Pediatric Obstructive Cardiac Lesions Flashcards by Bronwyn Delacruz

Acyanotic heart disease causing CHD

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

aortic stenosis

coarctation of the aora

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cyanotic CHD ped

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inflow obstruction causes “backward effects”– outline

systemic venous congestion
pulmonary venous congestion and then hypertension
increased RV afterload and RV failure

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outflow obstruction: forwards effects

decreased end organ perfusion
decreased coronary perfusion, myocardial ischemia

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pulmonary stenosis causes __obstruction

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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pulmonary stenosis symptoms

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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Pulmonary stenosis physical exam findings

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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heave is a sign of

ventricular hypertrophy

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thrill is a sign of

an incompetent valve

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CXR findins of pulmonary stenosis

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treatment for pulmonary stenosis

balloon valvulo plasty

if mild, don’t do surgery

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hypoplastic heart syndrome is a ___ obstruction of the __ heart

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

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

left heart outflow obstruction.

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most common congential heart defeect

aortic stneosis

gender most affected by aortic stenosis

male 4:1

associated diseases of aortic stenosis

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

aortic stenosis symptoms in infants, children and adolescents

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

ohysical exam findings of aortic stenossi

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.

Aortic Stenosis: CXR

findings

prominent right mediastinal border represents dilated ascending aorta

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

aortic stenosis treatment

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

subaortic stenosis is an ____ obstructive problem of the left heart

is outflow

subaortic stenosis cause

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)

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

supravalvar stenosis

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

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

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

- 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.

symptoms of coarctation of the aorta

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

coarctation of the aorta physical exam

- 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

coarctation chest Xray findings

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

coarctation treatment

• **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

life sided obstruction: heritability

• 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

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

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

coarctation of the aorta is an ___ obsturction issue

outflow obstruction

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.

PDA augments pulmonary blood flow from aorta - when it closes, the PDA of pulmonary blood flow resulting in severe cyanosis and reduced cardiac output.

T/f critical left outflow tract obstruction causes cyanosis

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.