eisenmenger syndrome

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E. Syndrome 2


  • 5/20/2018 Eisenmenger Syndrome





    [email protected]

    http://www.anaesthesia.co.in/mailto:[email protected]:[email protected]://www.anaesthesia.co.in/
  • 5/20/2018 Eisenmenger Syndrome


    In 1897, Eisenmenger reported the case ofa 32-year-old man who had showedexercise intolerance, cyanosis, heart

    failure, and haemoptysis prior to death.Autopsy showed a large ventricular septaldefect (VSD) and overriding aorta. Thiswas the first description of a link between

    a large congenital cardiac shunt defect andthe development of pulmonary hypertension

  • 5/20/2018 Eisenmenger Syndrome


  • 5/20/2018 Eisenmenger Syndrome



    Patients with large congenital cardiac or surgically createdextracardiac left-to-right shunts increased pulmonary bloodflow pulmonary vascular disease pulmonary hypertension

    Early stages remains reactive to pulmonary vasodilators

    With continued insult becomes fixed & ultimately the level of PVRbecomes so high resulting in reversed or bidirectional shunt flowwith variable degrees of cyanosis.

    Lesions with high shear rate e.g.-large VSD/PDA- pulm. Htn inearly childhood

    Lesions with low shear rate- pulm. Htn in late middle age High altitude- early onset

  • 5/20/2018 Eisenmenger Syndrome


  • 5/20/2018 Eisenmenger Syndrome


    Approximately 50% of infants with a large, nonrestrictive VSD or PDAdevelop pulmonary hypertension by early childhood.

    40% of patients with VSD or PDA and transposition of the greatarteries develop pulmonary hypertension within the first year of life.

    Large ASD 10% progress to pulmonary hypertension, slowly andusually not until after the third decade of life.

    All patients with persistent truncus arteriosus and unrestrictedpulmonary blood flow, and almost all patients with commonatrioventricular canal, develop severe pulmonary hypertension by thesecond year of life.

    10% of those with a Blalock-Taussig anastomosis (subclavian arteryto pulmonary artery) develop pulmonary hypertension compared to30% of those with a Waterston (ascending aorta to pulmonary artery)or a Potts (descending aorta to pulmonary artery) shunt.

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    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

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  • 5/20/2018 Eisenmenger Syndrome



    Pulmonary hypertension- Breathlessness, Fatigue,Lethargy, Severely reduced exercise tolerance witha prolonged recovery phase, Presyncope, Syncope

    Heart failure- Exertional dyspnea, Orthopnea, PND,Edema, Ascites, Anorexia, Nausea

    Erythrocytosis- Muscle weakness, Anorexia,Myalgias, Fatigue, Lassitude, Paresthesias of thedigits and lips, Tinnitus, Blurred or double vision,Scotomata, Slowed mentation

    Bleeding tendency

    Palpitations- often due to AF/flutter

    Haemoptysis- pulmonary infarction, rupture ofpulmonary vessels or aortopulmonary collateralvessels

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    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 ofpulmonic insufficiency

    Right-sided fourth heart sound

    Pulmonary ejection click The continuous murmur of a PDA disappears when

    Eisenmenger physiology develops; a short systolic murmurmay remain audible.

  • 5/20/2018 Eisenmenger Syndrome


    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

  • 5/20/2018 Eisenmenger Syndrome


    Lab investigations

    Complete blood count Erythrocytosis increases hematocrit and hemoglobin concentration.

    Phlebotomy-related iron deficiency decreases the mean corpuscular volume andmean 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 byincreased 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

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    Chest radiograph

    Right ventricular and right atrial enlargement

    Features of pulmonary hypertension - dilated main pulmonary

    artery, increased hilar vascular markings, and pruned

    peripheral vessels

  • 5/20/2018 Eisenmenger Syndrome


  • 5/20/2018 Eisenmenger Syndrome



    Almost always abnormal results and includes signs ofright heart hypertrophy in addition to abnormalitiesassociated with the underlying defect

    Tall R wave in V1, deep S wave in V6, ST and T waveabnormalities

    P pulmonale

    Atrial and ventricular arrhythmias

    Incomplete right bundle branch block is present in 95% ofASDs.

    Vertical frontal plane QRS axis usually is present withostium secundum ASD.

    Left axis deviation commonly is present with ostiumprimum ASD.

  • 5/20/2018 Eisenmenger Syndrome


  • 5/20/2018 Eisenmenger Syndrome



    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 colorDoppler 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 demonstrateincreased right-to-left shunting with exercise.

    Transesophageal echocardiogram is useful for imaging posteriorstructures, including the atria and pulmonary veins.

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    Apical 4-chamber transthoracic view demonstrating an

    ostium ASD with enlarged right-side chambers.

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    Cardiac catheterization

    Severity of pulmonary vascular hypertension

    Conduit patency and pressure gradient Coexisting coronary artery anomalies (rare)

    Degree of shunting

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  • 5/20/2018 Eisenmenger Syndrome


    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 exacerbatevasodilatation, causing syncope and increased right-to-left shunting.

