Congenital Heart Defects - Exam 1 Flashcards
Which congenital heart defects are considered acyanotic?
ASD
VSD
PDA
PV Stenosis
Coarctation of the Aorta
Aortic Stenosis
Which congenital heart defects are considered cyanotic?
Transposition of the great arteries
Tetralogy of Fallot
Hypoplastic left heart syndrome
What 3 Con HD all cause Left to Right shunting? What does it cause? What can it lead to over time?
ASD
PDA
VSD
oxygenated blood flows redundantly through pulmonary circulation
may become Eisenmenger syndrome over time
What is Eisenmenger Syndrome?
infants with exertion present with cyanosis, palpitations dyspnea, chest pain, syncope
What 3 Con HD are due from an outflow obstruction?
Pulmonary stenosis
aortic stenosis
coarctation of aorta
What is a atrial septal defect?
A hole in the heart wall (muscle) dividing left/right atria (left-to-right shunt)
Fixed, split S2 and pulmonic ejection murmur that increases with age
What am I?
How will it present in children?
atrial septal defect
Respiratory infections, Failure to thrive
How does a normal atrial septum develop? What develops as a result?
the septum primum grows from the top down towards the endocardial cushion but does not connect all the way
leads a hole called the ostium primum “first opening”
What is considered a small con HD?
LESS than 5mm
Where is the MC place for a Con HD to occur? What is it associated with?
at the ostium secundum
fetal alcohol syndrome
______ is considered a endocardial cushion defect and is associated with _____
ostium primum
25% of Down’s Syndrome
How do you dx atrial septal defects?
echo: will show right heart dilation with prominent pulm vascularity
If a right heart cath is performed in atrial septal defect what will it show?
increased oxygen saturation in the right atrium, right ventricle and pulmonary artery
What is the tx for ASD in a In the asymptomatic child with a large hemodynamically “significant” defect? Why?
Closure is performed electively at 1-3 yrs
before late complications of RV dysfunction and dysrhythmias occur.
What is the tx for ASD in a moderate to large defect? What size is unlikely to close on their own?
when the child is between 4 - 6 years of age
defects >8 mm unlikely to close on their own
What will ventricular septal defect present like? Which one is the major one? What is happening?
Heart failure
Pulmonary HTN
Arrhythmias: usually SVT
Stroke
more blood is being pumped into the lungs and pulmonary artery causing 4 things mentioned above
How does the ventricular septum grow normally?
membraneous region grows downward and the muscular ridge grows upward
What are the 3 main types of vent septal defects? Which one is MC?
Membranous - upper septum (most common)**
Muscular - lower septum
Inlet - in the posterior portion of the septum beneath the TV
______ is the MC con HD and account for 25%. What is considered a small-moderate one? Will they have symptoms?
vent septal defect
3-6mmg
usually asymptomatic
Small - moderate VSD ____ are usually asymptomatic and ____ will close spontaneously by age____
: 3-6mm
50%
2yrs.
What is the tx for moderate-large vent septal defect? Will they have symptoms? When do they present?
surgical repair
almost always have symptoms
larger ones will present earlier when compared to smaller VSD
What is the pressure like in the Right ventricle of a pt with vent septal defect?
increased blood volume in Right ventricle leads to higher pressure and pulmonary hypertension
Holosytolic murmur located at the lower left sternal border
dypsnea
cough
What am I?
What will a smaller defect sound like when compared to a larger one?
vent sept defect
smaller defect will be a LOUDER murmer
What is Eisenmerger syndrome?
when the pressure gets high enough in the kiddos heart that the shunt reverses and becomes a RIGHT to LEFT shunt
_______ is hard to pick up on echo and will ALWAYS have to have sx to fix it
infindibular VSD
What is the tx for small Vent sept def? What is symptomatic?
most small VSD will close on their own
**treat the symptoms : diuretics if CHF, higher calorie feeds
What is the tx for larger vent septal defects? What age? Why is it important?
Repair larger shunts by age 2 to prevent pulmonary hypertension with PATCH closure
What is patient ductus arteriosus? When does it normally close? How common is it?
Persistence of the normal fetal vessel that joins the pulmonary artery to the Aorta
Normally closes in the 1st wk of life
10% of all congenital heart defects especially in preterm infants, more common in females
What is the purple structure? What does it become after birth?
patent ductus arteriosus
becomes ligamentum arteriosum
holosystolic “machine-like” murmur in neonates
continuous murmur and can lead to exercise intolerance
wide systemic pulse pressure
What am I?
