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Eisenmenger Syndrome

Eisenmenger Syndrome. www.anaesthesia.co.in anaesthesia.co.in@gmail.com.

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Eisenmenger Syndrome

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  1. EisenmengerSyndrome www.anaesthesia.co.in anaesthesia.co.in@gmail.com

  2. In 1897, Eisenmenger reported the case of a 32-year-old man who had showed exercise intolerance, cyanosis, heart failure, and haemoptysis prior to death. Autopsy showed a large ventricular septal defect (VSD) and overriding aorta. This was the first description of a link between a large congenital cardiac shunt defect and the development of pulmonary hypertension

  3. Pathophysiology • Patients with large congenital cardiac or surgically created extracardiac left-to-right shunts increased pulmonary blood flow pulmonary vascular disease pulmonary hypertension • Early stages remains reactive to pulmonary vasodilators • With continued insult becomes fixed & ultimately the level of PVR becomes so high resulting in reversed or bidirectional shunt flow with variable degrees of cyanosis. • Lesions with high shear rate e.g.-large VSD/PDA- pulm. Htn in early childhood • Lesions with low shear rate- pulm. Htn in late middle age • High altitude- early onset

  4. Approximately 50% of infants with a large, nonrestrictive VSD or PDA develop pulmonary hypertension by early childhood. • 40% of patients with VSD or PDA and transposition of the great arteries develop pulmonary hypertension within the first year of life. • Large ASD 10% progress to pulmonary hypertension, slowly and usually not until after the third decade of life. • All patients with persistent truncus arteriosus and unrestricted pulmonary blood flow, and almost all patients with common atrioventricular canal, develop severe pulmonary hypertension by the second year of life. • 10% of those with a Blalock-Taussig anastomosis (subclavian artery to pulmonary artery) develop pulmonary hypertension compared to 30% of those with a Waterston (ascending aorta to pulmonary artery) or a Potts (descending aorta to pulmonary artery) shunt.

  5. Prognosis • Median survival- 80% at 10 yrs after diagnosis & 42% at 25 yrs. Saha etal Int J cardiol. 45:199,1994 • Long-term survival depends on the age at onset of pulmonary hypertension and right ventricular function • Syncope, increased CVP, SPO2 < 85%- poor short term outcome. Vongpatanasin W etal Ann. Intern. Med. 128:745,1998 • Most deaths- sudden cardiac death • Other- heart failure, haemoptysis, thromboembolism, brain abscess & complications of pregnancy and non cardiac surgery

  6. History • Pulmonary hypertension- Breathlessness, Fatigue, Lethargy, Severely reduced exercise tolerance with a prolonged recovery phase, Presyncope, Syncope • Heart failure- Exertional dyspnea, Orthopnea, PND, Edema, Ascites, Anorexia, Nausea • Erythrocytosis- Muscle weakness, Anorexia, Myalgias, Fatigue, Lassitude, Paresthesias of the digits and lips, Tinnitus, Blurred or double vision, Scotomata, Slowed mentation • Bleeding tendency • Palpitations- often due to AF/flutter • Haemoptysis- pulmonary infarction, rupture of pulmonary vessels or aortopulmonary collateral vessels

  7. Cardiovascular findings • Central cyanosis (differential cyanosis in the case of a PDA) • Clubbing • JVP- dominant A-wave • central venous pressure may be elevated. • Precordial palpation- right ventricular heave, palpable S2. • Loud P2 • High-pitched early diastolic (Graham steell) murmur of pulmonic insufficiency • Right-sided fourth heart sound • Pulmonary ejection click • The continuous murmur of a PDA disappears when Eisenmenger physiology develops; a short systolic murmur may remain audible.

