Eisenmenger’s syndrome - Questions & Answers For A Long Case

Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis. The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease.

What are your differential diagnoses?

 As follows:

• Congestive cardiac failure
• Chronic cor pulmonale.

Q. What investigations would you suggest ?

 As follows:

1. X-ray chest (enlargement of central pulmonary arteries with peripheral pruning of pulmonary vessels).

2. ECG (RVH, RAH, right axis deviation).

3. Echocardiography.

Q. What are the clinical features of Eisenmenger’s syndrome?

As follows:

• Dyspnea
• Fatigue
• Syncope
• Angina
• Hemoptysis
• Features of CCF.

On examination:

• Central cyanosis (not corrected by giving 100% oxygen. Differential cyanosis (cyanosis in toes, not in the hand) occurs in PDA.
• Clubbing (differential clubbing—clubbing in toes, not in the hand, occurs in PDA).
• Pulse—low volume.
• Prominent “a” wave in JVP.
• Other signs of pulmonary hypertension – palpable P2, left parasternal lift, epigastric pulsation due to RVH. Ejection click and ejection systolic murmur may be present.
• Tricuspic regurgitation may occur (in such case, prominent V wave in JVP, also there may be a pansystolic murmur in left lower parasternal area).
• Polycythemia.
• Original murmur of VSD, ASD or PDA—decrease in intensity, even may disappear.

Q. What is the treatment?

As follows:

• Diuretic.
• Digoxin may be given in some cases.
• Venesection may be required, especially if there is polycythemia.
• Heart lung transplantation may be done (mortality rate is very high than heart transplantation alone).
• Surgery of shunt is contraindicated in Eisenmenger’s syndrome, as it aggravates right sided heart failure.

Q. What are the causes of death in Eisenmenger’s syndrome?

As follows:

• Right heart failure
• Infective endocarditis
• Pulmonary infarction
• Cerebral thrombosis or abscess
• Arrhythmias.

Remember the following points:

• If a patient has cyanosis with evidences of pulmonary hypertension, the more likely diagnosis is Eisenmenger’s syndrome.
• If a patient with VSD develops Eisenmenger’s syndrome, there is appearance of cyanosis, clubbing and evidences of pulmonary hypertension. However, pansystolic murmur of VSD may disappear, because of equalization of pressure between right and left ventricles.
• Patient with Eisenmenger’s syndrome is at particular risk from abrupt changes in afterload that exacerbate right to left shunting (e.g. during anesthesia and also in pregnancy).