Lecture Congenital Heart Disease

Question 1

You are a 4th year medical student
Hi Dr I have just done an examination of a 1 day old newborn baby I’m pretty sure I can hear a murmur but there were no other abnormal findings on cardiovascular examination. Is this a sign of aortic stenosis?

Answer 1

It depends
in the absence of any other abnormal pathology and just hearing the soft systolic murmur alone in a neonate no
If anything it is more likely to be due to tricuspid regurgitation

Question 2

Hi Dr one of the medical students was asking me if you hear a murmur on a 3 day old baby is this normal what should I tell her

Answer 2

no if this is a benign murmur you would expect it to disappear on day 2

Question 3

Hi Dr I’m about to do the cardiovascular examination of a child with Down syndrome trisomy 21 and I was wondering what type of mermaids should I expect to hear if he does have one

Answer 3

The two types of murmurs associated with trisomy 21 are ventricular septal defects and ventricular septal defect

Question 4

Hi
I have just done an examination of a 12 year old girl who has a wet neck wide carrying angle and widely spaced nipples I suspect he has Turner syndrome (XO) and so I wanted just double check in my CVs examination as I know there’s a specific congenital heart defects associated with this syndrome. Which congenital heart problem

Answer 4

Aortic coarctation

Question 5

Hi Dr I am one of the fourth year medical students I’ve just finished doing an examination of a 13 year old girl with the diagnosis of Turner syndrome I know that aortic coarctation is sometimes associated with this syndrome what is aortic coarctation

Answer 5

Aortic coarctation refers to the narrowing of the aorta typically around the insertion of the ductus arteriosus

Question 6

Hi Dr I am about to do an examination of a 2 week old boy with Turner’s syndrome which major blood vessel narrowing condition is associated with Turner syndrome

Answer 6

Aortic coarctation

Question 7

Hi Dr I am one of the fourth year medical students who is doing some research into to trilogy of Fallot I’ve just finished examining 1 week old baby mum reports that the baby has been having difficulty feeding and often seems out of breath after feeding it’s also has a bluish tint to its skin. She asked me what I thought was going on and I told her about the condition called tetralogy of Fallot. although I don’t believe I explained it correctly can you explain it to me

Answer 7

In simple terms to tetralogy of Fallot refers to a congenital malformation of the heart to resulting in inefficient blood flow research has shown that tetralogy of Fallot along with the George syndrome associated with a chromosomal deletion at 22q 11.2

Question 8

Hi Dr
I have just seen a one week old baby boy who has an unusually shaped chest undescended testes a long face and on auscultation I could hear a loud murmur. We learnt about conditions such as Down’s Edwards tetralogy of fallot di George noonans. Can you tell me what actually is Noonan syndrome

Answer 8

Noonan syndrome refers to the presentation of a wide variety of symptoms including heart defects abnormality in chest formation micrognathia high nasal Bridge and a series of other signs that all results are due to a problem with the ptpn11 Gene which is an autosomal dominant Gene

Question 9

Hi Dr I have just seen a child with Downs
Because the consultant was worried that the child may have a heart defect they sent the child for an echocardiogram which revealed a ventricular septal defect. I was wondering is down the only trisomy which may be associated with ventricular septal defect

Answer 9

No in fact Edwards is also associated with ventricular septal defect and downs may also be associated with atrioventricular septal defect

Question 10

Hi Dr I have just witnessed a baby being born however the baby looks very blue I was wondering which congenital heart defects typically present with a blue baby

Answer 10

Tetralogy of Fallot, transposition of the great arteries and eisenmenger complex

Question 11

Hi Dr I have just seen a baby being delivered and noticed it looks very pink which gives it a 2 on the APGAR score for appearance however I know that some babies with congenital heart defects may look pink. Which congenital heart defects

Answer 11

Atrial septal defect ventricular septal defect and patent ductus arteriosus

Question 12

Hi Dr I have just seen a one week old girl who was born with Down syndrome I know that down syndrome can be associated with ventricular septal defect. There was one more which was a mixed shunt but I can’t remember what it was called

