9-Eisenmenger- final lecture presentation.ppt

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

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

 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.

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

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

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.

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

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

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

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.

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.

Apical 4-chamber transthoracic view demonstrating an
ostium ASD with enlarged right-side chambers.

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

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 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.

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

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

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.

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.

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

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.

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

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

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,

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.

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

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.

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

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

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

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

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

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