Pediatric Obstructive Cardiac Lesions Flashcards
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
cyanotic CHD ped
inflow obstruction causes “backward effects”– outline
- systemic venous congestion
- pulmonary venous congestion and then hypertension
- increased RV afterload and RV failure
outflow obstruction: forwards effects
- decreased end organ perfusion
- decreased coronary perfusion, myocardial ischemia
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.
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.
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
heave is a sign of
ventricular hypertrophy
thrill is a sign of
an incompetent valve
CXR findins of pulmonary stenosis
treatment for pulmonary stenosis
balloon valvulo plasty
- if mild, don’t do surgery
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
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
aortic stenosis is an ___ obstruction of the Left side of heart
left heart outflow obstruction.
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.
