Eisenmenger's syndrome

Question 1

What is it?

Answer 1

When blood flows from the right side of the heart to the left side across a structural heart lesion, bypassing the lungs.

Question 2

What can cause it?

Answer 2

ASD, VSD, PDA.

Question 3

When does it develop?

Answer 3

Can develop after 1-2 years with large shunts or in adulthood with smaller shunts.
- It can develop more quickly during pregnancy, so women with a history of having a ‘hole in the heart’ need an echo and close monitoring by a cardiologist during pregnancy.

Question 4

What normally happens in a septal defect?

Answer 4

Blood flows from the left side of the heart to the right because the pressure is greater in the left side than the right. This means that blood still travels to the lungs and gets oxygenated; therefore, the patient is acyanotic.

Question 5

What then happens over time and how does the patient become cyanotic?

Answer 5

• overtime, the extra blood flowing into the right side of the heart and the lungs increases the pressure in the pulmonary vessels ⇒ pulmonary hypertension
• when the pulmonary pressure > systemic pressure, blood begins to flow from the right of the heart to the left, across the septal defect
• this is a right to left shunt
• this causes deoxygenated blood to bypass the lungs and enter the body = cyanosis

Question 6

What happens to the body when cyanotic?

Answer 6

• cyanosis related to a low level of oxygen saturation in the blood
• the bone marrow responds to low oxygen sats by producing more RBCs and Hb to increase the oxygen carrying capacity of the blood
• this leads to polycythaemia which is a high concentration of Hb in the blood
• polycythaemia gives the patients a plethoric complexion
• a high concentration of RBCs and Hb makes the blood more viscous, making the patient more prone to to developing blood clots

Question 7

What examination findings are associated with pulmonary hypertension?

Answer 7

• RV heave: RV contracts forcefully against increased pressure in the lungs
• loud P2: loud second heart sound due to forceful shutting of the pulmonary valve
• raised JVP
• peripheral oedema

Question 8

Examination findings relating to the underlying septal defect?

Answer 8

• ASD: mid systolic, crescendo-decrescendo murmur loudest at the upper left sternal border
• VSD: pan systolic murmur loudest at the left lower sternal border
• PDA: continuous crescendo-decrescendo ‘machinery’ murmur
• Arrhythmias

Question 9

Findings related to the right to left shunt and chronic hypoxia?

Answer 9

Cyanosis, clubbing, dyspnoea, plethoric complexion

Question 10

What’s the main cause of death with this syndrome?

Answer 10

Heart failure, infection, thromboembolism, haemorrhage.

Question 11

Management?

Answer 11

• The underlying defect should be managed/corrected surgically to prevent the development of this syndrome
• Once the pulmonary pressure is high enough to cause the syndrome, it’s not possible to medically reverse the condition; the only definitive treatment is a heart-lung transplant.

Question 12

Medical management?

Answer 12

• oxygen to help symptoms
• treatment of pulmonary hypertension eg. sildenafil
• treatment of arrhythmias
• treatment of polycythaemia with venesection
• prevention and treatment of thrombosis with anticoagulation
• prevention of infective endocarditis using prophylactic antibiotics