Congenital Heart Diseases

Question 1

Definition of congenital cardiac diseases

Answer 1

Structural defects occur during embryogenesis (complex process of apoptosis & morphogenesis)
Complex = forms from primitive heart tube & formation of chambers & outflow tracts/valves

Question 2

6 clinical presentation of congenital cardiac disease

Answer 2

Cyanosis
Tachypnoea
Failure to thrive
Poor feeding
Murmur
Clubbing

Question 3

2 types of congenital cardiac diseases & define

Answer 3

1. Shunts: abnormal blood flow between chambers (direction is dependent on pressure)
2. Obstructions: due to valve narrowing/atresia or blood vessel/outflow narrowing

Question 4

What is a characteristic of a right to left shunts presentation

Answer 4

Cyanosis as it bypasses pulmonary circulation
T’s

Question 5

4 subtypes of right to left shunts

Answer 5

1. Tetralogy of Fallot
2. Tricuspid atresia
3. Truncus arteriosus
4. Transposition of great vessels

Question 6

Left to right shunt presentation

Answer 6

Late cyanosis & pulmonary HPT (excess blood in pulmonary circulation)
Eisenmenger complex = due to PHPT switch to R to L shunt causing late cyanosis
D’s

Question 7

3 subtypes of left to right shunts

Answer 7

1. Atrial septal defect
2. Ventricular septal defect
3. Patent ductus arteriosus

Question 8

2 subtypes of obstructions

Answer 8

1. Aortic co-arctation
2. Tubular hypoplasia of aortic isthmus

Question 9

What is tetralogy of Fallot

Answer 9

VSD
Pulmonary valve stenosis (protect lungs from high cardiac pressure to prevent pulmonary HPT)
RV hypertrophy
Aorta override VSD

Question 10

What is tricuspid atresia

Answer 10

Regurgitation causing RV hypertrophy

Question 11

What is truncus arteriosus

Answer 11

No septation between pulmonary and aortic arteries = mixing of blood (one vessel leaving heart)
Blood shunts to lungs = low pressure = fluid overload = heart failure

Question 12

What is transposition of great vessels

Answer 12

Failure of spiral septation = heart failure (heart not adapted for ventricles to switch roles)
Pulmonary & systemic circuits do not mix

Question 13

What is atrial septal defect

Answer 13

At foremen ovale
Can cause paradoxical embolism
Murmur or healthy child

Question 14

What is ventricular septal defect

Answer 14

At membranous part
Small closes spontaneously
Pan systolic murmur (smaller = louder murmur)

Question 15

What is patent ductus arteriosus

Answer 15

Connect aortic & pulmonary trunk = blood mix & hyperkinetic PHPT
Loud machinery murmur
Sometimes beneficial to keep open
In premature, maternal rubella & hyaline membrane disease

Question 16

What is aortic co-arctation

Answer 16

Narrowing of aorta at ductus arteriosus
More common in male
Hypertension before obstruction & hypotension after obstruction
Causes co-lateral arteries to open at intercostals & cause rib erosion

Question 17

What is tubular hypoplasia of aortic isthmus

Answer 17

Aortic valve stenosis/atresia
Infantile coarctation, Turner syndrome, cystic hygroma or inter utero death

Question 18

What is 3 complications of congenital cardiac disease

Answer 18

Cardiac failure
Pulmonary hypertension
Infective endocarditis