  8. 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, rubeosis iridis, and retinal hyperviscosity change

  9. Lab investigations • Complete blood count • Erythrocytosis increases hematocrit and hemoglobin concentration. • Phlebotomy-related iron deficiency decreases the mean corpuscular volume and mean corpuscular hemoglobin concentration. • Red cell mass is increased with erythrocytosis. • Bleeding time is prolonged by platelet dysfunction, VWF dysfunction • Biochemical profile • Increased conjugated bilirubin • Increased uric acid • Urea and creatinine sometimes elevated • Erythrocytic hypoglycemia is an artifactually low blood glucose level caused by increased in vitro glycolysis in the setting of increased red cell mass. • Iron studies • Reduced serum ferritin due to phlebotomy-related iron store reduction • Increased total iron binding capacity • Urine biochemical analysis reveals proteinuria. • Arterial blood gases • Reduced resting PaCO2 due to resting tachypnea and reduced PaO2 due to right-to-left shunting • Mixed respiratory and metabolic acidosis

  10. Chest radiograph • Right ventricular and right atrial enlargement • Features of pulmonary hypertension - dilated main pulmonary artery, increased hilar vascular markings, and pruned peripheral vessels

  11. Electrocardiogram • Almost always abnormal results and includes signs of right heart hypertrophy in addition to abnormalities associated with the underlying defect • Tall R wave in V1, deep S wave in V6, ± ST and T wave abnormalities • P pulmonale • Atrial and ventricular arrhythmias • Incomplete right bundle branch block is present in 95% of ASDs. • Vertical frontal plane QRS axis usually is present with ostium secundum ASD. • Left axis deviation commonly is present with ostium primum ASD.

  12. Echocardiogram • Transthoracic echocardiogram • The structural cardiac defect responsible for the shunt can be defined by the 2-dimensional imaging. • The location of cardiac shunt can be demonstrated by color Doppler or venous agitated saline contrast imaging. • The pressure gradient across the defect can be estimated. • Estimated pulmonary artery systolic and diastolic pressures • Identification of coexistent structural abnormalities • Left and right ventricular size and function • Identification of surgical systemic-to-pulmonary shunts • The addition of supine bicycle ergometry can demonstrate increased right-to-left shunting with exercise. • Transesophageal echocardiogram is useful for imaging posterior structures, including the atria and pulmonary veins.

  13. Apical 4-chamber transthoracic view demonstrating anostium ASD with enlarged right-side chambers.

  14. Cardiac catheterization • Severity of pulmonary vascular hypertension • Conduit patency and pressure gradient • Coexisting coronary artery anomalies (rare) • Degree of shunting

  15. Medical Treatment Fluid balance and climate control • Avoid sudden fluid shifts or dehydration, which may increase right-to-left shunting. • Avoid very hot or humid conditions, which may exacerbate vasodilatation, causing syncope and increased right-to-left shunting. Oxygen supplementation • Use is controversial • Oxygen therapy has been shown to have no impact on exercise capacity and survival in adult patients with Eisenmenger syndrome Sandoval etalAm J Respir Crit Care Med. 2001 Nov 1;164(9):1682-7 • Continuous home oxygen therapy better than nocturnal supplementation • Better results in children and at early stages. Bowyer etal Br Heart J. 1986 Apr;55(4):385-90 • Most useful as a bridge to heart-lung transplantation.

  16. Medical Treatment • Erythrocytosis - rule out dehydration. Then, if symptoms of hyper viscosity and the haematocrit is greater than 65%, venesect 250-500 mL of blood and replace with an equivalent volume of isotonic sodium chloride (or 5% dextrose if in heart failure). • For resuscitation in the event of massive acute bleeding, replace losses with FFP, cryoprecipitate, and platelets. • Infective endocarditis prophylaxis • Encourage good oral hygiene • Anticoagulation- increased risk of bleeding, hence not routinely used. Silversides et al J Am Coll Cardiol 2003 Dec 3; 42(11): 1982-7 • Digoxin, diuretics for right heart failure

