Pediatric Obstructive Cardiac Lesions Flashcards

1
Q

Acyanotic heart disease causing CHD

A
  1. left to right shunt lesions: ASD, VSD, AVSD, PDA
  2. obstructive lesions (without septal defects)
    - pulmonnary stenosis,

aortic stenosis

  • coarctation of the aora
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2
Q

cyanotic CHD ped

A
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3
Q

inflow obstruction causes “backward effects”– outline

A
  • systemic venous congestion
  • pulmonary venous congestion and then hypertension
  • increased RV afterload and RV failure
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4
Q

outflow obstruction: forwards effects

A
  • decreased end organ perfusion
  • decreased coronary perfusion, myocardial ischemia
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5
Q

pulmonary stenosis causes __obstruction

A

outflow (in the sense that blood from heart can’t get OUT to the lungs) obstruction. Usually it’s due to valvular misshaping.

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6
Q

pulmonary stenosis symptoms

A
  • fatigue
  • dyspnea (blood having a harder time getting to the lungs)
  • if there is an atrial defect present, R-L shunting may occur, resulting in cyanosis.
  • eventually RV failure may develop with peripheral edema, liver congestion, ascites– cause the right ventricle is oveer workerd.
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7
Q

Pulmonary stenosis physical exam findings

A
  • systolic ejection murmur
  • left upper sternal border
  • crescnedo decrescendo pattern
  • S3 or S4 because of ventricular hypertrophy/rapid filling.
  • RV heave
  • as the stenosis becomes more severe, the systolic mrumur peaks later in systole, with splitting of the S2.

- thrill at the second left intercostal space

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8
Q

heave is a sign of

A

ventricular hypertrophy

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9
Q

thrill is a sign of

A

an incompetent valve

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10
Q

CXR findins of pulmonary stenosis

A
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11
Q

treatment for pulmonary stenosis

A

balloon valvulo plasty

  • if mild, don’t do surgery
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12
Q
A
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13
Q

hypoplastic heart syndrome is a ___ obstruction of the __ heart

A

inflow obstruction of the left heart.

  • ventricle is super small and occluded, cannot allow all the flow of blood in. shunt between atria usually occurs (ASD) to alleivate pressure
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14
Q

LV outflow tract obstruction
includes __ stenosis,
valvar stenosis (bicuspid __
valve), __ stenosis
and __ coarctation

A
LV outflow tract obstruction
includes **subvalvar** stenosis,
valvar stenosis (bicuspid **aortic**
valve), **supravalvar** stenosis
and **aortic** coarctation
  • May be isolated lesion or in
    combination with septal
    defects or conotruncal
    anomalies
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15
Q

aortic stenosis is an ___ obstruction of the Left side of heart

A

left heart outflow obstruction.

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16
Q

most common congential heart defeect

A

aortic stneosis

17
Q

gender most affected by aortic stenosis

A

male 4:1

18
Q

associated diseases of aortic stenosis

A

Associated with aortopathy similar to Marfan
syndrome,
with abnormalities of smooth muscle, extracellular matrix, collagen and elastin, leading to dilatation and aneurysm with risk of rupture

BAV may vary between three leaflets with cusp
fusion (functionally BAV), true BAV, or unicuspid valve

19
Q

aortic stenosis symptoms in infants, children and adolescents

A
  • variable presentation based on age

newborns with severe AS present with DYSPNEA AND LEFT HEART FAILURE

  • majority of children with mild to moderate AS have a systolic murmur and possible S4
  • children and adolescents may have easy fatigability or chest pain. syncope may be a presenting complain in some children with severe AS
20
Q

ohysical exam findings of aortic stenossi

A
  • systolic ejection murmur, crescendo
  • right upper sternal border, radiating to apex and carotids,
  • possible heave (vent hypertrophy), thrill (valvular issue aka the AS itself)
  • parvus et tardus/weak and delayed carotid pulse
  • soft S2 because aortic valves is rigid and close together– can’t slam
  • s4 because of hypertrophy.
21
Q

Aortic Stenosis: CXR

findings

A

prominent right mediastinal border represents dilated ascending aorta

  • cardiomegaly when LVH is present (also would see heaves on exam and sustained apex/dilated apex)
22
Q

aortic stenosis treatment

A
  • balloon valvuloplasty
  • surfical valvuloplasty
  • aortic valve replacement
  • have to consider the possibility of reintervention if first attempt fails
23
Q

subaortic stenosis is an ____ obstructive problem of the left heart

A

is outflow

24
Q

subaortic stenosis cause

A

discrete ring of tissue below the valve.

