESCRIPTION….

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EISENMENGER SYNDROME

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  1. EISENMENGER SYNDROME DR SANDEEP R SR CARDIO 70 SLIDES

  2. FIRST DESCRIPTION…. “The patient was a powerfully built man of 32 who gave a history of cyanosis and moderate breathlessness since infancy.He managed well enough ,until January 1894 when dyspnoea increased and edema set in. Seven months later he was admitted to hospital in a state of heart failure.He improved with rest and digitalis, but collapsed and died more or less suddenly on November 13 following a large haemoptysis. At necropsy , a 2 – to 2.5 cm defect was found in the perimembranous septum along with overriding of aorta”

  3. HISTORY 1897: Victor Eisenmenger Austrian Physician described history and postmortem details of 32 year old man with VSD and cyanosis

  4. EISENMENGER SYNDROME • 1958: Paul Wood’s Croonian Lectures coined the term “Eisenmenger Syndrome” • 8% of first 1000 cases of CHD in WOOD’S SERIES • Prevalence decreased to 4% in recent studies

  5. Eisenmenger Syndrome Definition: Pulmonary hypertension at or near systemic level with reversed or bidirectional shunt between the pulmonary and systemic circulation and pulmonary vascular resistance above 800dyn/cm-5 (10 Wood Units) Paul Wood, Br Med J, 1958

  6. EISENMENGER’SCOMPLEX • VSD with reversed shunt in absence of pulmonary stenosis • Reversed shunt was initially attributed to overriding of aorta • This term was coined by MAUDE ABOTT in 1927 • Later found to be due to increased PVR by PAULWOOD PAULWOOD;DISEASES OF THE HEART & CIRCULATION:3RD EDITION:CHAPTER 8;467- 499

  7. EISENMENGERREACTION • The gradual process of development of pulmonary hypertension and pulmonary vascular disease in a large left to right shunt lesions sooner or later leading to bidirectional or reversed shunt • It prevents natural process of lowering the pulmonary vascular resistance(PVR) after birth to normal PAULWOOD;DISEASES OF THE HEART & CIRCULATION:3RD EDITION:CHAPTER 8;467- 499

  8. CAUSES OF EISENMENGER’S • PRE TRICUSPID SHUNT LESIONS • ASD-OSTIUM SECONDUM • OSTIUM PRIMUM • SINUS VENOSUS • TAPVC/PAPVC • POST TRICUSPID SHUNT LESIONS • VSD • PDA • AP WINDOW • COMPLEX CCHD • COMPLETE AVSD • TGA WITH VSD/PDA • TRUNCUS ARTERIOSUS • SINGLE VENTRICLE PHYSIOLOGY WITH UNINTERRUPTED PBF

  9. PHYSIOLOGICAL CHANGES AFTER BIRTH • In fetus • there is minimal pulmonary circulation • 5 to 10% of cardiac output through lungs • Systemic & pulmonary pressures are same and PVR is high( 8-10 wood units) After birth • Systemic vascular resistance increases • PVR falls rapidly to systemic level at birth and then gradually decreases to adult level by 6 to 8 weeks

  10. PHYSIOLOGICAL CHANGES AFTER BIRTH Reasons for sudden decrease in PVR • Breathing causes expansion of lungs & pulmonary vessels – straightening of kinked pulmonary vessels • As blood flows through arteries to capillaries the the PVR • Increased oxygen content reflexly produces vasodilation & PVR • Change in elasticity of pulmonary arteries • Gradual decrease of PVR -6-8 WKS • Due to regression of the medial muscular layer • Due to increase in number of alveolar units

  11. FACTORS FAVOURING EISENMENGER RN. • Failure of regression of thickened muscular arteries which are present in fetus • Persistence of long densely packed elastic fibres in large pulmonary arteries resembling aorta • Decrease arterial oxygen saturation due to any cause • Abnormal contractile response of pulmonary vasculature to increase flow ARTERIAL REMODELLING Progress in Pediatric Cardiology 12 (Ž001.) 223247

  12. ENDOTHELIAL DYSFUNCTION Imbalance b/w vasoconstrictor & vasodilators • Endothelins,thromboxane A2 • prostacycline, NO Pathology of pulmonary hypertension Progress in Pediatric Cardiology 12 (Ž001). 223-247

  13. Eisenmenger Syndrome – A progressive disease

  14. HEATH EDWARDS CLASSIFICATION OF PAH • GRADE I – Medial hypertrophy in small PA • GRADE II – Medial hypetrophy + intimal proliferation/prolifrn. • GRADE III- Progressive intimal fibrosis + lumen occlusion of smaller PA • GRADE IV- Plexiform lesions in muscular arteries & plexiform capillary channels • GRADE V – Complex plexiform l +angiomatosis & cavernous lesions • GRADE VI- Necrotizing arteritis & fibrinoid necrosis • UPTO GRADE III CHANGES ARE REVERSIBLE Circulation 1958;18:533-547

