Congenital Heart defects

Question 1

What are the features of Tetralogy of Fallot(ToF)?

Answer 1

1. Large VSD
2. Right ventricular outflow tract obstruction secondary to infundibular and/or pulmonary valvular stenosis
3. Overriding aorta (overhang less than 50% of diameter of VSD)
4. right ventricular hypertrophy

Question 2

Aetiology of ToF?

Answer 2

• unknown

- occasionally associated with 22q11.2 deletion

Question 3

Prognosis of ToF?

Answer 3

• 36 year survival rate post surgical repair is 85%
• increased risk of developing arrhythmias and pulmonary regard
• excellent survival rate if obstruction of the RV outflow tract (RVOT) is corrected and VSD closed completely

Question 4

Does the patient need prophylaxis for subacute bacterial endocarditis before dental procedures?

Answer 4

Some, post repair.

Question 5

What is Eisenmengers Syndrome

Answer 5

A consequence of a large unrepaired VSD.

• increased right ventricular pressure from the Left to right shunt leads to pulmonary hypertension.
• pulmonary hypertension leads to increased pulmonary vascular resistance, increased pressure in the right ventricle become greater than those in the left ventricle
• left to right shunt is reversed.
• leads to a systemic cyanosis.

Question 6

what is the most common congenital heart defect in babies?

Answer 6

VSD.
- ASD is the most common congenital heart defect overall.
- However, VSD is more common in babies, but 30-50% close spontaneously in childhood.
-

Question 7

what sort of murmur do you hear with a VSD

Answer 7

holosystolic murmur at the lower left sternal edge

Question 8

Which parts of the heat have increased SpO2 levels with a VSD?

Answer 8

• right ventricle

- pulmonary artery

Question 9

When does a VSD require surgical repair

Answer 9

when it is large.

Question 10

What are the most common congential heart defects?

Answer 10

ASD

Question 11

Which syndromes are associated with VSD and ASD’s

Answer 11

Foetal alcohol syndrome
Down’s Syndrome
Other congenital heart defects.

Question 12

Describe the features of TAPVR.

Answer 12

• the pulmonary veins drain into the venous system rather than the left atrium
• left atrium isolated from the systemic circulation
• is a cyanotic lesion
• incompatible with life unless there is an ASD or PFO
• paediatric cardiac surgical emergency as prostaglandins are not usually effective.

Question 13

Describe the ECG changes seen in TAVPR

Answer 13

Right axis deviation, right ventricular hypertrophy, P waves are tall and spiked,

Question 14

In which congenital cardiac condition does the CXR reveal a “snowman” appearance?

Answer 14

TAPVR.

Question 15

What is the most likely diagnosis given these echo findings?

• large right ventricle
• abnormal pulmonary venous connections
• right to left shunt across the atria

Answer 15

TAPVR

(Any vein showing doppler flow away from the heart is pathognomonic for TAPVR

Question 16

What is the pattern of Spo2 in the heart in TAPVR?

Answer 16

similar SpO2 in the aorta, atria and ventricles

Question 17

What is the treatment for TAPVR?

Answer 17

• surgical repair

- ECMO if surgery cannot be perform as an emergency