Eisenmenger Syndrome

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EisenmengerSyndrome
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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
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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

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• 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.

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Prognosis

• Median survival- 80 at 10 yrs after diagnosis
  42 at 25 yrs. Saha etal Int J cardiol.
  45199,1994
• Long-term survival depends on the age at onset of
  pulmonary hypertension and right ventricular
  function
• Syncope, increased CVP, SPO2 lt 85- poor short
  term outcome. Vongpatanasin W etal Ann. Intern.
  Med. 128745,1998
• Most deaths- sudden cardiac death
• Other- heart failure, haemoptysis,
  thromboembolism, brain abscess complications of
  pregnancy and non cardiac surgery

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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

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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 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.

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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

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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

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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

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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.

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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.

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Apical 4-chamber transthoracic view demonstrating
anostium 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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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 etal
  Am J Respir Crit Care Med. 2001 Nov
  1164(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 Apr55(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, 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

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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

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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.

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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.

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• 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

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• Anaesthetic considerations

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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 19876237985.
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Preoperative assessment

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

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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

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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 Oct61(10)996-1000
• Avoid factors known to increase PVR viz. cold,
  hypercarbia, acidosis, hypoxia,

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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.

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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-Oct27(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
  Jun86(6)882-6.
• Muscle relaxation with atracurium, vecuronium
• TIVA with propofol, remifentanil. Kopka A, Acta
  Anaesthesiol Scand. 2004 Jul48(6)782-6
• Some patient may not tolerate positive pressure
  ventilation and PEEP well

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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
  Mar106(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
  May86(5)723-6.

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Pulmonary vasodilator therapy intraop.

• 100 oxygen
• Nitric oxide- 5 -20 ppm. Bouch DC etal,
  Anaesthesia. 2006 Oct61(10)996-1000
• Prostacycline- infusion or nebulization

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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

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• Eisenmenger and pregnancy

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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

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Risks related with pregnancy

• Spontaneous abortions- 20- 30
• Premature delivery- 50
• IUGR- 50 of born. Avila WS Eur. Heart J.
  16460,1995
• Maternal death- 30-45 intrapartum or first post
  partum weak
• Successful first pregnancy doesnt preclude
  maternal death during subsequent pregnancy
  Gleicher N Obstet Gynecol Surg 34721, 1979
• Factors influencing mortality- thromboembolism,
  hypovolumia, preeclampsia
• 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
  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.
  200418(4)300-3.
• G.A
• Post op monitoring

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Thank you!!
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