  15. Haemodynamic stages 1)LOW PULMONARY PRESSURE LEFT TO RIGHT SHUNT INCREASED PULMONARY SATURATION 2) SYSTEMIC PULMONARY PRESSURE SMALL BIDIRECTIONAL SHUNT NO SATURATION CHANGES 3) SUPRASYSTEMIC PULMONARY PRESSURE,RT. TO LT. SHUNT CYANOSIS

  16. CLINICAL CLASSIFICATION OF CONGENITAL SYSTEMIC TO PULMONARY SHUNTS ASSOC. WITH PAH ROBBINS,BAGHETI ET AL .UPDATED CLINICAL CLASSIFICATION OF PULMONARY HYPERTENSION.JACC 2009;54:S43-S54

  17. ANATOMICAL-PATHOPHYSIOLOGICAL CLASSIFICATIONOF CONGENITAL SYSTEMIC-TO-PULMONARY SHUNTS ASSOCIATED WITH PAH (MODIFIED FROM VENICE 2003) Guidelines for the diagnosis and treatment of pulmonary hypertensionEuropean Heart Journal (2009) 30, 2493–2537

  18. TYPES OF PRESENTATION • 1) CHF DURING INFANCY & CYANOSIS LATER ( POSTTRICUSPID SHUNT) AFTER POSTNATAL FALL IN PVR INCREASED PBF( CHF SYMPTOMS BUT NO CYANOSIS) PULMONARY VASCULAR DISEASE SYMPTOMS IMPROVE,MURMUR DECREASE,NO CYANOSIS SUPRASYSTEMIC PULMONARY PRESSURE CAUSING RT. TO LT. SHUNT CYANOSIS, REAPPEARANCE OF MURMUR SYMPTOMS

  19. TYPES OF PRESENTATION • 2)low Level Symptoms During Childhood & PAH In Adulthood • Asymptomatic In Childhood & Dvp Symptoms Like Fatigue Cyanosis In Adulthood • Pretricuspid Shunt • 3) Cyanosis From Beginning • Seen In Complex CCHD • Pulmonary Atresia With Large MAPCA Etc

  20. EISENMENGER SYNDROMEUNDERLYING BASIC LESIONS Type of lesionSomerville ‘98 Daliento et al ‘98 (n=132) (n=188) Ventricular Septal Defect 45 71 Atrial Septal Defect 6 21 Patent ductusarteriosus 12 36 Atrio ventricular septal defect 16 23 Truncusarteriosus 15 11 Single ventricle 13 9 Transposition of great arteries 5 8 Others 20 9

  21. CAUSES & FREQUENCY OF EISENMENGERS SYNDROME ( BASED ON PAULWOOD’S STUDY)

  22. WHY EARLY ES IN POSTTRICUSPID SHUNT THAN ASD? • POST TRICUSPID SHUNT (VSD/PDA) • Pvr never comes down to normal due to high pressure flow from infancy • Regression of medial hypertrophy of smc & rvh does not occur • Dvppah & reversal of shunt at an early age • PRETRICUSPID SHUNTS( ASD) • Direction of shunt is determined by the Right ventricular compliance so no shunt occurs till 3 months • Pvr reaches normal by 3 mths • PAH & ES occurs late in life especially in a large ASD • PAH in ASD believed to be acquired or unrelated to the defect PAULWOOD;DISEASES OF THE HEART & CIRCULATION:3RD EDITION:CHAPTER 8;467- 499

  23. EISENMENGER –AN INDIAN SCENARIO • STUDY DONE FROM 1976-92 IN SCT TVM • 201 PT, Mean age of presentation 19yr • 12 anatomic lesion most common VSD(33.3%),ASD(29.85%),PDA (14.3%) • SCD (30%),CHF(25%)& HAEMOPTYSIS(15%) • 5YR,10YR,15YR SURVIVAL was 86.95%,79.6%&76.9% • Prognostic factors identified were syncope, elevated rt. Sided filling pressures,SpO2 < 85% Prognosis for patients with Eisenmenger syndrome of various aetiologySaha;International journal of cardiology,vol45,issue 3July 1994, Pages 199–207

  24. Eisenmenger Syndrome Natural History • Life expectancy reduced by about 20 years Survival Pattern: At one year 97% At 5 years 87% At 10 years 80% At 15 years 77% At 25 years 42% In IPAH 3YR SURVIVAL < 20 – 30%

