Pathophysiology of Congenital Heart Disease

Question 1

What is specific to congenital heart disease as opposed to broader heart disease?

Answer 1

You are born with it - resulting from incorrect embryological development

Question 2

Name two syndromes resulting from chromosome abnormalities that are associated with congenital heart disease (CHD for the purpose of these flashcards).

Answer 2

Downs

Turners

Question 3

What is a branchial arch?

Answer 3

Primitive arches giving rise to blood vessels post-natallly

Question 4

What does the 4th arch give rise to?

Answer 4

Right: Right Subclavian Artery
Left: Arch of Aorta

Question 5

What is the most common failure leading to CHD?

Answer 5

Septation

Question 6

How is the fossa ovalis kept open?

Answer 6

Pressure from r. atrium is strong so blasts through premium and secundum

Question 7

What does failure of septation lead to

Answer 7

VSD & ASD

Question 8

Failure of development leads to…?

Answer 8

Obstruction - atresia, stanosis or coarctation (narrowing) (of aorta)
Hypoplasia

Question 9

What is dextracardia?

Answer 9

Heart is on the wrong side

Question 10

What does failure of or incorrect rotation lead to?

Answer 10

TGA & congenitally corrected transposition of great vessels; dextrocardia

Question 11

What does ‘failure of closure’ refer to?

Answer 11

Ductus arteriosus

Question 12

What can be the result of pulmonary flow being greater than systemic flow (Qp>Qs)? (shunt ratio)

Answer 12

Heart failure

Reactive pulmonary hypertension

Question 13

What is the definition of cyanosis?

Answer 13

Greater than 5g of haemoglobin per decilitre of blood being deoxygenated

Question 14

What is the definition of pulmonary hypertension?

Answer 14

> 30mmHg systolic pressure (resulting from increased Qp)

Question 15

What two things can keep the foetal circulation going during surgery?

Answer 15

Prostaglandins

Rashkind

Question 16

What is polycythaemia?

Answer 16

Abnormally increased haemoglobin in the blood, most likely through overexertion of erythropoietin so increased RBC production and large hematocrit population

Question 17

What is pulmonary hypertension and what is it caused by?

Answer 17

High flow through pulmonary bed caused by genetic influences

Question 18

Where can differential cyanosis (pink top, blue bottom) be observed? Give more details about this ‘complex’.

Answer 18

Eisenmenger’s Complex
Left to right shunt reverses to become right to left shunt causing cyanosis
- murmur disappears

Question 19

What is the difference between ostium secundum ASD and ostium premium ASD?

Answer 19

Where the hole is: in secundum it is generally in isolation; in primum it is generally in association with other defects or factors

Question 20

Generally speaking, is there a murmur present in ASD?

Answer 20

No

Question 21

Generally speaking, is there a murmur present in VSD?

Answer 21

Yes

Question 22

What does aortic coarctation cause?

Answer 22

Systemic hypertension

Question 23

In which gender is aortic coarctation more common?

Answer 23

Males

Question 24

What is the treatment of aortic coarctation?

Answer 24

Balloon dilatation (or surgery)

Question 25

What does the Tetralogy of Fallot cause?

Answer 25

Cyanotic Heart Disease

Question 26

What does the Tetralogy of Fallot cause?

Answer 26

Cyanosis