  17. Medical Treatment Pulmonary vasodilator therapy • Long-term prostacyclin therapy- Improvement in haemodynamics, suturation & 6 minute walk test. Rosenzweig etal, Circulation 1999 Apr 13; 99(14): 1858-65 Fernandes etal Am J Cardiol 2003 Mar 1; 91(5): 632-5 • Bosentan, an endothelin receptor antagonist Christensen,Am J Cardiol 2004 Jul 15; 94(2): 261-3 Schulze-Neick et al Am Heart J 2005 Oct; 150(4): 716 • Treatment with prostacyclin analogues and/ or endothelin receptor antagonists delayed the need for transplantation. Adriaenssens, Eur Heart J 2006 Jun; 27(12): 1472-7 • Sildenafil- Singh TP etal Am Heart J 2006 Apr; 151(4): 851 Pregnancy • To be avoided • Therapeutic abortion in first trimester

  18. Surgical options Heart lung transplant • Procedure of choice if repair of the underlying cardiac defect is not possible. • Performed successfully for the first time in 1981. • Reported actuarial survival rates are 68% at 1 year, 43% at 5 years, and 23% at 10 years. • The main complications are infection, rejection, and obliterative bronchiolitis Bilateral lung transplantation • Preferable procedure if the cardiac defect is simple (e.g.- ASD) • Repair of the underlying cardiac defect is required • Better than single-lung transplantation in terms of mortality, New York Heart Association functional class, cardiac output, and postoperative pulmonary edema. • Advantages over heart-lung grafting include no transplant coronary artery disease or cardiac rejection.

  19. Corrective surgery options • Repair of the primary defect is contraindicated in the context of established severe pulmonary hypertension. • Corrective surgery may be possible if a significant degree of left to- right shunting remains and if responsiveness of the pulmonary circulation to vasodilator therapy can be demonstrated. • Limitation - transient dynamic right ventricular outflow tract obstruction.

  20. Activity Intense athletic activities carry the risk of sudden death. Exercise prescription can be individualized based on exercise testing that documents a level of activity that meets the following 3 criteria: Oxygen saturations remain greater than 80%. No symptomatic arrhythmias. No evidence of symptomatic ventricular dysfunction Diet Excessive sodium intake to be avoided

  21. Anaesthetic considerations…

  22. Eisenmenger pts pose a difficult challenge as they have lost the ability to adapt to sudden changes in haemodynamics because of fixed pulmonary vascular disease Colon-Otero G etal Mayo Clin Proc 1987;62:379–85.

  23. Preoperative assessment • Assessment of medical condition • Assessment of anotomical defect and physiology • Non-cardiac/ cardiac surgery/ pregnancy for labour analgesia

  24. Goals • Prevent further increase in Rt to Lt shunt • Maintain CO • Prevent arrhythmias • Avoid hypovolemia, PVR, SVR • Marked increase in SVR should also be avoided as excessive systemic vasoconstriction can precipitate acute LVH

  25. What To Do? • Prevention of prolonged fasting & dehydration • Sedation to reduce preop anxiety and oxygen consumption • Keep phenylephrine/ Norepinephrine infusion, anticholinergic, antiarrythmics ready • Monitoring- Pulse oximetry, ECG, ETCO2, Arterial catheter for IBP monitoring and serial ABG monitoring, CVP, AWP. (PAC- better to avoid) • TOE- to know status of the shunt, to guide fluid therapy by looking at ventricular function, to measure pulmonary artery pressure. Bouch DC, Anaesthesia. 2006 Oct;61(10):996-1000 • Avoid factors known to increase PVR viz. cold, hypercarbia, acidosis, hypoxia,

  26. Air Bubble precautions • To prevent paradoxical air embolism • Remove all bubbles from iv tubing • Connect the iv tubing to the venous cannula while there is free flowing in fluid . • Eject small amount of solution from syringe to clear air from the needle hub before iv injection • Aspirate injection port before injection to clear any air • Hold the syringe upright to keep bubbles at the plunger end • Do not leave a central line open to air • Use air filters • ? No N2O.

