Cardiology - Cyanotic Heart Disease

Question 1

Definition

Answer 1

Heart defects that cause decreased oxygenation of the blood. Deoxygenated blood bypasses the lungs and enters systemic circulation through the aorta, causing cyanosis.
RIGHT TO LEFT SHUNTING.

Question 2

Types of heart defects (5,4,3,2,1)

Answer 2

5: Total Anomalous Pulmonary Venous Connection
4: Tetralogy of Fallot
3: Tricuspid Atresia
2: Transposition of the Great Arteries
1: Truncus Arteriosus

Question 3

What is Total Anomalous Pulmonary Venous Connection (TAPVC)

Answer 3

The pulmonary veins connect to either the right atrium or the systemic venous system

Question 4

What is tetralogy of Fallot

Answer 4

1. Ventricular septal defect (VSD)
2. An overriding aorta: the aorta opens to the septum, and in TOF the VSD
3. Pulmonary valve stenosis
4. Right ventricular hypertrophy

Question 5

What is tricuspid atresia

Answer 5

There is no direct blood flow between the right atrium and ventricle because the tricuspid valve is not properly formed

Question 6

What is Transposition of the Great Arteries (TGA)

Answer 6

The aortic and pulmonary artery vessel attachments are swapped, resulting in two separate circulations , one oxygenated through the pulmonary system and one systemic system that is deoxygenated

Question 7

What is truncus arteriosus

Answer 7

There is one great artery as opposed to two. This supplies the coronary arteries, pulmonary arteries and the aorta. This occurs in combination with a large VSD

Question 8

Epidemiology

Answer 8

• Genetic syndromes: DiGeorge syndrome
• Maternal illness during pregnancy: rubella
• Maternal alcohol or drug use
• Maternal use of certain medications during pregnancy

Question 9

Signs

Answer 9

Peripheral and central cyanosis
Clubbing
Failure to thrive
Systolic murmur from the turbulence of the pulmonary stenosis:
- Ejection systolic with TOF (pulmonary valve stenosis)

Question 10

Symptoms

Answer 10

• Bluish-purple discolouration, most often visible at the lips, mouth, fingernails, and earlobes
• Difficulty feeding
• Poor weight gain or weight loss

Question 11

Investigations

Answer 11

• Antenatal USS: second trimester
• Echo: “boot shaped” - right ventricular thickening
• Chest X-ray: cardiomegaly
• ECG

Question 12

Management

Answer 12

TET SPELL:
- Knees to chest
- Oxygen
- IM morphine

Immediate management:
- Prostaglandin E1: used to keep ductus arteriosus open maintaining systemic blood flow
- Balloon septostomy: large atrial septal defect is made

Definitive management:
- Surgical correction: arterial switch operation for TGA or Blalock-Taussig shunt followed by corrective surgery for TOF
- Palliation

Question 13

Complications

Answer 13

Eisenmenger syndrome
Bacterial endocarditis
Hypoxic brain injury
Polycythaemia and thrombosis