Answer 12

Atrio ventricular septal defect

Question 13

Hi Dr can you tell me what hypoplastic left heart syndrome is

Answer 13

Hypoplastic left heart syndrome is a congenital defect where by the left side of the baby’s heart doesn’t develop properly resulting in a sick child who may have poor feeding

Question 14

Hi Dr
I have just completed and cardiovascular examination of a two week old baby who has a soft systolic murmur. I could hear the murmur most clearly at the upper left sternal Edge. I know that there is a type of heart defect which is associated with something called a fixed split S2 alongside a soft systolic murmur. But I don’t actually know what S2 refers to

Answer 14

S2 refers to the end of ventricular systole or in other words ventricular contraction and the sound that you hear is the aortic and pulmonic valves closing

Question 15

Hi Dr I am one of the first year medical students doing boring heart embryology. Make it more interesting I have come here on the ward to see congenital heart defects and how they present in young babies. Specifically talking about EG real septal defect I remember that you can get a primum or secundum which one is more common

Answer 15

Secundum

Question 16

Hi Dr the mother of the two week old baby was told that her daughter has an atrial septal defect. The cardiologists aren’t exactly sure how large the defectors as of right now and the Mum what was wondering what would happen if she doesn’t get it treated

Answer 16

It completely depends on how big the hole is if the hole is small it can be left if the hole is big enough then the long term complication from allowing an opening between the left and right atria is one slow from the left atria to the right atria which initially isn’t problem but after time the increased pressure in the right atria will result in right sided heart hypertrophy the right sided heart hypertrophy results in pulmonary hypertension as the right side of the heart is pumping blood directly to the pulmonary vasculature if pulmonary hypertension is sustained then this increases the pressure in the left side of the heart this this increased pressure then flows back to the right side of the heart in a positive feedback cycle. Eventually the flow reverses so that the right side of the heart which is hypertrophy and under high pressure send it to deoxygenated blood into the left atrium. It is at this point but the atrial septal defect is now referred to as eisenmenger’s complex

Question 17

Hi Dr I have just been speaking to a well-educated set of parents who have a medic in the family they want to know in detail how you close a secundum atrial septal defect

Answer 17

Secundum atrial septal defect can either be closed with percutaneous procedure in which a catheter is inserted into the appropriate section of the heart and then a mesh placed to fill the hole which is more common in developed countries

Question 18

Hi Dr I know that you can get a secundum atrial septal defect and primum atrial septal defect and that the primum atrial septal defect can go under another name what is the other name for a primum atrial septal defect

Answer 18

Partial atrioventricular septal defect which can be associated with Down’s

Question 19

Hi Dr I was just wondering there a lot of congenital heart defects and how am I supposed to distinguish between a primum atrial septal defect and secundum atrial septal defect

Answer 19

The only difference with the primum atrial septal defect is that you hear an apical systolic murmur everything else is still the same her first at the upper left sternal edge with a fixed split S2 sound

Question 20

Hi Dr I just need some quick tips to pass my exam how my supposed to remember that a ventricular septal defect is associated with a pansystolic murmur heard best at the left lower sternal Edge

Answer 20

The ventricles are lower down that’s how you remember that it’s lower do you remember that it’s left because that is where the escalation site closest to the sternum are located and because it’s a ventricular septal defect it’s going to be heard most clearly when the ventricles contract which is why you get a systolic murmur where systolic refers to ventricular contraction

Question 21

Hi Dr I have just seen a 2 week old baby the baby is Mum was wondering how long she would have to wait before she’s able to get the treatment for her baby as her baby has a partial atrioventricular septal defect

Answer 21

The surgeries are usually done when a child is 3 years old

Question 22

Hi Dr I know that ventricular septal defect can be associated with Downs and Edwards syndrome the mum of a 3 week old baby boy was wondering when her child would be able to have the operation to fix the problem

Answer 22

Normally this operation is done at 3 months

Question 23

Hi Dr how do I remember what the treatment is surgically for a persistent ductus arteriosus

Answer 23

The hint is in the name and in your anatomical knowledge if you can remember that the persisting arteriosus duct is a connection between the aorta and the pulmonary artery then the surgery logic is straightforward you simply have to occlude the connection and everything will go back to normal and therefore we can do this with a device percutaneously