What do larger ones lead to?
patent ductus arterious
larger ones lead to heart failure
smaller ones can be asymptomatic
What is the tx for Eisenmenger syndrome?
need complete heart and lung transplant
What happens to a patent ductus arterious if it is not caught until later in life?
leads to a RIGHT to left shunt
increased pulmonary volume, pulm HTN and very high pressures
How do you dx PDA?
echo with doppler and will see the color flow
What is the tx for small asymptomatic PDA?
monitor, usually if small, will close by itself
**What is the tx for PDA in neonates?
IV Indomethacin to close PDA
What is the drug class of Indomethacin?
prostaglandin inhibitor
What is the tx for a symptomatic moderate/large PDA that is causing heart failure?
digoxin increases contractility, furosemide decreased excess fluid
and surgical ligation
What are the 3 different versions of pulmonary stenosis? Which one is MC?
stenosis of the valve itself
thickening or fusing of the valve- MC
Thickened muscle below valve causes stenosis of the pulmonary artery below valve
What is microangiopathic hemolytic anemia?
fragmentation of the RBC due to damage when it flows through the stenotic pulm valve
How will pulm stenosis present? What is the obstruction is severe?
increased work by the right ventricle and it will thicken overtime leading to pressure overload and cause RVH
increased pressure causes Eisenmenger syndrome
What is the tx in neonates with critical pulm stenosis?
prostaglandin is given at the time of birth to keep the PDA open
Systolic ejection murmur at the left upper sternal border which increases with inspiration
S₂ usually followed by an opening click that becomes louder with expiration
RV lift on palpation of the precordium
What am I?
pulmonary stenosis
CXR that shows normal heat size with post stenotic dilation of the main pulmonary artery and the left pulmonary artery. What are you thinking? ____ confirms the diagnosis
pulm stenosis
echo
What is the tx for pulmonary stenosis at each severity level? mild/mod, mod/severe?
mild/mod: no intervention, monitor
mod/severe: Percutaneous balloon valvuloplasty
Why is Percutaneous balloon valvuloplasty preferred in pulmonary stenosis?
effective at relieving obstruction and causes LESS valve insuffieciency
What is coarctation of the aorta? What part specifically? How common is it?
part of the aortic arch that is narrower than usually causing blockage of the blood flow to the body thus causing backing of blood flow into the LEFT ventricle
proximal descending aorta
6% of con HD
Is coarctation of the aorta more common in males or females? What syndrome?
more common in MALES
20% females have Turner syndrome
What valve is more likely to become involved in a pt with coarctation of the aorta?
bicuspid aortic valve is involved 80-85% of the time
**_________ is the leading cause of heart failure in the first month of life
COARCTATION OF THE AORTA
How is blood distributed differently if there is a coarctation of the aorta present? Specifically what organ? What happens as a result?
Blood flow increases into aortic branches before coarctation—> blood flow, pressure increases in upper extremities and head
Decreased blood flow downstream of narrowing, decreased pressure in lower extremities
Kidneys receive less blood —> activate renin-angiotensin-aldosterone system (RAAS) —>
secondary hypertension
What is a good PE test to help you determine coarctation of the aorta?
check pulses and the brachial pulses will be much stronger than the femoral pulses
preductal coarctation is associated with ____ and _____. When is postductal likely to present?
Turner syndromea and PDA
presents in adulthood with BP being higher upstream than downstream
Postductal coarctation is consider distal to ______
ligamentum arteriosum
in adult coarctation the increased pressure in the upper extremities/head has an increased risk of _____ and ______
berry aneurysms
aortic dissection
What do the s/s of coarctation of the aorta depend on?
presence/severity of PDA
Systole diamond-shaped murmur
Diastole high-pitched decrescendo murmur
absent or delayed femoral pulses
BP higher in upper extremities when compared to lower extremities
What am I?
What is likely to develop as the pt ages?
coarctation of aorta
secondary hypertension, especially when PDA closes
What 3 things need to be ordered for coarctation of aorta?
angiogram
CXR
echo
What will the CXR show for coarctation of aorta?
Rib notching: 3-sign (narrowed aorta resembles notch of number 3 due to prestenotic of aortic arch & postenotic of descending aorta dilatation)
What medication is used in coarctation of aorta? Why?
