textbook - cyanotic heart disease: most common fallot's tetralogy

Question 1

what is FT thought to be due to?

Answer 1

abnormalities in separation of the truncus arteriosus into the aorta and pulmonary arteries that occur during early gestation

Question 2

what are the four features of FT? PROVe

Answer 2

Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta
Ventricular septal defect (VSD)

Question 3

occasionally children can have an a___ as well

Answer 3

ASD

Question 4

what is the presentation at infancy?

Answer 4

• may be acynatotic at birth

- pulmonary stenosis murmur as the only initial finding.

Question 5

Gradually (especially after closure of the ductus arteriosus) infants become ____ due to decreasing flow of blood to the ____ as well as right to left shunt across the ___

Answer 5

Gradually (especially after closure of the ductus arteriosus) they become cyanotic due to decreasing flow of blood to the lungs as well as right to left shunt across the VSD

Question 6

what may children with FT present with?

Answer 6

dyspnoea, fatigue or with hypoxic episodes on exertion - deep cyanosis and possible syncope

this can result in seizures, cerebrovascular events or sudden death

Question 7

adults tend not to suffer spells but fatigue easily with ______ on exertion.

Answer 7

adults tend not to suffer spells but fatigue easily with dyspnoea on exertion.

Question 8

what are the signs of FT? 6

Answer 8

• pulmonary stenosis murmur.
• Parasternal heave is evident
• central cyanosis,
• clubbing,
• polycythaemia and
• failure to thrive.

Question 9

what investigations should be done?

Answer 9

CXR

• ECG
• CC

Question 10

what may be shown on CXR?

Answer 10

shows a large right ventricle and a small pulmonary artery

Question 11

what may be seen on ECG?

Answer 11

right ventricular hypertrophy

Question 12

why is CC performed?

Answer 12

to evaluate size and degree of right ventricular outflow obstruction

Question 13

what is the management?

Answer 13

• Give oxygen.
• morphine can sedate the child as well as relaxing the pulmonary outflow tract
• long term B blockers may be needed
• Give endocarditis prophylaxis only if recommended by a microbiologist

Question 14

surgery is usually done before __ year, with the closure of the ____ and correction of the _________

Answer 14

surgery is usually done before 1st year, with the closure of the VSD and correction of the pulmonary stenosis