Congenital Heart Disease

Question 1

What are adult cyanotic congenital heart diseases?

Answer 1

It is a group of cyanotic congenital heart defects that have persisted in to adulthood as they have not been repaired or just treated with temporary palliative surgeries (Fallon’s). Examples include:

• Tetralogy of Fallot
• Transposition complexes
• Double outlet right ventricle
• Treated hypoplastic left ventricle

Or undetected septal defects with Eisenmenger physiology.

Also known as GUCHD (Grown up congenital heart disease and is categorised based on complexity)

Question 2

What are the major complications which can occur in adult cyanotic congenital heart defects?

Answer 2

-Arrhythmias:
A common complication of ACHD. The loss of sinus rhythm may lead to significant haemodynamic decompensation and also stasis and thromboembolism. Arrhythmia’s are more common in patients with dilated right atrium or ventricles.

• Sudden cardiac death
• Heart Failure
• Increased risk of infective endocarditis

-Bleeding and thromboembolic events:
Tend to have a bleeding risk due to platelet counts usually being in the low normal range and their function is often abnormal.
Have an increased thromboembolic risk particularly in patients with cyanotic lesions as they develop a compensatory erythrocytosis which predisposes to clotting.

-Pregnancy
Increased risk of maternal death (25% of maternal deaths due to underlying congenital heart defects), not contraindicated but need special management

Question 3

What is Eisenmenger’s syndrome?

Answer 3

Eisenmenger’s syndrome is a condition in which there is pulmonary hypertension caused by increased pressures due to a left to right shunt.

Due to the pulmonary hypertension there is right ventricular hypertrophy and the pressure gradient reverses causing a right to left shunt.

Question 4

What are the clinical features of Eisenmenger’s?

Answer 4

Cyanosis and breathlessness.

There may be syncope attacks. May have chest pain.

O/e:
Cyanosis and clubbing.
Right ventricular heave.
Loud second heart sound with a narrow split.
Ejection systolic murmur audible along the left sternal border.