  • disteinct from hypertrophic cardiomyopathy
  • most commonly an isolated lesion.
  • may be part of a concimteant lesion (VSD or AVSD)
25
Q

Fixed obstruction just above the sinus of
Valsalva, usually above the coronary arteries

A

supravalvar stenosis

26
Q

coarctation of the aorta:

Discrete narrowing in the distal __ arch

Posterior shelf or ridge of tissue extending into aorta just distal to the left __ artery at the __ arteriosum

  • associated with a __ arch, ___ syndrome, ___ arotic balve
  • extensive __ vessels may be present
A

Discrete narrowing in the distal aortic arch

Posterior shelf or ridge of tissue extending into aorta just distal to the left subclavian artery at the ligamentum arteriosum

  • associated with a hypoplastic arch, turners syndrome, bicuspid aortic valve
  • extensive collateral vessels may be present
27
Q
A
28
Q

why might an infant not be immediately symptomatic with coarctation of aorta?

A
  • they are typically dependnt on the PDA to supply their distal aorta
  • when PDA closes later on, there is. a poor distal flow beyond coarctation, acidosis, shock in severe forms.
  • children with less severe obstruction are usually asymptomatic.
  • if symptoms are prsent, usually due to hypertension: headache, epistaxis, dizziness, palpitations, caludication.
  • diagnosis is made during physical exam.
29
Q

symptoms of coarctation of the aorta

A
  • if symptoms are prsent, usually due to hypertension: headache, epistaxis, dizziness, palpitations, caludication.
  • diagnosis is made during physical exam.
30
Q

coarctation of the aorta physical exam

A
  • systemic arterial hypertension in arms with diminished or absent femoral
  • ejection systolic murmur
  • Continuous murmur may be heard in back due
    to collaterals in older patients
31
Q

coarctation chest Xray findings

A

Notching of the underside of the ribs
(usually 3 to 9) from collateral vessels

32
Q

coarctation treatment

A

Coarctation is managed surgically or by catheterization

• Surgical options include resection with end-to-end
anastomosis or subclavian patch aortoplasty

• Balloon angioplasty +/- endovascular stenting is
considered for native coarctation in older children and
adolescents, and in recurrent coarctation

• Similar short- and long-term outcomes as surgery but
increased risk of aneurysm formation and dissection or rupture

• 25-38% patients have residual hypertension

• Late-onset coarctation patients should be screened for
intracranial aneurysms

33
Q

life sided obstruction: heritability

A

• Does not follow simple
Mendelian genetics but
“complex inheritance”
• Heritability of BAV 89%;
10-15% of first degree
relatives will have some
form of LVOTO

34
Q

Critical Outflow Tract Obstruction

Severe obstruction to either the right or left outflow tracts with inadequate forward flow to maintain either pulmonary or systemic circulation respectively

  • Dependent on the __ __ __ to maintain output
A

Severe obstruction to either the right or left outflow tracts with inadequate forward flow to maintain either pulmonary or systemic circulation respectively

  • Dependent on the PATENT DUCTUS ARTERIOSUS to maintain output
35
Q
A
36
Q

coarctation of the aorta is an ___ obsturction issue

A

outflow obstruction

37
Q

critical pulmonary stenosis

____ augments pulmonary blood flow from aorta

  • when it closes, the __ of pulmonary blood flow resulting in severe cyanosis and reduced cardiac output.
A

PDA augments pulmonary blood flow from aorta

  • when it closes, the PDA of pulmonary blood flow resulting in severe cyanosis and reduced cardiac output.
38
Q

T/f critical left outflow tract obstruction causes cyanosis

A

false. PDA augments systemic blood flow via pulmonary artery. when it closes ,there is a loss of systemic blood flow results in cardiogenic shock.
- there is no cyanosis. pulmoanry blood flow usually preserved.

39
Q
A