Adult congenital heart disease

Question 1

What defect is associated with orthodeoxia-platypnoea syndrome?

Answer 1

PFO or ASD

Question 2

ASD loads the…

VSD loads the…

Answer 2

right heart

left heart

Question 3

WHere is ostium secundum?

Answer 3

Middle of call near fossa ovalis

can see with TTE

Question 4

Where is ostium secundum?

Answer 4

Low down IA wall near cardiac crux
Often assoc with VSD
Can see with TTE

Question 5

What is an unroofed coronary sinus defect?

Answer 5

Type ASD where coronary sinus drains to LA

need TOE or MR or CT

Question 6

What is a sinus venosus defect?

Answer 6

Near SVC, associated with partial anomolous pulm venous drainage
need TOE or MR or CT

Question 7

When to close an ASD?

Answer 7

Over 1cm or shunt is 1.5 or more
whenever it is not an ostium secundum
before getting PPM put in or increase risk systemic thromboemb

Question 8

ASD gives what murmur?

Answer 8

systolic flow murmur at second left ICS

fixed split S2

Question 9

What are the cyanotics?

Answer 9

Transposition
TAPV connection
Eisenmengers
Tetralogy of fAllot
persistent truncus arteriosus 
PDA later stage
hypoplastic left heart

Question 10

Most common VSD

Answer 10

Perimembranous

Question 11

WHAT IS THE MOST COMMON CONGEN HEART DEFECT AT BIRTH?

Answer 11

VSD

Question 12

What VSD do you see in Down?

Answer 12

Inlet defect (above or below tric valve)

Question 13

When do you fix a VSD?

Answer 13

Vent dysfunction (NOT pressure gradient)
Endocarditis
post MI
Qp:Qs over 2
or ration over 1.5 with LV dysfunction

Question 14

Murmur in VSD

Answer 14

Pansystolic lower sternal border

depends on pressure gradient for volume

Question 15

When not to close VSD?

Answer 15

If there is Eisenmengers!

Question 16

PDA- when to close?

Answer 16

All!
High IE risk
Need cath to check reversibility in severe pulmonary artery hypertension or increased pulmonary artery pressures- ie cannot close if severe PAH

Question 17

Murmur in PDA

Answer 17

Continuous machinery murmur under L clavicle
Bounding pulse
Wide pulse pressure

Question 18

Clubbing feet and not hands?

Answer 18

Eisenmengers in PDA- due to desaturation of blood reaching feet but not hands

Question 19

What is PDA connecting?

Answer 19

Pulmonary artery and aorta

Question 20

What can you hear in PS?

Answer 20

Pulmonary ejection click (lost when severe)
Prominent A wave
RV lifting
RV S4 if severe

Question 21

What is PS associated with (syndrome)?

Answer 21

Noonans

Question 22

Treat PS?

Answer 22

pulm balloon angioplasty if no PR

Fix if peak gradient over 60 or mean over 40 (asympt)
With symptoms if peak over 50 or mean over 30

If fix need lifetime follow up for PR

Question 23

Association between coarctation and…

Answer 23

bicuspid AV (more than half)
Turners

Question 24

coarctation hear…

Answer 24

systolic murmur left infraclavicular region or over back
can be continuous if severe
Ejection click if bicuspid AV
Often S4

Question 25

Most likley to happen to BP post coarctation repair?

Answer 25

Stays high in75%

Question 26

Most common residual feature of Tetrallogy repair?

Answer 26

PR

Question 27

How to manage the polycythaemia in cyanotic heart disease?

Answer 27

If hyperviscosity, can do phlegbotomy to aim Hb 180-200 and haematocrit 60-65. If iron def–>microcytosis–>increase stroke risk so be careful
DO give iron if iron def