Prostaglandin E
increases flow to lower extremities
What is the sx tx for coarctation of the aorta? What if it is long?
Resection with end-to-end anastomosis or bypass graft
subclavian aortoplasty
consider balloon angioplasty with possible stent (usually done with pt is younger)
_____ is rare in coarctation of the aorta before adolescence but can occur in both repaired and unrepaired coarctation
infective endocarditis
aka bacteria really like the narrowed part of the aorta
What must kids do before getting cleared for sports with coarctation of the aorta?
Exercise testing is mandatory for these children prior to their participation in athletic activities
aka can do low level activities
What are the 3 types of aortic stenosis? When does severe heart failure occur?
valvular
subvalvular
supravalvular
Severe heart failure occasionally occurs when critical obstruction is present at birth
In aortic stenosis where is the obstruction occuring? How common is it? MC in males or females?
Obstruction of the outflow from the left ventricle at or near the aortic valve
7% of Con HD
MC in males
Crescendo-decrescendo murmur with a click
Harsh, systolic ejection murmur with radiation to the neck
Systolic ejection click at the apex
What am I?
What will the CXR show?
aortic stenosis
dilation of the ascending aorta
Use ____ to dx aortic stenosis. When would you want to perform a heart cath on these patients?
Catheterization reserved for patients whose resting gradient has reached 60 - 80 mmHg and in whom intervention is planned
What is the tx for VALVULAR aortic stenosis? SUBvalvular or SUPRAvavular?
Valvular aortic stenosis: Percutaneous balloon valvuloplasty is the standard INITIAL treatment
Subvalvular or supravalvular aortic stenosis: interventional cath is not effective; surgery is required!!
What is the Ross procedure? When is it used?
The Ross procedure is an alternative to mechanical valve replacement in infants and children.
The patientʼs own pulm valve is moved to the aortic position, and a RV-to-pulmonary artery conduit (donor graft) is used to replace the pulm valve
aortic stenosis prognosis tends to be _______. What is the prognosis for a mild/mod obstruction? What is the prognosis for severe obstruction?
progressive
Mild - moderate obstruction: normal O₂ consumption and maximum exercise capacity. Children with normal resting and exercise (stress) EKGs may safely participate in vigorous physical activity, including non-isometric competitive sports
Severe obstruction: predisposed to ventricular dysrhythmias and refrain from vigorous activity; avoid all isometric exercise
What does the presence of cyanosis indicate? What O2 stat does it correlate with?
babies extremities are not getting oxygenated
Presence > 3 g/dl deoxy HgB correlates with 80-85% SpO2.
______ is used in suspected cyanotic disease when an echo is available. What can it tell you?
hyperoxia test
Differentiates cardiac and non-cardiac causes of cyanosis
If hyperoxia test is _____ = no structural cyanotic heart disease. If < 100 mmHg what does it indicate? 100-250 mmHg?
PaO2 >250
< 100 intracardiac shunting most likely due to a ductal dependent lesion
100-250 mmHg - intracardiac mixing lesions
How do you perform hyperoxia test?
ABG at baseline then give 100% oxygen for 10 minutes then check ABG again
What does a hyperoxia test suggest if the PaO2 is greater than 100mm/Hg?
suggest an underlying lung disease
What is the CCHD test? How old does the newborn have to be in order to be accurate?
CYANOTIC CONGENITAL HEART DISEASE
infant must be older than 24 hours to be accurate
What does the CCHD screening test consist of? What does passing result look like?
What is the tetralogy of fallot consist of?
- Pulmonary stenosis (PS)
- Large VSD
- Overriding aorta: aka big ass aorta
4.Right ventricular hypertrophy (RVH)
How common is tetralogy of fallot? What chromosome is effected?
10% of Con HD
chromosome 22 deletions and DiGeorge Syndrome
**_____ is the MC cause of CYANOSIS in infancy/childhood
**tetralogy of fallot
What does pulm stenosis in TOF result in? RVH?
pulm stenosis: harder for deoxygenated blood to get into pulm circulation
RVH: myocardium has to contract harder to push blood past stenotic valve
**Boot shaped heart, what should you instantly be thinking?
RVH associated with tetralogy of fallot
Kids with TOF will sometimes exhibit ______. What can cause them?
