Congenital Heart Defects (The Bare Minimum)

Question 1

type of murmur in PDA?

Answer 1

continuous “machine like” murmur

loudest under left clavicle

Question 2

other presenting features of PDA?

Answer 2

often asymptomatic if small and can close spontaneously
generally doesnt cause cyanosis but can do eventually if not corrected
can be asymptomatic until adulthood then cause heart failure
symptoms include:
- SOB
- difficulty feeding
- poor weight gain
- LRTI

Question 3

diagnosis of PDA?

Answer 3

ECHO

doppler during ECHO can assess size of shunt

Question 4

how is PDA managed if symptomatic?

Answer 4

NSAIDs can close PDA pharmacologically (eg ibuprofen or indomethacin)
if associated with another congenital heart defect amenable to surgery then prostaglandin E1 is useful to keep duct open until surgery

Question 5

how is asymptomatic PDA managed?

Answer 5

can be monitored up to 1 year old and see if it closes on its own
if it hasnt closed by 1 year then its closed surgically

Question 6

most likely congenital heart defect to be found in adulthood?

Answer 6

ASD

Question 7

examination features in ASD?

Answer 7

can be asymptomatic
ejection mid-systolic murmur heart best at upper left sternal border
fixed split second heart sound (S2)

Question 8

2 types of ASD?

Answer 8

ostium primum
ostium secundum (70% of ASDs,)

Question 9

ECG features of ostium primum?

Answer 9

RBBB with LAD

prolonged PR interval

Question 10

ECG features of osttium secundum?

Answer 10

RBBB with RAD

Question 11

presenting symptoms of ASD in children?

Answer 11

SOB
difficulty feeding
poor weight gain
LRTI

Question 12

presnting symptoms of ASD in adults?

Answer 12

heart failure
dyspnoea
stroke

Question 13

complications of ASD?

Answer 13

stroke (DVT becomes an embolus and travels to right side of heart, through ASD to left side then up to brain)
AF
atrial flutter
pulmonary hypertnesion and right sided heart failure (blood shunted from left to right causing increased pressure on right side)
eisenmenger syndrome

Question 14

what is eisenmenger syndrome?

Answer 14

where pressure builds up so much in right side of heart that the pressure becomes higher than the left side so the shunt changes from left > right to right >left

Question 15

how is ASD managed?

Answer 15

can watch and wait if small and asymptomatic
can be surgically corrected via transvenous catheter closure or open heart surgery
anticoagulants/antiplatelets used in adults to reduce stroke risk

Question 16

what syndrome is associated with ASD?

Answer 16

downs

also associated with atrioventricular septal defect

Question 17

most common congenital heart disease?

Answer 17

VSD

Question 18

what syndromes are associated with VSD?

Answer 18

downs
edwards
pataus
cri-du-chat
turners?

Question 19

other associations with VSD?

Answer 19

congenital infections

post-MI

Question 20

how does VSD present?

Answer 20

often asymptomatic initially and patients can present in adulthood
possible symptoms in newborns
- poor feeding
- dyspnoea
- tahcypnoea
- failure to thrive 
can have symptoms of heart failure

Question 21

examination findings in VSD?

Answer 21

pan-systolic murmur heard best at left lower sternal border in 3rd/4th intercostal space
can have systolic thrill on palpation

Question 22

how can VSD (or ASD) be picked up?

Answer 22

during 20 week antenatal scan

ECHO in childhood/adulthood when presenting with symptoms

Question 23

possible complications of VSD?

Answer 23

aortic regurgitation 
eisenmengers
right heart failure
pulmonary hypertension
endocarditis

Question 24

management of VSD?

Answer 24

small, asymptomatic VSDs with no signs of pulmonary hypertension or heart failure can just be monitored, often close spontaneously
can be corrected surgically with TV catheter closure or open heart surgery

Question 25

main heart defect associated with turners?

Answer 25

coarctation of aorta

Question 26

examination features of coarctation of aorta?

Answer 26

crescendo-decrescendo systolic murmur at upper left sternal edge weak femoral pulses high blood pressure in limbs supplied by arteries that branch off before the narrowing and low BP in limbs supplied by branches after narrowing systolic murmur heard best below left clavicle and below left scapula

Question 27

symptoms of coarctation of aorta?

Answer 27

``` tachypnoea increased work of breathing poor feeding grey and floppy baby other signs may develop over time including left ventricular heave and underdevelopment of limbs where there is reduced blood flow ```

Question 28

management of coarctation of aorta?

Answer 28

some can be left until adulthood without causing an issue | some need emergency surgery shortly after birth

Question 29

management while waiting for surgery in cases of critical coarctation of aorta where there is risk of heart failure and death?

Answer 29

prostaglandin E used to keep ductus arteriosus open which allows some blood flow through PDA into the systemic circulation distal to the coarctation surgery then done to correct coarctation and to ligate the PDA

Question 30

4 features of tetralogy of fallot?

Answer 30

VSD overriding aorta pulmonary valve stenosis RVH

Question 31

what is overriding aorta?

Answer 31

entrance to aorta is further to the right than normal, above the VSD meaning that when the right ventricle contracts and sends blood upwards towards the pulmonary trunk, some of the deoxygenated blood enters the aorta

Question 32

direction of blood shunt in tetralogy of fallot?

Answer 32

right to left | - bc of pulmonary stenosis causing increased resistance to flow and higher pressure in right side of heart

Question 33

how is tetralogy of fallot diagnosed?

Answer 33

ECHO (doppler done during) | CXR shows "boot shaped heart" due to RVH

Question 34

examination features of tetralogy of fallot?

Answer 34

ejection systolic murmur heard best at left sternal border (due to pulmonary stenosis) cyanosis may develop signs of heart failure before 1 year or older if less severe

Question 35

signs and symptoms of tetralogy of fallot?

Answer 35

``` cyanosis (due to right to left shunt) clubbing poor feeding poor weight gain "tet spells" ```

Question 36

what are tet spells?

Answer 36

intermittent symptomatic periods where the right to left shunt is worsened causing a cyanotic episode can be precipitated by physical exertion, crying, waking etc

Question 37

symptoms of tet spells?

Answer 37

child becomes irritable, cyanotic and short of breath | can lead to reduced consciousness, seizures and potentially death

Question 38

management of tet spells?

Answer 38

older children = squat younger children = knees to chest can give oxygen, beta blockers, fludis, morphine, sodium bicarb or phenylephrine

Question 39

management of tetralogy of fallot?

Answer 39

prostaglandin infusion to maintain DA allowing blood to flow into pulmonary arteries total surgical repair via open heart surgery is definitive treatment

Question 40

when does tetralogy of fallot present?

Answer 40

1-2 months

Question 41

what can present similarly to tetralogy of fallot and how can they be differentiated?

Answer 41

transposition of great vessels TGV presents in first days of life TOF present at 1-2 months

Question 42

cyanotic vs non-cyanotic congenital heart diseases?

Answer 42

``` cyanotic = tetralogy of fallot, transposition of great vessels non-cyanotic = PDA, ASD, VSD, coarctation ```