Eisenmenger Syndrome

5/20/2018 Eisenmenger Syndrome

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Eisenmenger

Syndrome

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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


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Pathophysiology

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


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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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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


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History

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.


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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 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


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Electrocardiogram

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.


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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 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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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

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, 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

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 haemodynamicsbecause of fixed pulmonary vascular

disease

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

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 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,


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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 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.


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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

cardiotoxicity

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

pregnancy


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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

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. 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,

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 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


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Eisenmenger Syndrome