Cardio - structural

Question 1

what are the four features of tetralogy of fallot?

Answer 1

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

Question 2

what % of congenital heart disease is tetralogy of fallot?:

Answer 2

10%

Question 3

what are some risk factors for tetralogy of fallot?

Answer 3

male
1st degree FHx
teratogens - alcohol, warfarin, trimethadione
genetics - 22q11 deletion

Question 4

what two components make up the septum of the ventricles?

Answer 4

smaller membraneous septum superiorly

larger muscular septum inferiorly

Question 5

what is the most common type VSD?

Answer 5

perimembraneous VSD

Question 6

state three types of VSD?

Answer 6

perimembraneous VSD
muscular VSD
doubly committed VSDs (near pulmonary and aortic valves)

Question 7

why are most people with VSD acyanotic ?

Answer 7

the left ventricle has a higher pressure than the right ventricle so there is a left to right shunt which can have a complication of pulmonary hypertension and ultimately right to left shunt

Question 8

what is the most common type of pulmonary stenosis?

Answer 8

infundibular septum

Question 9

what % of PS involves valvular stenosis?

Answer 9

10%

Question 10

what are tet spells ?

Answer 10

intermittent RVOT (right ventricle outflow tract) obstruction 
- hypoxic episodes

Question 11

what sign on CXR does RVH have?

Answer 11

boot sign

Question 12

in severe tetralogy of fallot what may form around the aorta?

Answer 12

multiple aorto-pulmonary collateral arteries (“MAPCAs”) may also form to help increase pulmonary blood flow

Question 13

what are the clinical signs of TOF?

Answer 13

asymptomatic 
murmur 
cyanosis 
respiratory distress 
faltering growth

Question 14

what ages are usually affected by tet spells ?

Answer 14

2-4 months of life

Question 15

what three things characterise tet spells?

Answer 15

paroxysm of hyperpnoea
irritability
increasing cyanosis

Question 16

what can precipitate hypoxic spells?

Answer 16

dehydration, anaemia, prolonged crying

Question 17

what type of murmur is present with VSD?

Answer 17

pan systolic murmur

Question 18

what murmur is present with dilated aorta?

Answer 18

ejection click

Question 19

do murmurs increase or decrease in intensity during hypoxic spells?

Answer 19

decrease in intensity

Question 20

what are some signs of congestive heart failure?

Answer 20

sweating, pallor, tachycardia, HSM, generalised oedema, bilateral basal crackles

Question 21

what murmur is present with pulmonary stenosis?

Answer 21

loud single S2 murmur

Question 22

what are DD for TOF?

Answer 22

isolated VSD 
sepsis 
transposition of the great arteries 
hypo plastic left heart syndrome 
totally anomalous pulmonary venous drainage

Question 23

what investigations should be done for TOF?

Answer 23

ECG 
Microarray 
CXR
ECHO
cardiac CT angiogram 
cardiac MRI 
cardiac catheter

Question 24

what medical treatment is available for TOF?

Answer 24

squatting - increases venous return and increases systemic resistance

prostaglandin infusion (PGE1 - alprostadil or PGE2 - dinoprostone)

beta blockers

morphine

saline 0.9% bolus (increases pulmonary flow through the RVOTO)

Question 25

what surgical treatment is available for TOF?

Answer 25

transcatheter RVOT sent insertion (done until child is bigger for valve replacement) modified blalock taussig shunt (increases pulmonary flow) stenosis resection, VSD repair

Question 26

what are the complications of TOF?

