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Cardiovascular > Cardiac Defects > Flashcards

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
Q

Aortic coartication therapy

A
  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
Q

Treatment of choice for infants w/ aortic coartication

A

Subclavian flap aortoplasty

27
Q

Persistent PDA causes what?

A

Heart failure and poor growth in infants and children

28
Q

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

A

ASD - L to R

PDA - R to L

29
Q

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

A

ASD - R to L

PDA - L to R

30
Q

When is a PDA needed?

A

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
Q

What is transposition?

A

Ventricular-Arterial discordance

32
Q

Gender and transposition

A

M>F

33
Q

Transposition is seen commonly in what infants

A

Infants of diabetic mothers

34
Q

Embryological cause of transposition

A

Abnormal spiraling/conotruncal rotation during septation with aorticopulmonary septum

35
Q

How to create intercirculatory mixing in transposition?

A

Atrial level - ASD

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

Arterial level - PDA (use PGE1)

36
Q

Transposition; associated lesions

A

50% - TGA w/ PFO or PDA

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

37
Q

Transposition w/ VSD treatment

A

Does not require PGE1

38
Q

The effect of transposition on coronary circulation

A

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

39
Q

Clinical features of transposition

A

Early cyanosis

Loud S2 (due to anterior location of aorta)

Hyperoxia - excess blood oxygen; due to separation of circulation

40
Q

CXR appearance of transposition

A

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

Cardiomegaly

41
Q

Management of transposition

A

Intercirculatory mixing

  • Create ASD w/ balloon atrial septostomy
  • PGE1

Oxygen

Volume - IV fluids

Correct metabolic abnormalities

42
Q

What is the Senning operation?

A

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

43
Q

Complications of Senning

A

RV dysfunction, arrhythmias

44
Q

What is the Jatene arterial switch?

A

Transposition surgery

45
Q

Risks of Jatene switch

A

Coronary issues
Aortic root dilation
Aortic insufficiency
PA stenosis

46
Q

VSD - locations

A

Outlet/subarterial (type 1) - near LV outlet

Perimembranous (type 2) - most common

Inlet (type 3)- near RV inlet

Muscular (type 4)

47
Q

VSD complications

A

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
Q

What determines VSD magnitude

A

Size of VSD

Pulmonary vascular resistance

49
Q

VSD survival

A

Depends on VSD size and location and development of pulmonary HTN

Cardiovascular (12 decks)

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