Question 24

Hi Dr how do I remember what the first line treatment is for large left to right shunt referring to atrial septal defect ventricular septal defect and persistent ductus arteriosus

Answer 24

Simply remember the explanation behind the formation of eisenmenger’s complex as a result of long term large secundum atrial septal defect. Essentially the problem with these holes in the heart is associated with the pressure imbalance specifically placed by the right side of the heart please have normal pressures can result in signs of hypertrophy hypertrophy which is prolonged becomes heart failure and this the management is the same as heart failure where you would use diuretics to remove any excess fluid build-up and then you would use blood pressure medications such as ACE inhibitors to reduce excess pressure

Question 25

Hi Dr can you explain to me what ebsteins anomaly anomaly is

Answer 25

a congenital malformation characterized by Shrinkage of the right ventricle to such a point where it is closer to the size of a right atria

Question 26

Hi Dr can you explain to me what the similarities between pulmonary atresia and ebstein’s anomaly

Answer 26

In epsteins anomaly you get a reduced right ventricle which means you get less blood flow to the lungs. In pulmonary atresia you have a problem with the pulmonary valve which is separating the right ventricle and the pulmonary artery which means you get reduced blood flow to the lungs

Question 27

Hi Dr can you explain the similarity between ebstein’s anomaly and pulmonary atresia please

Answer 27

In epsteins anomaly you get a reduced right ventricle which means you get less blood flow to the lungs. In pulmonary atresia you have a problem with the pulmonary valve which is separating the right ventricle and the pulmonary artery which means you get reduced blood flow to the lungs which is why you end up with the blue baby because the baby is not getting enough oxygenated blood around its system

Question 28

Hi Dr I was reading over the congenital heart defects and finally understood why transposition of great arteries can be considered blue baby syndrome as opposed to to tetralogy of Fallot I am going to tell you what I learnt. What is the medical student going to tell the doctor. In other words explain transposition of great arteries

Answer 28

In transposition of great arteries you get two separate circuits formed the left ventricle which normally should be connected to the aorta sending oxygenated blood around the body becomes connected to the pulmonary artery instead which means the oxygenated blood get sent back to the lungs and then back to the left atrium and then back into the left ventricle and round and round in an oxygenated lung heart feedback loop on the other side of the heart the right side the right ventricle should normally be connected to the pulmonary artery so deoxygenated blood from the body can go back to the lungs pick up oxygen return to the left side of the heart then to the left ventricle and then to the aorta but the right ventricle becomes connected to the aorta instead so deoxygenated blood from the body gets circulated back round the body again resulting in inadequate oxygen supply to the rest of the body which is why this condition will present as blue baby syndrome in other words cyanosis if there are no other heart defects present specifically a ventricular septal defect or atrial septal defect

Question 29

Why is it almost essential for a baby survival that is it has transposition of great arteries syndrome it’s also has an atrial septal defect or ventricular septal defect

Answer 29

A hole in any of the chambers of the heart will allow for some of that oxygenated blood to circulate around the body which will be essential if the baby is to survive

Question 30

If the baby does not have an issue septal defect or ventricular septal defect associated with its transposition of great arteries then what surgical operation can be performed in order to create an opening

Answer 30

Atrial septostomy in which a balloon is inflated to allow oxygenated blood to enter the deoxygenated circuit.

Question 31

Alongside atrial septostomy which medication needs to be given to keep the opening open if a baby has transposition of great arteries also known as blue baby syndrome

Answer 31

Prostaglandin

Question 32

Hi doctor

I have just seen a 6 month old boy who has had a temperature for the past 5 days. His temp is 39, his HR shows tachy, his RR is elevated, sats are fine. Examination findings reveal rash, cracked lips and conjunctivitis and a strawberry tongue on inspection. I suspect the child may have Kawasaki disease but i cant remember what the treatment is

