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Flashcards in Cardiac Defects Deck (49):
1

Most common defect in Down's

AV septal defect

2

Most common defect in DiGeorge

1. Tetralogy of Fallot
2. Truncus arteriosus

3

DiGeorge Symptoms

1. Facial phenotype
2. Developmental delay
3. Parathyroid hypoplasia - hypocalcemia
4. Thyroid hypoplasia - immune deficiency

4

Tetralogy of Fallot Types

Pink TOF - less severe RVOT obstruction = more BF to lungs

Cyanotic TOF - more severe RVOT obstruction

5

Signs and symptoms of Tetralogy of Fallot

-LUSB systolic murmur - PS
-+/- cyanosis
-+/- tachypnea = increased BF = increased tachypnea

6

Treatment of Tetralogy of Fallot

-PGE2 = to maintain PDA and lower BF to lungs

-Surgery = at 3-6 months of age

Ventriculotomy, transannular patch repair, and RV to pulmonary artery circuit

7

Tet spells; who they happen in and how to treat

Tetralogy of fallot

Tx:
First line = calm down, O2, and knee to chest

IV line = morphine, saline, ketamine, phenyleprine

Beta-blocker

8

Types of AV septal defects

Partial/Transitional = like ASDs

Complete/Intermediate = like ASD + VSD

9

Most common defect w/ a diabetic mother

Truncus arteriosus

10

Blood flow in truncus arteriosus; how does it change over time

As pulmonary vascular resistance decreases after birth = more BF to lungs = heart failure and pulmonary overcirculation/HTN

11

Types of Truncus arteriosus and mortality

Moratilty - 5 weeks of age w/out intervention

Depend on pulmonary
BF:

Increased BF
Decreased BF (rare)

12

Symptoms of increased pulmonary BF in truncus arteriosus

Mild cyanosis
Increased pulse pressure
Hyperdynamic precordium
Normal S1 and loud single S2
Pansystolic murmur at LLSB, systolic murmur at USB
Diastolic murm

Tachypnea, hepatomegaly, poor feeding, diaphoresis

13

Sympotms of decreased pulmonary BF w/ out pulmonary regurgitation

Due to PA stenosis or pulmonary vascular disease

Moderate to severe cyanosis

Normal pulses and pulse pressure

Systolic murmur - PA stenosis

14

Truncus arteriosus common valve abnormalitites

3 cusps = most common
Can be stenotic or insufficient

15

Truncus arteriosus surgery

Repair VSD so LV pumps to aorta

RV to PA conduit

16

Aortic Valve precursor

Subendocardial mesenchyme

17

Effects of aortic valve stenosis

Aortic root dilation
Leaflet destruction
Ventricular hypertrophy

18

Aortic stenosis treatment

Maintain PDA
Valvuloplasty
Surgical commissurotomy

19

Effect of aortic stenosis on fetal circulation

Lower blood O2 content to the brain and coronary

20

Effect of coarctation on fetal circulation

Not much effect; babies without an aortic isthmus still get good perfusion to all tissues

Born healthy and of normal birth weight

21

How does coartication develop?

Hemodynamic theory - disturbances cause reduced blood flow and volume in fetal aortic arch

Ductal tissue theory - migration of ductal arteriosus smooth muscle into the aorta = causes narrowing and constrictuion

Genetic causes - Turner's syndrome

22

Most common Turner's syndrome anomalies

1. Bicuspid valve
2. aortic coartication

23

Aortic coartication presentation

1. Infant w/ CHF - complex coarticatoin

2. Child/adolescent w/ systolic hypertension

3. murmur; continous chest murmur due to collaterals, mitral stenosis/VSD murmurs

24

CXR signs of aortic coartication

Aortic knob
Aortic indentation at LSB w/ opposing esophageal indentation (barium swallow)

25

Aortic coartication therapy

1. End to End anastomosis; no circumferential suture = less recurrence

2. Prosthetic patch aortoplasty; high incidence of late aneurysms

3. Subclavian flap aortoplasty; sacrifices left subclavian artery (has growth potential)

4. Angioplasty/stenting

26

Treatment of choice for infants w/ aortic coartication

Subclavian flap aortoplasty

27

Persistent PDA causes what?

Heart failure and poor growth in infants and children

28

Hypoplastic Left Heart Syndrome; what shunts must remain for fetus to be viable

ASD - L to R
PDA - R to L

29

Hypoplastic Right Heart Syndrome; what shunts must remain for fetus to be viable

ASD - R to L
PDA - L to R

30

When is a PDA needed?

Systemic blood flow when there is severe aortic flow obstruction (R to L)

Pulmonary BF when there is severe obstruction to pulmonary flow (L to R)

31

What is transposition?

Ventricular-Arterial discordance

32

Gender and transposition

M>F

33

Transposition is seen commonly in what infants

Infants of diabetic mothers

34

Embryological cause of transposition

Abnormal spiraling/conotruncal rotation during septation with aorticopulmonary septum

35

How to create intercirculatory mixing in transposition?

Atrial level - ASD

Ventricular level - VSD (common in 40-45%)

Arterial level - PDA (use PGE1)

36

Transposition; associated lesions

50% - TGA w/ PFO or PDA

50% - TGA w/ other anomalies = VSD (40-45%), LVOT (5%), coartication

37

Transposition w/ VSD treatment

Does not require PGE1

38

The effect of transposition on coronary circulation

Provides deoxygenated blood to heart; many variants in coronary artery branches - ie circumflex from RCA, single RCA or LCA, etc

39

Clinical features of transposition

Early cyanosis

Loud S2 (due to anterior location of aorta)

Hyperoxia - excess blood oxygen; due to separation of circulation


40

CXR appearance of transposition

"Egg on a string" - egg shaped heart w/ a narrow mediastinum

Cardiomegaly

41

Management of transposition

Intercirculatory mixing
-Create ASD w/ balloon atrial septostomy
-PGE1

Oxygen

Volume - IV fluids

Correct metabolic abnormalities

42

What is the Senning operation?

Surgical correction of transposition- Pulmonary veins into RA, atrial baffle (shunt) for SVC/IVC to LV

43

Complications of Senning

RV dysfunction, arrhythmias

44

What is the Jatene arterial switch?

Transposition surgery

45

Risks of Jatene switch

Coronary issues
Aortic root dilation
Aortic insufficiency
PA stenosis

46

VSD - locations

Outlet/subarterial (type 1) - near LV outlet

Perimembranous (type 2) - most common

Inlet (type 3)- near RV inlet

Muscular (type 4)

47

VSD complications

CHF due to large volume - L to R shunting

Pulmonary HTN leading to shunt reversal - Eisenmenger's syndrome

LV dysfunction due to chronic volume overload

Bacterial endocarditis

LATE:
Aortic regurgitation
Arrhythmias
Double chamber RV

48

What determines VSD magnitude

Size of VSD

Pulmonary vascular resistance

49

VSD survival

Depends on VSD size and location and development of pulmonary HTN