Other Cyanotic Heart Defects (1)

Question 1

Transposition of the Great Arteries:
What occurs here?

What is it associated with?

What does the prognosis depend on?

Answer 1

➊ Aorta and Pulmonary trunks are swapped – 2 separate circulations that don’t mix

➋ • VSD
• Coarctation of aorta
• Pulmonary stenosis

➌ TGA isn’t compatible with life, therefore immediate survival depends on if there’s a shunt, like a PDA/SD (Septal defect)

Question 2

How does it present?

What will be heard O/E?
→ What’s the mechanism behind this?

How is it managed?

Answer 2

➊ * Most diagnosed antenatally
* Will present with Cyanosis at birth or within a few days when PDA/SD closes and no longer compensates

➋ Loud single S2
→ Normally, the aorta lies posteriorly to the pulmonary trunk. But in TGA, they lie beside eachother, so the sound of the valves closing superimposes (doubled up).

➌ * Prostaglandin infusion to maintain a PDA
* Balloon septostomy – Inflating balloon in Foramen Ovale via umbilicus to create a large ASD
* Surgical repair

Question 3

Truncus Arteriosus:
What occurs here?

What is it usually associated with?

How does it present?

How is it managed?

Answer 3

➊ • Single blood vessel comes out of the heart instead of two
• Blood from both sides is mixed and pumped to lungs and the rest of the body – Too much blood goes to the lungs so the heart has to work harder to pump to the rest of the body
• There’s 1 valve below this trunk, which is usually abnormal

➋ VSD

➌ • Most diagnosed antenatally
• Cyanosis and respiratory distress after birth

➍ Surgical repair

Question 4

Total Anomalous Pulmonary Venous Return:
What occurs here?

What is usually associated with this for compensation?

Hypoplastic Left Heart Syndrome:
What occurs here?

Answer 4

➊ Pulmonary veins form a connection with the pulmonary artery, therefore going back into the pulmonary circulation instead of the systemic circulation

➋ ASD

➌ • Hypoplasia of LV, Aortic valve (AS), Mitral valve (MS), and of ascending aorta
• Oxygenated blood from pulmonary veins is shunted left to right through an ASD. RV pumps blood through pulmonary arteries and ductus arteriosus to the descending aorta