Congenital Heart Disease 2 (Mayo) Flashcards

1
Q

Pink hands and blue feet. Dx?

A

Eisenmenger’s with PDA

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2
Q

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

A

Eisenmenger’s

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3
Q

Eisenmenger’s CXR

A

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

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4
Q

Drug class to avoid in eisenmenger’s

A

Vasodilators (no NTG)

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5
Q

Rx for eisenmenger’s

A

PAH Rx: Bosentan, sildenafil, prostacyclin

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6
Q

Tet of fallot

A
  • large sub aortic VSD
  • RVOT obstruction (+/-PS)
  • overriding Ao
  • RVH
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7
Q

DDR of overriding Ao

A

TOF, Pulm atresia, truncus

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8
Q

Which lesion in TOF determines security of TOF?

A

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

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9
Q

Difference between RV and LV pressures in TOF

A

RVp = LVp

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10
Q

Name 3 associated lesions with TOF

A
  • R- Ao arch on CXR
  • anomalous coronary artery
  • Secundum ASD
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11
Q

TOF PE

A
Cyanosis
RV lift
Thrill if PS severe
Systolic murmur over pulmonic area
ABSENT P2
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12
Q

Most common problem after TOF repair

A

PR–> RV inc–> inc QRS–> VT–> SCD

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13
Q

EKG findings in TOF

A

RBBB

Wide QRS

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14
Q

What is the repair for TOF?

A

RVOT patch
Close VSD
Excise PV

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15
Q

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

A

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

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16
Q

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

A

Increase blood flow through pulmonary vasculature

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17
Q

Glenn

A

SVC–> RPA

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18
Q

Bidirectional Glenn

A

SVC to RPA and LPA

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19
Q

Blalock-Tausigg

A

Subclavian Artery–> PA

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20
Q

Waterston shunt

A

Asc Ao–>RPA

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21
Q

Potts Shunt

A

Desc Ao–> LPA

22
Q

Complications of creating shunts for congenital heart disease

A

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

23
Q

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

A

Hgb> 20 , Hct > 65

24
Q

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

A

Stroke

25
Q

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

A

Hydrate

Suspect iron def, give gentle iron suppl.

26
Q

How much should you phlebotomize a pt with cyanotic CHD?

A

Max 1 u

27
Q

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

A

Bleeding

28
Q

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

A

Place air filters to avoid air emboli

29
Q

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

A

Scoliosis

No- unacceptable bleed risk

30
Q

Name a rheumatologic dz that cyanotic CHD patients get

A

Gout

31
Q

Mortality rate for patients with cyanotic CHD who get pregnant

A

50%

32
Q

D-loop

A

RV on R

Ao- anterior and to R

33
Q

L-loop

A

RV on Left

Ao- anterior and to left

34
Q

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

A

TGA

just switch arteries back to correct ventricles

35
Q

Main step in Mustard procedure for TGA

A

Take blue blood from SVC/IVC into LA

36
Q

Long term complication of Mustard procedure for TGA

A

RV Failure needing OHT

37
Q

Name the lesion in L-TGA

A

RV and LV switched

38
Q

Echo for L-TGA

A

TV is on Left. And as always apically displaced

39
Q

What is L-TGA associated with?

A

VSD, PS, TR, CHB, RV Dysfxn

40
Q

L-TGA ECG

A

Q waves in II, III, aVF

AVB

41
Q

3 types of single ventricle, atrioventricular connections

A
  • double inlet
  • single inlet (tricuspid atresia)
  • common inlet (one AV valve)
42
Q

What lesion must a single inlet ventricle have to survive?

A

ASD

43
Q

Rx for single ventricle?

A

Fontan

44
Q

What is goal of very contain procedure?

A

To separate blue and red blood

45
Q

What arrhythmias are our tan patients prone to? Why?

A

Atrial

Inc RA size

46
Q

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

A

RA Thrombus–> PE

47
Q

What to remember about treating atrial arrhythmias in fontan patients?

A

Always r/o clot with TEE first

48
Q

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

A

Protein Losing Enteropathy

Serum albumin, Upr, STOOL alpha1 AT

49
Q

Rx for protein losing Enteropathy after fontan

A

Repair fontan obstruction

50
Q

Pathophysiology of protein losing Enteropathy in fontan. Mortality rate?

A

Inc’d systemic venous P–> inc’d thoracic duct P

50% at 5yrs

51
Q

Another name for congenitally corrected transposition

A

L-TGA

52
Q
Large shunt (usu VSD)--> VSD--> reverse to R to L shunt w/ cyanosis. Sxs = SOB, Syncope, hemoptysis, stroke
Dx?
A

Eisenmenger’s