  25. ES VS OTHER PAH • Structural changes in the pulmonary vasculature are qualitatively similar in all forms of PAH • Difference in clinical presentation • Cerebral abcess,haemoptysis,arrythmia,CVAetc • Adult patients exhibit survival & a favourable hemodynamic profile and prognosis • cyanosis in early stages • Superior survival seen VS IPAH • RV dysfunction occurs late • Rt to left shunt maintains the cardiac output Model of chronic adaptation: right ventricular function in Eisenmenger syndrome European Heart Journal Supplements (2007) 9 (Supplement H), H54–H60

  26. CLINICAL FEATURES Eisenmenger syndrome Factors relating to deterioration and death L. DalientoET ALEuropean Heart Journal (1998) 19, 1845–1855

  27. CARDIOVASCULAR FINDINGS • Central cyanosis (differential cyanosis in the case of a PDA) • Clubbing • JVP- dominant A-wave/ V wave (TR) • Precordial palpation- right ventricular heave, • palpableP2 /Loud P2 • High-pitched EDM (Graham steell) of PR • Right-sided S4 • Pulmonary ejection click • All shunt murmurs disappear during eisenmenger’s

  28. Other findings • Respiratory - cyanosis and tachypnea. • Hematologic - bruising and bleeding; funduscopic abnormalities related to erythrocytosis include engorged vessels, papilledema, microaneurysms, and blot hemorrhages. • Abdominal - jaundice, right upper quadrant tenderness, and positive Murphy sign (acute cholecystitis). • Vascular - postural hypotension and focal ischaemia (paradoxical embolus). • Musculoskeletal - clubbing, hypertrophic osteoarthropathy • Ocular signs include conjunctival injection, rubeosisiridis, and retinal hyperviscosity change

  29. DIFFERENTIAL DIAGNOSIS OF EISENMENGER SYNDROME

  30. ECG RAE,RVH – ASD ( OS SEC.) Features OF LV Enlargement + RVH – PDA/VSD KALTZ-WACHTEL – equiphasic QRS complexes in mid precordial leads –VSD PAT/Flutter – seen in ASD Left axis deviation -ostiumprimum ASD. RV VOLTAGES ,QRS DURN. & QTc interval are poor prognostic markers

  31. RADIOLOGY • Rt sided aortic arch – 16% of VSD • Rounded shadow overlying aortic knuckle – PDA • Calcification of the duct • Dilatation of MPA-90% • Pulmonary oligaemia • Cardiomegaly PAULWOOD;DISEASES OF THE HEART & CIRCULATION:3RD EDITION:CHAPTER 8;467- 499

  32. RADIOLOGY • “Pulmonary neovascularization” • it is a specific sign for eisenmenger’s • Distinctive vascular lesions on CXR &CT • correlated histologically with collateral vessels seen in posttricuspid communications. Circulation. 2005;112:2778-2785

  33. Eisenmenger SyndromeNoninvasive Evaluation Echocardiography is very useful • Defines the large defect (PDA may be difficult) • Estimates PA pressure by TR/PR jets • Contrast echo demonstrates R L shunting • TEE is safe and may be required in adults for precise delineation of the abnormality

  34. ECHO

  35. ECHO PREDICTORS • A composite score based on the strongest echocardiographic predictors of outcome, including 1 point for each of the following: • TAPSE<15 mm • Ratio of right ventricular effective systolic to diastolic duration> 1.5 • RA area > 25 cm2, • Ratio of RA to left atrial area> 1.5 • This score was strongly related to mortality (odds ratio, 3.69; 95% confidence interval, 2.31–5.91 by bootstrap analysis) Echocardiographic Predictors of Outcome in Eisenmenger Syndrome Pamela Moceri et alCirculation. 2012;126:1461-1468

  36. Eisenmenger Syndrome: Invasive Evaluation • Cardiac cath can be safely performed • It must be done in borderline cases to assess operability • Response of pulmonary vasculature to pulmonary vasodilators like 02, tolazoline and nitric oxide should be assessed • Limit the use of contrast agent to minimal

  37. COMPLICATIONs • HAEMATOLOGY • Chronic hypoxia causes erythrocytosis & secondary polycythemia • Increased iron utilization causes iron deficiancy and microcytes and hypochromia • Increased erythrocytes & increased hematocrit – hyperviscosity • Hyperviscosity along with dilated chambers arrythmia, prothrombotic materials –Thrombosis • Bleeding-thrombocytopenia & decreased coagulation factors • HAEMOPTYSIS • Pulmonary artery thrombosis causing pulmonary infarction

