Congenital Heart Disease 2

Question 0

Pink hands and blue feet. Dx?

Answer 0

Eisenmenger’s with PDA

Question 1

Large shunt (usu VSD)--> VSD--> reverse to R to L shunt w/ cyanosis. Sxs = SOB, Syncope, hemoptysis, stroke
Dx?

Answer 1

Eisenmenger’s

Question 2

Cyanosis/clubbing, a-wave JVP, RV heave, click, diastolic murmur of PR from PA dilation, loud P2. Dx?

Answer 2

Eisenmenger’s

Question 3

Eisenmenger’s CXR

Answer 3

Big “mogul #2” from big PA, calcified PA

Question 4

Drug class to avoid in eisenmenger’s

Answer 4

Vasodilators (no NTG)

Question 5

Rx for eisenmenger’s

Answer 5

PAH Rx: Bosentan, sildenafil, prostacyclin

Question 6

Tet of fallot

Answer 6

• large sub aortic VSD
• RVOT obstruction (+/-PS)
• overriding Ao
• RVH

Question 7

DDR of overriding Ao

Answer 7

TOF, Pulm atresia, truncus

Question 8

Which lesion in TOF determines security of TOF?

Answer 8

RVOT obstruction (b/c blue blood sneaks over from RV to LV and causes cyanosis)

Question 9

Difference between RV and LV pressures in TOF

Answer 9

RVp = LVp

Question 10

Name 3 associated lesions with TOF

Answer 10

• R- Ao arch on CXR
• anomalous coronary artery
• Secundum ASD

Question 11

TOF PE

Answer 11

Cyanosis
RV lift
Thrill if PS severe
Systolic murmur over pulmonic area
ABSENT P2

Question 12

Most common problem after TOF repair

Answer 12

PR–> RV inc–> inc QRS–> VT–> SCD

Question 13

EKG findings in TOF

Answer 13

RBBB

Wide QRS

Question 14

What is the repair for TOF?

Answer 14

RVOT patch
Close VSD
Excise PV

Question 15

What is next step if pt with TOF repair comes in with arrhythmia ?

Answer 15

Look for Hemodynamic abnormality (most likely PR which should be promptly corrected with PVR)

Question 16

What is the purpose of creating a shunt for congenital heart diseases?

Answer 16

Increase blood flow through pulmonary vasculature

Question 17

Glenn

Answer 17

SVC–> RPA

Question 18

Bidirectional Glenn

Answer 18

SVC to RPA and LPA

Question 19

Blalock-Tausigg

Answer 19

Subclavian Artery–> PA

Question 20

Waterston shunt

Answer 20

Asc Ao–>RPA

Question 21

Potts Shunt

Answer 21

Desc Ao–> LPA

Question 22

Complications of creating shunts for congenital heart disease

Answer 22

Distortion of PA
Large shunt= Pulm HTN and LV overload
No heart/lung Tx if Lateral thoracotomy

Question 23

When do you consider phlebotomy for a patient with cyanosis HD?

Answer 23

Hgb> 20 , Hct > 65

Question 24

If pt has iron deficiency and cyanotic CHD, what are they at risk for?

Answer 24

Stroke

Question 25

What do you do if pt has sxs of hyperviscosity and Hct> 65? <65?

Answer 25

Hydrate | Suspect iron def, give gentle iron suppl.

Question 26

How much should you phlebotomize a pt with cyanotic CHD?

Answer 26

Max 1 u

Question 27

Are cyanotic CHD pt's at risk for thromboemboli or bleeding?

Answer 27

Bleeding

Question 28

What should be done for all IV lines attached to cyanotic CHD pt's?

Answer 28

Place air filters to avoid air emboli

Question 29

Name a spinal problem that cyanotic CHD pt's get and can you do Sx?

Answer 29

Scoliosis | No- unacceptable bleed risk

Question 30

Name a rheumatologic dz that cyanotic CHD patients get

Answer 30

Gout

Question 31

Mortality rate for patients with cyanotic CHD who get pregnant

Answer 31

50%

Question 32

D-loop

Answer 32

RV on R | Ao- anterior and to R

Question 33

L-loop

Answer 33

RV on Left | Ao- anterior and to left

Question 34

What is an arterial switch and what dz do you use it for?

Answer 34

TGA | just switch arteries back to correct ventricles

Question 35

Main step in Mustard procedure for TGA

Answer 35

Take blue blood from SVC/IVC into LA

Question 36

Long term complication of Mustard procedure for TGA

Answer 36

RV Failure needing OHT

Question 37

Name the lesion in L-TGA

Answer 37

RV and LV switched

Question 38

Echo for L-TGA

Answer 38

TV is on Left. And as always apically displaced

Question 39

What is L-TGA associated with?

Answer 39

VSD, PS, TR, CHB, RV Dysfxn

Question 40

L-TGA ECG

Answer 40

Q waves in II, III, aVF | AVB

Question 41

3 types of single ventricle, atrioventricular connections

Answer 41

- double inlet - single inlet (tricuspid atresia) - common inlet (one AV valve)

Question 42

What lesion must a single inlet ventricle have to survive?

Answer 42

ASD

Question 43

Rx for single ventricle?

Answer 43

Fontan

Question 44

What is goal of very contain procedure?

Answer 44

To separate blue and red blood

Question 45

What arrhythmias are our tan patients prone to? Why?

Answer 45

Atrial | Inc RA size

Question 46

What is the major complication of increased RA size in fontan?

Answer 46

RA Thrombus--> PE

Question 47

What to remember about treating atrial arrhythmias in fontan patients?

Answer 47

Always r/o clot with TEE first

Question 48

Fontan pt comes in with pleural effusion, ascites, diarrhea, JVD. Suspected Dx and next tests?

Answer 48

Protein Losing Enteropathy | Serum albumin, Upr, *STOOL alpha1 AT*

Question 49

Rx for protein losing Enteropathy after fontan

Answer 49

Repair fontan obstruction

Question 50

Pathophysiology of protein losing Enteropathy in fontan. Mortality rate?

Answer 50

Inc'd systemic venous P--> inc'd thoracic duct P | 50% at 5yrs

Question 51

Another name for congenitally corrected transposition

Answer 51

L-TGA