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Cardiac Defects Flashcards

1
Q

Most common defect in Down’s

A

AV septal defect

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2
Q

Most common defect in DiGeorge

A
  1. Tetralogy of Fallot

2. Truncus arteriosus

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3
Q

DiGeorge Symptoms

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

Tetralogy of Fallot Types

A

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

Cyanotic TOF - more severe RVOT obstruction

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5
Q

Signs and symptoms of Tetralogy of Fallot

A
  • LUSB systolic murmur - PS
  • +/- cyanosis
  • +/- tachypnea = increased BF = increased tachypnea
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6
Q

Treatment of Tetralogy of Fallot

A
  • 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

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7
Q

Tet spells; who they happen in and how to treat

A

Tetralogy of fallot

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

IV line = morphine, saline, ketamine, phenyleprine

Beta-blocker

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8
Q

Types of AV septal defects

A

Partial/Transitional = like ASDs

Complete/Intermediate = like ASD + VSD

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9
Q

Most common defect w/ a diabetic mother

A

Truncus arteriosus

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10
Q

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

A

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

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11
Q

Types of Truncus arteriosus and mortality

A

Moratilty - 5 weeks of age w/out intervention

Depend on pulmonary
BF:

Increased BF
Decreased BF (rare)
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12
Q

Symptoms of increased pulmonary BF in truncus arteriosus

A 
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

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13
Q

Sympotms of decreased pulmonary BF w/ out pulmonary regurgitation

A

Due to PA stenosis or pulmonary vascular disease

Moderate to severe cyanosis

Normal pulses and pulse pressure

Systolic murmur - PA stenosis

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14
Q

Truncus arteriosus common valve abnormalitites

A

3 cusps = most common

Can be stenotic or insufficient

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15
Q

Truncus arteriosus surgery

A

Repair VSD so LV pumps to aorta

RV to PA conduit

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16
Q

Aortic Valve precursor

A

Subendocardial mesenchyme

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17
Q

Effects of aortic valve stenosis

A

Aortic root dilation
Leaflet destruction
Ventricular hypertrophy

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18
Q

Aortic stenosis treatment

A

Maintain PDA
Valvuloplasty
Surgical commissurotomy

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19
Q

Effect of aortic stenosis on fetal circulation

A

Lower blood O2 content to the brain and coronary

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20
Q

Effect of coarctation on fetal circulation

A

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

Born healthy and of normal birth weight

21
Q

How does coartication develop?

A

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
Q

Most common Turner’s syndrome anomalies

A
  1. Bicuspid valve

2. aortic coartication

23
Q

Aortic coartication presentation

A
  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
Q

CXR signs of aortic coartication

A

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

Cardiovascular (12 decks)

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