Answer 26

``` Polycythaemia Cerebral abscess Stroke Infective endocarditis Congestive cardiac failure Death (up to 25% in the 1st year of life4) Arrhythmias ```

Question 27

when should TOF be followed up ?

Answer 27

life long | - with regular ECG, ECHO, cardiopulmonary exercise testing (CPET)

Question 28

what is the gold standard for diagnosing TOF?

Answer 28

echo

Question 29

what genetic defect has the highest percentage incidence of TOF associated with it?

Answer 29

CHARGE syndrome

Question 30

what gives the boot shaped heart on CXR?

Answer 30

RVH

Question 31

what is the mortality of TOF in the first year of life?

Answer 31

25%

Question 32

what is dextro-transposition of the great arteries?

Answer 32

the aorta is anterior and to the right of the pulmonary artery

Question 33

what is levo-transposition of the great arteries?

Answer 33

the aorta may be anterior and to the left of the pulmonary artery

Question 34

is dextro-transposition or levo-transposition more common?

Answer 34

dextro-transposition

Question 35

what is the most common cause of cyanosis in a new born?

Answer 35

transposition of great arteries

Question 36

what gender is most affected by transposition of great arteries ?

Answer 36

males

Question 37

what three locations can mixing of blood occur to sustain life during transposition of great arteries ?

Answer 37

patent foramen ovale ventricular septal defect patent ductus arteriosus

Question 38

what valvular problem can result from levo-TGA ?

Answer 38

tricuspid regurgitation and heart failure due to high pressure on left side of heart

Question 39

what are maternal risk factors for TGA?

Answer 39

``` >40yrs DM rubella poor nutrition alcohol ```

Question 40

what would be found on examination with TGA?

Answer 40

Prominent right ventricular heave Single second heart sound, loud A2 Systolic murmur potentially if VSD present No signs of respiratory distress

Question 41

what would be since on ECG for tricuspid atresia?

Answer 41

left axis deviation

Question 42

what would be seen on CXR for TGA?

Answer 42

egg on a string

Question 43

what investigation is used to diagnosis TGA?

Answer 43

echo

Question 44

what is the management of TGA?

Answer 44

Emergency prostaglandin E1 infusion to keep the ductus arteriosus patent as a temporary solution that allows mixing of blood Correct metabolic acidosis Emergency atrial balloon septostomy to allow for mixing

Question 45

what are the complications of TGA?

Answer 45

Neopulmonary stenosis Neoaortic regurgitation Neoaortic root dilatation Coronary artery disease

Question 46

what acid base imbalance does TGA have?

Answer 46

metabolic acidosis | - distal organs have low oxygen supply and this respire anaerobically producing lactate

Question 47

what two things are poor development predictors for TGA?

Answer 47

low gestational age | high pre operative lactate

Question 48

what is the most common congenital heart defect?

Answer 48

VSD

Question 49

what is right to left shunt called?

Answer 49

Eisenmengers syndrome

Question 50

what are risk factors for VSD?

Answer 50

``` maternal DM rubella alcohol uncontrolled maternal phenylketonuria FHx of VSD Downs syndrome Trisomy 18/13 ```

Question 51

what clinical features will patients with small VSD have?

Answer 51

systolic murmur | asymptomatic

Question 52

what clinical features will patients with moderate VSD have?

Answer 52

excessive sweating tachypnoea most obvious at 2-3months

Question 53

what clinical features will patients with large VSD have?

Answer 53

symptoms of congestive heart failure development problems frequent chest infections

Question 54

what are some DD for VSD?

Answer 54

``` mitral regurg tricuspid regurg ASD patent ductus arteriosus pulmonary stenosis tetralogy of fallot ```

Question 55

what is the medical management of VSD?

Answer 55

increased caloric density of feedings - ensures weight gain diuretics ACEi digoxin - increases heart muscle strength during heart failure

Question 56

what advice should be given long term management of VSD due to increased risk of endocarditis ?

Answer 56

good dental hygiene | avoid piercing and tattoos

Question 57

what are complications of VSD?

Answer 57

``` Congestive heart failure Growth failure Aortic valve regurgitation Eisenmenger’s Syndrome Frequent chest infections Infective Endocarditis Arrhythmias Sudden death ```

Question 58

what % of small VSDs close spontaneously by 10years?

Answer 58

75%

Question 59

what % of downs syndrome have cardiac abnormalities ?

Answer 59

40-50%