Eisenmenger Syndrome

Question 1

What is Eisenmenger Syndrome?

Answer 1

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

Question 2

What are the 3 underlying lesions that can result in Eisenmenger syndrome?

Answer 2

• Atrial Septal defect
• Ventricular septal defect
• Patent ductus arteriosus

Question 3

When can Eisenmenger Syndrome develop?

Answer 3

• Develop 1-2 years with large shunts
• In adulthood with small shunts
• 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 is the Pathophysiology of Eisenmenger Syndrome?

Answer 4

• When there is a septal defect blood will flow from the left side of the heart to the right
• This leads to Pulmonary hypertension
• The pulmonary pressure exceeds the systemic pressure and blood begins to flow from the right side to the left side of the heart
• This causes blood to bypass the lungs and deoxygenated blood to enter the body
• This causes cyanosis - low level of oxygen saturations in the blood
• The bone marrow responds to low sats by producing more RBC and haemoglobin to increase oxygen carrying capacity of the blood leading to polycythaemia = a high concentration of haemoglobin in the blood

Question 5

What are the examination findings for Pulmonary Hypertension?

Answer 5

Pulmonary Hypertension:
- Right Ventricular Heave = Right ventricle contracts forcefully against increased pressure in the lungs
- Loud S2 = loud second heart sound due to forceful shutting of the pulmonary valve
- Raised JVP
- Peripheral oedema

Question 6

What are Examination findings related to septal defect?

Answer 6

• Atrial Septal Defect: mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border
• Ventricular Septal Defect: pan-systolic murmur loudest at the left lower sternal border
• Patent ductus arteriosus: continuous crescendo-decrescendo machinery murmur
• Arrhythmias

Question 7

What are Examination findings related to the right to left shunt and chronic hypoxia?

Answer 7

• Cyanosis
• Clubbing
• Dyspnoea
• Plethoric Complexion - red complexion related to polycythaemia

Question 8

What is the Managment for Eisenmenger Syndrome?

Answer 8

• Ideally the underlying defect should be managed optimally/ corrected surgically to prevent the development of Eisenmenger Syndrome
• Once the Pulmonary pressure is high enough to cause the syndrome, it is not possible to medically reverse the condition.
• Only definitive treatment would be a heart-lung transplant
• Closely followed up with:
• Oxygen
• Pulmonary hypertension treatment = sildenafil
• Treatment of arrhythmias
• Treatment of Polycythaemia = venesection
• Prevention and treatment of thrombosis with anticoagulation
• Prevention of IE with prophylactic abx