4.3 Eisenmenger's Syndrome

Question 1

What is the association of VSD with ‘blue spells’?

Answer 1

VSD is an acyanotic heart disease,

but in the presence of increased right heart pressures

causes a right-to-left shunt leading to cyanotic spells.

This is called Eisenmenger’s syndrome.

Question 2

What is eisenmenger’s syndrome?

Answer 2

Eisenmenger’s syndrome

(after German physician Dr Viktor Eisenmenger, 1897)

is an untreated congenital heart defect with intracardiac
communication that

causes
pulmonary hypertension,
reversal of flow,
cyanosis.

Question 3

What are the causes of Eisenmenger’s Syndrome

Answer 3

The initial cardiac defects could be VSD, PDA, or less commonly, ASD.

Other causes include AV septal defect,
double-outlet right ventricle,
tetralogy of Fallot,
transposition of great vessels,
and truncus arteriosus.

(Eisenmenger’s syndrome secondary to VSD
is called Eisenmenger’s Complex.)

Question 4

What are cyanotic heart diseases?

Answer 4

Cyanotic heart diseases are congenital cardiac defects
where deoxygenated
blood is shunted to systemic circulation.

Question 5

How is eisenmenger’s syndrome different from Cyanotic heart diseases?

Answer 5

Eisenmenger’s syndrome causes cyanosis at a later age.

The congenital cardiac defect is not cyanotic,
but secondary to the development of
pulmonary hypertension the previous

left-to-right shunt is converted to a
right-to-left shunt.

Question 6

Examples of cyanotic heart diseases

Answer 6

1. • Tetralogy of Fallot
2. • Total anomalous pulmonary venous connection
3. • Hypoplastic left heart syndrome
4. • Transposition of great vessels
5. • Truncus arteriosus
6. • Tricuspid atresia

Question 7

What is the pathophysiology of eisenmenger’s syndrome?

Answer 7

Systemic to pulmonary connection

↓

Left-to-right shunting

↓

Increased pulmonary flow

↓

Irreversible pulmonary vascular injury

↓

Increased pulmonary vascular resistance

↓

Right-to-left shunting

↓

Hypoxia and erythrocytosis

Question 8

What is the pathophysiology of eisenmenger’s syndrome?

Answer 8

1. Initially the communication between

right and left sides of chambers

2. allows blood flow from left to right,

as SVR (1000 dynes.sec/cm5)

is much higher than PVR (150 dynes.sec/cm5).

3. Increased blood flow in the right heart
   increases blood flow through
   the pulmonary artery and

produces shear forces in pulmonary microvasculature.

4. This with volume overload causes increase in PVR.

Gradually PVR becomes equal or
higher than SVR over years.

5. Thus the shunt becomes bidirectional, and later,

when reversal occurs, deoxygenated blood flows

from right to left causing cyanosis and chronic hypoxaemia.

6. This stage with right-to-left shunt and cyanosis is
   termed Eisenmenger’s syndrome.

Question 9

What are the signs and symptoms?

Answer 9

Eisenmenger’s syndrome is an insidious disease process.

In patients with left-to-right shunt,
only 11% develop reversal of shunt
and Eisenmenger’s syndrome.

Main symptoms include
dyspnoea on exertion,
palpitations,
syncope,
fatigue,
angina, and
haemoptysis.

Important signs are
cyanosis,
clubbing of fingers,
dysrhythmias on ECG,
polycythaemia,
signs of congestive cardiac failure,
hyperviscosity,
and endocarditis.

It also causes cholelithiasis,
renal dysfunction,
gout,
haematological abnormalities.

The quality of life is poor, and exercise tolerance is limited.

Question 10

What are the implications of anaesthetising this patient?

Answer 10

The theoretical risks of anaesthesia in these patients are considerable.

The cornerstone of safe anaesthesia in such patients

is

1. maintenance of preoperative levels of systemic vascular resistance
2. to reduce the amount of right-to-left shunt during the perioperative period.

Question 11

PVR + SVR goals

Answer 11

Avoid any increase in PVR:
By reducing anxiety with premedication,
good analgesia,
avoiding acidosis,
hypoxia,
hypercarbia.

Avoid any reduction in SVR:
Titration of induction and inhalational agents,
avoiding regional techniques,
and use of alpha agonists to maintain SVR.

Question 12

In general for patients with shunt avoid

Answer 12

• Increase in PVR
• Decrease in SVR
• Air in IV line
• Hypoxia and hypercarbia

Question 13

Specifics of GA shunt

Answer 13

1. • Careful premedication with a benzodiazepine may be useful.
2. • For IV access, it is crucial to avoid any small air bubbles
     entering the circulation as this can cross to the arterial side
     and cause stroke and ischaemia to vital organs
     (paradoxical air embolus).
3. • General anaesthesia is better tolerated than spinal anaesthesia.
     Ketamine maintains SVR,
     while the other agents reduce it.

Inhalational agents also reduce SVR,
but their dose-dependent reduction in PVR might be useful.

4. • Controlled ventilation is recommended.
     Hyperoxemia and low CO2 reduce PVR and shunt.
5. • Adequate analgesia is vital as pain
     causes increase in PVR, SVR, and
     cardiac oxygen requirements.
6. • Invasive monitoring is recommended and
     postoperative HDU or ITU care might be needed.

Question 14

Will you consider regional anaesthesia in this case?

ADvantages

Answer 14

Advantages of regional technique

• Avoidance of cardiac effects of anaesthetic agents
• Avoidance of the need for airway management
  (possibly difficult in Down’s syndrome)
• Good postoperative analgesia

Question 15

Disadvantages RA

Answer 15

• Significant and uncontrolled drop in SVR
  caused by spinal can be detrimental.
• Also in a patient with learning difficulties,
  an awake procedure can be challenging and
  sedation can cause Co2 retention and worsening of
  right-to-left shunt.
• Epidural and incremental spinal (with spinal catheter) with invasive
  monitoring and use of fluids and vasopressors to avoid fall in SVR have
  been described in the literature.