Congenital Heart Disease 1 (Mayo)

Question 1

Physical exam findings in Secundum ASD

Answer 1

RV heave
PA SEM +/- TV diastolic murmur
FIXED SPLIT S2

Question 2

ECG w/ RBBB and RAD. CXR with enlarged PA (c/w Ao). Dx?

Answer 2

Secundum ASD

Question 3

If you are thinking of Secundum ASD and see enlarged LA, what is Dx?

Answer 3

Primum ASD

Question 4

Main complications of Secundum ASD

Answer 4

Afib (from enlarged RA)

Question 5

What class indication is cath for Secundum ASD?

Answer 5

III

Question 6

When do you close Secundum ASD?

Answer 6

If any RV enlargement (shunt calc doesn’t matter!)

Question 7

If echo shows RV volume overload and no ASD, what do you think of?

Answer 7

Anomalous Pulm veins

Question 8

Diagnosis of defect in superior atrial septum, posterior to fossa ovalis and anomalous pulmonary veins

Answer 8

Sinus venosus ASD

Question 9

If RV overload, what do you do?

Answer 9

Find the shunt

Question 10

Defect of inferior septum, MV cleft and Down’s syndrome

Answer 10

Primum ASD

Question 11

What is the relation of AV valves in Primum ASD?

Answer 11

On same level

Question 12

Normally, which valve is apically displaced?

Answer 12

TV

Question 13

**What type of ASD has RBBB with LAD?

Answer 13

Primum ASD

Question 14

Name 3 associations with Primum ASD

Answer 14

Sub aortic stenosis
L-SVC
Coarctation

Question 15

A Primum ASD is synonymous with?

Answer 15

Partial A-V canal

Question 16

What type of VSD large, or small makes a loud murmur?

Answer 16

Small

Question 17

With a large defect, what type of murmur might you get?

Answer 17

MV diastolic (like MS)

Question 18

What type of murmur do you hear with eisenmenger’s?

Answer 18

None

Question 19

Which VSDs usually close spontaneously?

Answer 19

Muscular and membranous

Question 20

Which VSDs, even if small, need to be closed to prevent AR?

Answer 20

Supracristal (sub aortic or sub pulmonary)

Question 21

Which congenital lesion is associated with maternal rubella?

Answer 21

PDA

Question 22

Physical exam in PDA

Answer 22

Wide pulse pressure
Brisk upstroke
Dynamic LV
machinery murmur

Question 23

F:M incidence of PDA

Answer 23

3:1

Question 24

Class I indications to close PDA

Answer 24

If LV enlarged
PAH if net shunt is L–>R
Endocarditis

Question 25

What syndrome is associated with PS?

Answer 25

Noonan's

Question 26

Physical exam in PS

Answer 26

Soft and late P2 A wave on JVP RV heave

Question 27

If RV dilated with PS, what to do?

Answer 27

Look for an associated lesion

Question 28

In what part of systole will click be if PS is severe?

Answer 28

Early (b/c of RVH)

Question 29

Which R-sided sound decreases with inspiration?

Answer 29

PS click (not murmur)

Question 30

If CXR shows PA sticking out into lung, Dx?

Answer 30

PS (post stenotic dilatation of PA)

Question 31

Class I indications for balloon valvotomy in PS

Answer 31

asymptomatic: domed PV/peak grad > 60/mean grad> 40 Sxs: domed PV/peak grad> 50/ mean grad> 30

Question 32

How and when will complications of ballon valvotomy for PS present?

Answer 32

20y later with arrhythmias, TR, RV enlargement from PR

Question 33

where in Ao does coarctation usually occur?

Answer 33

just distal to subclavian artery

Question 34

what dz is commonly associated with Turner's and bicuspid AV?

Answer 34

Coarctation

Question 35

CXR findings of coarctation

Answer 35

Figure 3 sign, rib notching

Question 36

Histopathology of Ao in coarctation

Answer 36

cystic medial necrosis

Question 37

Class I indications for coarctation repair

Answer 37

P2P gradient >20 | if <20, need imaging showing sig coarct with collaterals

Question 38

why is echo doppler potentially misleading in coarctation?

Answer 38

because the gradient could be lower if good collaterals

Question 39

How do you decrease mortality in coarctation?

Answer 39

surgery early in life

Question 40

what f/u is needed after Dacron patch repair of coarctation?

Answer 40

- mandatory yearly imaging (e.g. MR) to r/o aneurysm at repair site - head imaging to r/o intracranial aneurysm - tight control of BP, CAD risk

Question 41

Inferiorly displaced TV and atrialized RV, Dx?

Answer 41

Ebstein's Anomaly

Question 42

two lesions associated with Ebstein's

Answer 42

ASD/PFO, bypass tract

Question 43

ECG shows RBBB, tall (Himalayan) P waves, prolonged PR, pre-excitation and AF/Afl. Dx?

Answer 43

Ebstein's

Question 44

cyanosis, cool periphery, V wave on JVP, subtle RV lift, Loud T1, holosystolic TR murmur that increases with inspiration. Dx?

Answer 44

Ebstein's

Question 45

CXR showing globular heart (xmas tree ornament) with small Ao and PA. Dx?

Answer 45

Ebstein's

Question 46

5 indications to repair Ebstein's (valve repair, ASD closure, reduce atrialized component of RV)

Answer 46

decreased exercise capacity/RV dysfunction Progressive cyanosis or paradoxical embolism severe TR bypass tract or arrhythmias nope amenable to catheter-based therapy cardiothoracic ratio > 60%

Question 47

Missing tissue in fossa ovalis

Answer 47

Secundum ASD