TET spell
Decreased SVR due to hot bath, fever, exercise, stress, prolonged crying, straining for BM
What is the management for a TET spell? What is the underlying process?
want to kid to SQUAT down to increase vascular resistance in peripheral arteries which increases pressure in systemic circulation which increases pressure in Left > RIght side and the shunt temporarily reverses
keep them calm (consider morphine), give O2, IV fluids, propanolol, bicarb if metabolic acidosis and phenylephrine to increase SVR
What is the surgical tx for TOF? What age?
complete repair via open heart surgery
before 2 years old
What is transposition of the great arteries?
The two main arteries carrying blood out of the heart - pulmonary artery and aorta, are switched in position (transposed)
Aorta comes off RV
PA comes off LV
What are the 2 different levels of severity for TOTGA? Which one typically presents later in life?
d-TGA: dextro-TGA/complete TGA (dextro = aorta on right)
1-TGA: levo-TGA/congenitally corrected TGA (levo = aorta on left) - is less common and presents later on with symptoms
Draw the figure of D-TOTGA
D-TOTGA is a death sentences unless pulm and systemic system mix
Draw L-TOTGA
**______ is the second MC cyanotic Con HD? More common in males or females?
TOTHGA
more common in males
TOTGA: The majority of mixing occurs at the (atrial/ventricle) level so an _______ is critically important. Without this, patient will be severely cyanotic at birth
atrial
interatrial communication (PFO or ASD)
What are maternal risk factors for TOTGA?
uncontrolled Diabetes
Rubella in the first trimester
Poor nutrition
Consumption of alcohol
> 40 years old
–>professor also said drug users in class
How will TOTGA present in utero? How will D-TGA present once born? I-TGA?
asymptomatic
D: Cyanosis unchanged with supplemental oxygen (less severe if VSD present), tachypnea, and acidosis
I: asymptomatic
What is the characteristic CXR finding associated with TOTGA?
Heart appears as egg on its side/”egg on a string” appearance
Pulmonary congestion
Cardiomegaly
What is the medication management for TGA? **Sx management? give both short term and long term solutions
prostaglandin E to keep the duct arteriosus open
Balloon atrial septostomy -> short-term solution. Hole created in atrial septum
**Surgically switch great vessels -> long term solution
What is the underlying cause of hypoplastic left heart syndrome? What does it lead to?
Underdeveloped left ventricle & ascending aorta
Aortic/mitral valves may also be affected - narrow or absent
If untreated: left-sided heart failure >cardiogenic shock > death
In patients with hypoplastic left heart syndrome they need _____ and _____ in order to be capable of sustaining life outside of the womb
ASD and PDA
What is happening in HLHS?
Right heart functions normally > oxygenated blood enters left atrium > flow backs up due to small mitral valve, small left ventricle > high pressure in left atrium and blood circulated ineffectively by left ventricle
aka not enough muscle
In HLHS and ASD/PDA are present, what will it present like at birth? At 1 day old?
right heart function present but impaired; sometimes asymptomatic at birth
Within one day: ductus arteriosus begins closing and the kiddo will become cyanotic
cyanosis
LEFT sided HF
respiratory distress
cardiogenic shock
What am I?
What will the CXR show?
HLHS
cardiomegaly on RIGHT side, LEFT side is tiny
What will an EKG show on a pt with HLHS?
Right ventricular hypertrophy/RAD after birth
What is the medication tx for HLHS? What is the surgical tx?
medication: prostaglandin E
sx:
Describe in detail the surgical procedures for tx of HLHS?
Can kid with HLHS participate in normal activites?
Children who undergo surgical repair can participate in recreational activities but are restricted from competitive and vigorous athletics
aka nothing that would increase the pressure in their heart
Presents in the first few days of life
LLSB without radiation
soft SEM
What am I?
When does it resolve?
newborn murmur
resolves by 1 month
**most common innocent murmur of childhood
2-7 years old
heard at the apex and LLSB
vibratory, soft systolic
What am I?
When does it get louder?
Still’s murmur
loud with supine and disappears with inspiration or sitting
also gets louder with anemia and fever
What are the 6 common innocent murmurs of childhood?
newborn murmur
peripheral pulmonary artery stenosis
still’s murmur
pulmonary ejection murmur
venous hum
neck/supraclavicular “carotid bruit”: harsh sounding
