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Flashcards in Structural heart defects Deck (42):
1

List some structural heart defects.

Ventricular septal defect
Atrial septal defect
Atrioventricular septal defect
Tetralogy of Fallot
Coarctation of Aorta
Eisenmenger Syndrome

2

What is a structural heart defect?

A problem with the structure of the heart, rather that the muscle (cardiomyopathy) or the ion channels (Channelopathies)

3

What are the structural problems in Tetralogy of Fallot?

4 problems (hence Tetralogy):

1. Ventricular Septal Defect

2. Pulmonary Artery Stenosis

3. Hypertrophy of R Ventricle

4. Overriding Aorta (overrides the V septal defect)

4

Describe the problems that occur as a result of the structural abnormalities in ToF.

1. Ventricular Septal Defect
2. Pulmonary Artery Stenosis
3. Hypertrophy of R Ventricle
4. Overriding Aorta

Pulmonary artery stenosis (2) causes RV pressure to be higher than LV

This causes the hypertrophy of the RV (3)

It also causes deoxygenated blood to flow through the ventricular septal defect (1) from RV to LV

The aorta overrides the ventricular septal defect (4) so blood from both the RV and LV goes up it

The body does not receive very well oxygenated blood

5

What impact does stress have on ToF?

Stress causes more deoxygenated blood to flow from the RV to the LV through the ventricular septal defect

Causing the blood that enters the aorta to be less oxygenated (hypoxaemia)

The body becomes starved of oxygen, cyanosis

6

How do patients with ToF present?

Very young

Tet spells
They go blue intermittently
Failure to grow & thrive
Dyspnoea
Heart murmur
Clubbing of fingers and toes

7

What are tet spells?

When babies with ToF suddenly get deep blue ski, nails & lips after crying or feeding or becoming stressed

Caused by a rapid drop in oxygen levels in the blood

8

What causes ToF?

Genetic mutations

9

Investigations for ToF?

Echo: best diagnostic tool, can even be done prenatally

10

Treatment of ToF.

Tet spells can be treated with B-blockers, or vasopressors

Surgical repair:
Patch across the ventricular septal defect, to restore a near normal circulation

11

When is surgical repair to treat ToF usually done?

About age 2

12

What's the prognosis of patients with ToF?

Good! Often they go on to live full and active lives

They do however often get pulmonary valve regurgitation in adult life, and require surgery to resolve this

13

What are the structural problems in Ventricular septal defect?

An abnormal connection (hole) between the 2 ventricles

14

What causes a ventricular septal defect?

Congenital most of the time

Acquired: after an MI (rare)

15

Is ventricular septal defect common?

Yes it makes up 20% of all congenital heart defects

16

If you are born with a ventricular septal defect, it will always need surgical intervention to close it up.
True or false?

False

Many ventricular septal defects close on their own

17

Describe the problems that occur as a result of ventricular septal defect.

Pressure in LV is higher than RV
So blood flows from LV to RV

This means the RV contains more blood than it should, so the lungs get a higher blood flow than they're used to.

18

Do patients go blue in ventricular septal defect?

No!

Since the lungs are not being starved of oxygen, they are getting more than they should be

19

Do all ventricular septal defects need repairing?

No, the small ones are asymptomatic and so they need no intervention.

20

What problems do you get with large ventricular septal defects?

Pulmonary hypertension
Which causes SOB, poor feeding, failure to thrive

21

What syndrome can occur as a result of ventricular septal defect?

Eisenenger's Syndrome

22

What are the clinical signs of a large ventricular septal defect?

Small, breathless, skinny baby

Increased resp rate
Tachycardia
Cardiomegaly
Murmur

23

What are the signs of a small ventricular septal defect?

Loud systolic murmur
Thrill: buzzing sensation
Well grown
Normal heart rate and size

24

Investigations for ventricular septal defect.

ECG: normal

CXR: may show cardiomegaly

Cardiac catheter into RV would show higher than normal O2 concentrations there

25

Management for ventricular septal defect.

Medical initially: check if hole will close up itself

If not surgical intervention

26

What are the structural problems in atrial septal defect?

There are 2 types.

A hole connecting the two atria

Ostium secundum: hole is high in septum

Ostium primum: hole is low in septum

27

What is the problem with Ostium primum atrial septal defects?

The hole often interferes with the AV valves... so more problems arise

28

When do each type of atrial septal defect present?
Why?

Primum: early in life

Secundum: later in life, 40-60, the severity of the L-R shunt depends on how well the ventricles are working, later in life they become less efficient so the L-R shunt causes more problems

29

Describe the problems that result from atrial septal defect?

L to R shunt

Blood flows from the left to the right atria

30

What are the clinical features of atrial septal defect?

Atrial fibrillation
Raised JVP
Pulmonary ejection systolic murmur

Pulmonary hypertension
Increased frequency of migraines

31

Investigation of atrial septal defect.

Echo

32

Management of atrial septal defect.

Closure before age 10

33

What structural problems do you get in atrioventricular septal defects?

A hole in the centre of the heart abnormally joining all of the heart chambers together

34

What genetic disorder is associated with atrioventricular septal defect?

Down's syndrome

35

Clinical features of atrioventricular septal defect.

Difficulty breathing (dyspnoea)

Cyanosis

Signs of heart failure such as oedema, fatigue, wheezing, sweating and irregular heartbeat

36

Investigations of atrioventricular septal defect.

Auscultation: there are murmurs and sounds characteristic of atrioventricular septal defect

Echo

37

Management of atrioventricular septal defect.

Close the hole

38

What is Eisenmengers Syndrome?

When high pressure pulmonary blood flow caused by a septal defect results in damage to the delicate pulmonary vasculature

39

What happens as a result of Eisenmengers syndrome?

Damage to pulmonary vasculature causes increased resistance to blood flow through the lungs

This means the pressure in the RV builds up due to backlog created by resistance

Raised RV pressure means that the shunt reverses and blood goes from R to L.

The LV fills with deoxygenated blood, the body receives this blood so becomes starved of oxygen

40

What are the clinical features of Eisenmengers syndrome?

Blue! Cyanosis

Heart failure
Fainting
Abnormal heart rhythms
Bleeding disorders
Coughing up blood
Iron deficiency
Infections (endocarditis and pneumonia)
Kidney problems
Stroke

41

What conditions can lead to Eisenmengers syndrome?

Ventricular septal defect
Atrial septal defect

42

What is the prognosis after developing Eisenmengers syndrome?

Poor