  27. Which anaesthetic technique to use? • Regional blocks - low mortality (5% vs 18% for G.A.).Mortality more dependent on the surgical procedure rather tan anaesthetic technique. Martin JT et al, Reg Anesth Pain Med. 2002 Sep-Oct;27(5):509-13. • General anaesthesia • Induction with high dose opioid (short acting) technique or with ketamine, etomidate or low dose thiopentone • Cardiostable inhalational agent- isoflurane, sevoflurane, xenon. Hofland J Br J Anaesth. 2001 Jun;86(6):882-6. • Muscle relaxation with atracurium, vecuronium • TIVA with propofol, remifentanil. Kopka A, Acta Anaesthesiol Scand. 2004 Jul;48(6):782-6 • Some patient may not tolerate positive pressure ventilation and PEEP well

  28. Anaesthetic technique • Single shot SAB contraindicated – rapid drop in SVR • Low-dose bupivacaine-fentanyl spinal anesthesia has been successfully used for lower extremity surgery in a nonparturient with Eisenmenger's syndrome Chen CW et al, J Formos Med Assoc. 2007 Mar;106(3 Suppl):S50-3 • Graded epidural can be safely used • Ropivacaine, Levobupivacaine theoretically better- less cardiotoxicity • Continuos spinal anaesthesia with slow increments of doses titrated against the haemodynamic and anaesthetic effects. Cole PJ, Br J Anaesth. 2001 May;86(5):723-6.

  29. Pulmonary vasodilator therapy intraop. • 100 % oxygen • Nitric oxide- 5 -20 ppm. Bouch DC etal, Anaesthesia. 2006 Oct;61(10):996-1000 • Prostacycline- infusion or nebulization

  30. Postoperative care • Observation on a monitored bed in ICU/HDU for 24 hours or overnight atleast because of their predisposition to develop ventricular/ supraventricular tachycardia, bradyarrhythmia and myocardial ischemia • Meticulous attention to fluid balance to prevent hypovolumia • Monitoring of blood pressure preferably invasive, Oxygen saturation and CVP • Position slowly- risk of postoperative postural hypotension with secondary increase in right to left shunting • Prevention of venous stasis by early ambulation and by applying effective elastic stocking or periodic pneumatic compression. • Adequate pain management – adverse hemodynamics and possibly hypercoagulable state

  31. Eisenmenger and pregnancy

  32. Pts with Eisenmenger do not tolerate pregnancy well because… • Decreased SVR during pregnancy • Decreased FRC & increased oxygen consumption – exacerbate maternal hypoxemia – decreased O2 delivery to fetus – IUGR & fetal demise

  33. Risks related with pregnancy • Spontaneous abortions- 20- 30% • Premature delivery- 50% • IUGR- 50% of born.Avila WS: Eur. Heart J. 16:460,1995 • Maternal death- 30-45% intrapartum or first post partum weak • Successful first pregnancy doesn’t preclude maternal death during subsequent pregnancy Gleicher N: Obstet Gynecol Surg 34:721, 1979 • Factors influencing mortality- thromboembolism, hypovolumia, preeclampsia • Mortality is similar with ceasarean section or vaginal delivery • Mortality reaches to 80% in presence of preeclampsia

  34. In O.T. • General measures- preparation and monitoring same as described before+ left uterine displacement, anti aspiration prophylaxis, preparation for neonatal resuscitation • If vaginal delivery planned- give labour analgesia • CSE technique preferred- Intrathecal fentanyl/ sufentanil + very low dose L.A. in first stage of labour, then small, incremental dose of L.A. • Use of continuous spinal anaesthesia and postop analgesia also reported. Sakuraba s, J Anesth. 2004;18(4):300-3. • G.A • Post op monitoring

  35. Thank you!! www.anaesthesia.co.in anaesthesia.co.in@gmail.com

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