Answer 32

Aspirin 10 mg / kg every 6 hours

IVIg 2g per kg

Question 33

Hi Doc one of the nurses was asking me

I have put in a catheter and have started the Ig drip of immunoglobulin at a dose of 2g per kg and I have been giving oral aspirin at a dose of 10 mg per kg every 6 hours. The handover for this particular patient wasnt good and so i dont actually know whats wrong with the little boy. I remember the doctor said it was some kind of vasculitis beginning with K

Answer 33

Kawasaki disease

Question 34

Hi doctor

I have just seen a newborn with cafe au lait macules and i was wondering if this was associated with any neuro skin conditions

Answer 34

yes neurofibromatosis

Question 35

Hi doctor

I am one of the medical students
I was feeling confident that i knew all about impetigo until i saw a child who had impetigo (golden crusted lesions) with blisters. Apparent there are 2 versions of impetigo called bullous and non bullous which are associated with different bacteria. Staph is associated with bullous right?

Answer 35

That is correct

Question 36

HI doctor

I was doing some reading on common almost pathognomic presentations of certain skin conditions. One buzzword that i keep hearing is strawberry tongue. I remember that this can be associated with Kawasaki but i cant remember the other one? Please can you help me. (I think the otherone was associated with a sandpaper rash

Answer 36

scarlet fever

Question 37

HI doctor I have just seen a 7 year old boy with a widespread rash all over his back that looks like gooose pimples and he has a bright red tongue. Im thinking that he mifht have scarlet fever but i cant remember the key investigations i need to go on to do

Can you tell me

Answer 37

So just think about it logically. Initially think throat swabs to try and catch the infection

Then you can do a special blood test to look for anti DNAase B titres

Question 38

Hi doctor

I have just finished clerking Mrs …. who has come in with her daughter. She has noticed an
A - a widespread macular rash, not itchy, not weeping, no vesicles but she has a strawberry tongue
B - with defined borders
C - pink in colour
D - each lesion is relatively small

I have taken bloods which i have sent off for anti - DNAase B titres

The result was positive for strep a infection. Which medication should i start the child on?

Answer 38

Antibiotics specifically the beta lactam penicillin or a macrolide like erythromycin

Question 39

You have a fourth year medical students and you have just been put on the spot

He’s just gone to 6 year old child who has a temperature which is been going on for the last couple of days. The mother tells you that the child has also had a cough he has a rash and has a runny nose so you decide to do an examination

On examination you notice on inspection he has conjunctivitis a runny nose.

The mum suddenly remembers an interesting detail about the rash she remembers that the rash originally started on his face but then has been spreading to his trunk and his limbs

Answer 39

The diagnosis here is measles a highly infectious viral infection

Question 40

Hi Dr I need to get some advice for the investigations I need to do for this four year old girl that I believe has measles

Answer 40

Start with swabs and then check the IGM levels

Question 41

You have just seen a child who has come in on day 6th of his measles infection from when his mum first noticed a rash. The mum is worried that the child will spread measles to his siblings and the other kids and his class will get it.
Based on what you know about when measles infectious what would you tell the mum

Answer 41

Measles can be transmitted 4 days before the appearance of a rash and 4 days after the rash has appeared

Question 42

Hi Dr I have just seen a child with a rash that Mum is concerned about the rash is quite itchy and started the couple of days ago there had been a rash going around school. He is eating and drinking and weeing and pooing normally. You find out that the child older brother recently had chickenpox. How long does it typically take from infection to presentation of symptoms

Answer 42

10 to 20 days

Question 43

Hi Dr I am on the third year medical students and I was wondering which type of virus causes chickenpox

Answer 43

Herpes virus specifically the varicella-zoster virus hhv 3

Question 44

Hi Dr I have just seen a patient who has an extensive family history of excellence so she can be a bit of an expert she was telling me about this protein called filaggrin that I had heard I had no idea what it was can you tell me what it is

Answer 44

Celebrities associated with the theories of what causes eczema so apparently there’s a protein called for lager in which is mutated in people who get eczema just resulting in a leaky skin barrier which is a precursor to the eczema

Question 45

Hi Dr I heard that apparently there was a specific types of T cells subtype associated with eczema wear this t subtype produces certain interleukins 34 and 13

Answer 45

Th2