  38. COMPLICATIONs • VASCULAR SYSTEM • Hyperviscosity leads to shear stress causing release of NO – vasodilation & syncope • CORONARY CIRCULATION • Increased NO causes – tortuous & large arteries • Increased demand due to enlarged LV mass & low saturation – increased resting coronary blood flow & decreased coronary reserve • HYPERBILIRUBINEMIA • Increased erythrocytosis causes increased RBC destruction – unconjugatedhyperbilirubinemia & gall stones

  39. COMPLICATIONs • RENAL DYSFUNCTION • Hyperuricemia • Hypoperfusion • Hyperuricemia • decreased renal clearence & increased production of uric acid • CEREBROVASCULAR EVENTS • Stroke or tia – hyperviscosity • Brain abcess • Paradoxical embolism- Rt. to Lt. shunting • HPOA/CLUBBING- • Systemic venous megakaryocytes are shunted into the systemic arterial circulation • PDGF & TGF-beta released promote cell proliferation ,protein synthesis, connective tissue formation & deposition of extracellular matrix • HEART FAILURE

  40. VSD WITH PAH FOLLOW UP ASD WITH PAH FOLLOW UP N1877 Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart diseaseHeart 2007;93:682–687

  41. CAUSES OF DEATH IN ES Eisenmenger syndrome Factors relating to deterioration and death L. DalientoET ALEuropean Heart Journal (1998) 19, 1845–1855

  42. PREDICTORS OF MORTALITY IN ES • NYHA/WHO Functional class • Heart failure- clinical & lab ( impaired LFT) • FEATURES OF right heart filling pressure • Ecg features- • voltage criteria of rvh, qrs duration, qtc • H/o arrythmia • Complex CHD • Creatinine ,uric acid • Pregnancy • Lv Dysfunction • Syncope Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case–control studyEuropean Heart Journal (2006) 27, 1737–1742

  43. Eisenmenger Syndrome Management Strategies Conventional therapy Advanced therapy Surgical therapy

  44. Conventional Therapy • Digitalis, diuretics – heart failure • Anti-arrhythmic drugs • Anticoagulants • Long term oxygen therapy • Avoidance of dehydration, high altitude, infections and IV lines • Avoidance of pregnancy • Moderate and severe strenuous exercise, particularly isometric exercise • I.E PROPHYLAXIS

  45. OXYGEN THERAPY NO DIFF. IN SURVIVAL • Long-term home O2 therapy may improve symptoms • No survival benefit with N.O.T in advanced ES • Recommended in pt. with improvement in saturation & symptoms with O2 ( ESC iia C) Open circle- patients with nocturnal O2 therapy Closed circle – pt in control Nocturnal Oxygen Therapy in Patients with the Eisenmenger Syndrome Am J RespirCrit Care Med Vol 164. pp 1682–1687, 2001

  46. PHLEBOTOMY Indication for Isovolumic Phlebotomy • Symptomatic hyper viscosity (PCV >0.65) ( ESC IIa & Aha class I) • Symptomatic Hb > 20gm%)( AHA CLASS I) Important issues to remember • Symptoms of hyper viscosity resemble those of iron deficiency • Phlebotomy may result in iron deficiency anemia and cerebrovascular accidents • Routine phlebotomies - not recommended( CLASS III AHA ) European Heart Journal (2009) 30, 2493–253

  47. TREATMENTOF ANAEMIA • Oral iron frequently results in a rapid and dramatic increase in red cell mass • Haematological parameters to be monitored regularily • Iron therapy stopped once serum ferritin and/ or transferrin saturation within normal range • Iron intolerant pt. – pulse IV iron therapy Current Cardiology Reviews, 2010, 6, 363-372 7

  48. ANTICOAGULANTSIN ES • Use of oral anticoagulant treatment in Eisenmenger’s syndrome is controversial • A high incidence of PA thrombosis & stroke vs high incidence of bleeding & haemoptysis • In the absence of significant haemoptysis, oral anticoagulant treatment should be considered in patients with PA thrombosis or signs of heart failure( ESC IIA level c) Current Cardiology Reviews, 2010, 6, 363-372 European Heart Journal (2009) 30, 2493–2537

  49. Haemoptysis • General measures • Hospital admission - Reduction of physical activity and suppression of nonproductive cough • Chest x-ray followed by CT thorax • Immediate discontinuation of aspirin, NSAID, anticoagulant • Treatment of hypovolemia and anemia • Specific diagnostic/ therapeutic aspects may be needed, if hemoptysis is severe or incessant: • PLATELET INFUSION in the presence of thrombocytopenia • Administration of FFP, vitamin K or coagulation factors • Angiography with selective embolization of the artery supplying the source of blood loss • Sputum culture and treatment of infectious disease • Risk reduction strategy: • Immediate treatment of respiratory tract infections • Pneumovax and annual fluvaccination

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