    Oxygen supplementation

    Use is controversial

    Oxygen therapy has been shown to have no impact on exercisecapacity and survival in adult patients with Eisenmenger syndromeSandoval etalAm J Respir Crit Care Med. 2001 Nov 1;164(9):1682-7

    Continuous home oxygen therapy better than nocturnalsupplementation

    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.

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    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, replacelosses with FFP, cryoprecipitate, and platelets.

    Infective endocarditis prophylaxis

    Encourage good oral hygiene

    Anticoagulation- increased risk of bleeding, hence not routinelyused. Silversides et al J Am Coll Cardiol 2003 Dec 3; 42(11): 1982-7

    Digoxin, diuretics for right heart failure

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    Medical TreatmentPulmonary vasodilator therapy

    Long-term prostacyclin therapy- Improvement in haemodynamics,

    suturation & 6 minute walk test. Rosenzweig etal, Circulation 1999 Apr 13; 99(14): 1858-65Fernandes 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): 261-3Schulze-Neick et al Am Heart J 2005 Oct; 150(4): 716

    Treatment with prostacyclin analogues and/ or endothelin receptorantagonists 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


    To be avoided Therapeutic abortion in first trimester

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    Surgical options

    Heart lung transplant Procedure of choice if repair of the underlying cardiac defect is not


    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


    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, andpostoperative pulmonary edema.

    Advantages over heart-lung grafting include no transplant coronary

    artery disease or cardiac rejection.

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    Corrective surgery options

    Repair of the primary defect is contraindicatedin the context of established severe pulmonaryhypertension.

    Corrective surgery may be possible if asignificant degree of left to- right shuntingremains and if responsiveness of thepulmonary circulation to vasodilator therapy

    can be demonstrated. Limitation - transient dynamic right ventricular

    outflow tract obstruction.

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    Activity Intense athletic activities carry the risk of sudden death.

    Exercise prescription can be individualized based onexercise 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


    Excessive sodium intake to be avoided

  • 5/20/2018 Eisenmenger Syndrome




  • 5/20/2018 Eisenmenger Syndrome


    Eisenmenger pts pose a difficult challenge

    as they have lost the ability to adapt to

    sudden changes in haemodynamicsbecause of fixed pulmonary vascular


    Colon-Otero G etalMayo Clin Proc 1987;62:37985.

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    Preoperative assessment

    Assessment of medical condition

    Assessment of anotomical defect and physiology

    Non-cardiac/ cardiac surgery/ pregnancy for labour


  • 5/20/2018 Eisenmenger Syndrome



    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

  • 5/20/2018 Eisenmenger Syndrome


    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 catheterfor 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 arterypressure. Bouch DC, Anaesthesia. 2006 Oct;61(10):996-1000

    Avoid factors known to increase PVR viz. cold, hypercarbia,

    acidosis, hypoxia,

  • 5/20/2018 Eisenmenger Syndrome


    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 freeflowing in fluid .

    Eject small amount of solution from syringe to clear air from theneedle 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.

  • 5/20/2018 Eisenmenger Syndrome


    Which anaesthetic technique to use?

    Regional blocks - low mortality (5% vs 18% forG.A.).Mortality more dependent on the surgical procedurerather 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 orwith 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 ventilationand PEEP well

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    Anaesthetic technique

    Single shot SAB contraindicatedrapid 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


    Continuos spinal anaesthesia with slow increments of doses titrated

    against the haemodynamic and anaesthetic effects. Cole PJ, Br J Anaesth. 2001May;86(5):723-6.

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    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

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    Postoperative care

    Observation on a monitored bed in ICU/HDU for 24 hours orovernight atleast because of their predisposition to developventricular/ supraventricular tachycardia, bradyarrhythmia andmyocardial ischemia

    Meticulous attention to fluid balance to prevent hypovolumia

    Monitoring of blood pressure preferably invasive, Oxygen saturationand CVP

    Position slowly- risk of postoperative postural hypotension withsecondary 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 managementadverse hemodynamics and possibly

    hypercoagulable state

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    Eisenmenger and


  • 5/20/2018 Eisenmenger Syndrome


    Pts with Eisenmenger do not tolerate

    pregnancy well because

    Decreased SVR during pregnancy

    Decreased FRC & increased oxygen

    consumptionexacerbate maternal hypoxemiadecreased O2 delivery to fetusIUGR & fetal


  • 5/20/2018 Eisenmenger Syndrome


    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 doesnt preclude maternal death duringsubsequent pregnancy Gleicher N: Obstet Gynecol Surg 34:721, 1979

    Factors influencing mortality- thromboembolism, hypovolumia,


    Mortality is similar with ceasarean section or vaginal delivery Mortality reaches to 80% in presence of preeclampsia

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    In O.T.

    General measures- preparation and monitoring same asdescribed 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

  • 5/20/2018 Eisenmenger Syndrome



    [email protected]

    http://www.anaesthesia.co.in/mailto:[email protected]:[email protected]://www.anaesthesia.co.in/