Congenital Heart Disease Flashcards
Pediatric Functional Murmurs
what are they
characteristics of the sound
Pediatric Functional Murmurs
- innocent, functional and physiologic murmurs which are non-pathological, a result of blood moving through the chambers
Characteristics
- soft, postional-dependent & often occurring in systole
The 7 S’s of innocent murmurs
- sensitive: change with position
- sort in duration
- single (no clicks or gallops)
- small
- soft: low amplutude
- sweet: non harsh
- systolic
a diastolic murmur in children is ALWAYS pathologic
Types
- Stills Murmur
- Venous Hum
- Pulmonary Ejection
Pediatric Murmurs: Still’s Murmur
- what is it
- chacteristics of it
Still’s Murmur: a functional pediatric murmur : most common innocent murmur
- comonly heard @ 2 years old
Characterisitcs
- systolic murmur, early-mid systole
- musical, vibratory with twanged high-pitch sound
- due to vibration of the leaflets
Changes
- will decrease in sound with sitting, standing or valsalva
- increase sound with fever or laying flat supine
Pediatric Murmurs: Venous Hum
- what is it
- chacteristics of it
Venous Hum
- the second most common innocent murmur
- a result of the sound of blood flowing from the
head and neck to the hear tvia the jugualr vein
Can be Graded as I or II
Charactersitics
- harsh sound
- systolic murmur: but can be continuous (into diastole = the only one that can and be nonpathologic!!)
- found at the upper right/left sternal boarder; infraclavcular
Increased sound with…
- upright or sitting with head extended
Decreased sound with
- valsalva, gentle pressure on jugular veins, supine position or head turned to contrlateral side
Pediatric Murmurs: Pulmonary Ejection Murmur
- what is it
- chacteristics of it
Pulmonary Ejection Murmurs
- due to blood flow across the pulmonary valve into the pulmonary artery
- common to see in older children and teens
Characteristics
- best heard mid-systole in the second-left ICS or superior aspect of left lower sternal boarder
- harsh quality
Congenital Heart Disease
definitions
etiology
CHD: a structural cardiac malformation that is present from birth
- the most common malformation of utero & the most common cause of neonatal death
- most babies with CHD will need surgery within the first year of life
Etiology
- largely unknonw etiology
- chromosomal abnormalities are assocaited: deletions, trisomy, etc.
- maternal disease: DM, rubella
- expsoures: alcohol
- if mom has it: risk of baby having it is increased
Genetics
- trisomy 13,18 and 21
- 22q.11
- turners
- noonan
- screen for CHD with genetic issues, and screen for gentic issues if CHD found
Defects in Morphology
- cardiac morphlogy: cardiac tube is created by 7 weeks
- defects reflect an error in the morphology of the heart in utero
Fetal Circulation
what are the three critial structures
how does blood flow
Critical Structures
- ductus venosus
- foramen ovale
- ductus arteriosus
Flow
- umbilical vein from placenta (mom) carries oxygenated blood to the baby
- umbilical vein connects to the fetal portal system of the liver; direcly connecting the umbilicla vein to the dutus venosus
- from the ductus venousus: travels up the IVC and to the heart
- from the IVC into the RA
- in teh RA: 2/3 of the oxygenated blood is shunted from the right A to the left A via the foramen ovale
- this oxygenated blood is now in the LA and transported out to the body
- the remaining 1/3 of oxygenated blood passes to teh right ventricle & goes to the pulmonary artery
the formane ovale is the key reason that oxygenated blood is able to get to the brain inutero
- the blood which was shunted to the pulmonary artery will not go into the lungs, but will pass through the ductus arteriosus into the aorta
- this connects just pass where teh great vessels off the aortic arch are: thus this blood (1/3) is responsible for supply the rest of the body
the way in which teh ductus arteriosis remains open is by a constant presence of prostoglandins
Role of PVR and the closure of the ductus arteriosus
PVR: pulmonary vascular resistance
- the resistance to blood flow from the pulmonary artery to the left atrium
- the PVR is high in utero : as there is fluid in the lungs = thus blood wont flow here
- when baby is born: fluid gone: PVR DROPS so blood begins to flow here
PVR: is lowest at 4-6 weeks
Closure of the Ductus arteriosis
- blod flow from the pulmonary arteries to the lungs begins and gas exchange occurs
- this results in an increase in blood returning to the left atrium from the pulmonary curcit
- Drop in prostoglains = closure of teh PDA
BLOOD IS LAZY: flows from high to low
Acyanotic Congenital Heart Diseases
left to right shunts
4 different ones
Left to Right Shunts
- take areas of high pressure and flow to areas of low pressure
- because the aorta is high pressure, so if there is a path of least resistance, the flow will probably want to go that way
Conditions
- Atrial Septal Defect (ASD)
- Ventricular Septal Defect (VSD)
- Atriventricualr Canal (AV Canal)
- Patent Ductus Arteriosus (PDA)
Symptoms of all congenital heart diseases in children
Commonly….
- poor weight gain
- poor feeding, decreased PO intake
- lethargy
- diphoresis with feeds
Explain the physiology behind Left to Right Shunts
Left to Right
- the blood will flow from high to low pressure
- the left heart is high pressure: thus if there is way to flow to low, the blood will go
- PVR is less than SVR : thus, when it can, itll go into the pulmonary circuit again and again
- this increased the flow to the lungs, (Qp) relative to the systemic flow (Qs) thuse Qp > Qs (so the ratio is >1)
with increased flow to the pulmonary circuit, consisntely recyling back to the pulmonary arteries and to the lungs, there is overload of the cardiac chambers as the blood just keeps coming back and through
tacypena, pulmonary edema and failure to thirve
Atrial Septal Defects
pathology
Ostium locations
Atrial Septal Defect (ASD)
second most common defect
Pathology
- a hole in the atrial septum walls: open flow from left to right (high –> low) (can be multiple locations of the hole)
- thus, blood will enter the left atrium from the pulmonary veins, oxygenated, and then flow directly into the right atrium and recylce back to the lungs again
Result: volume overload delivered to the RA from the LA, leading to pulmonary overcirualtion = congestive heart failure
Where is the Ostium
- ostium secundum = MC
- Ostium Primum = (right with the VS and AS come together) associated with trisomy 21
- sinus venous = at level of coranary sinuse
Atrial Septal Defects
Symptoms
Symptoms
- most pt. are asymptomatic or minimal symptoms under over the age of 30
Infants
- recurrent respiratory infections
- failure to thrive
- exertional dyspnea
Adolecnts and Adults
- easy fatigue
- dyspnea
- atrial arrythmias
- syncope
these pt. are at an increased risk for stroke since there is increase blood flowing through the atrium, increased risk of thrombus formation and it can easily pass from venous, to left herat and to brain
Atrial Septal Defects
PE finiding
Murmur
Imaging & Diagnositcs
PE Findings
- FIXED, WIDELY SPLIT second heart sound (S2) : not changing with respiration, heard due to the difference between atria in pressure
- systolic ejection cresendo-decresendo murmur in pulmonic area
- diastlic rumble in tricuspid area
Imaging & Diagnostics
CXR: cardiomegaly
EKG: incompltete RBB, Right heart increased size Crochetage sign: nothcing in the R wave on inferior leads
Echo: gold standard dx.
Atrial Septal Defects
Management
Management
small defects: less than 6 mm = may close spontaneously in first year of life
decongestive thearpy: rarely needed because they are asymptomatic in early life
Surgical: patch or suture ASD if symptomatic at 2-4 years
Devices: mesh can be delivered transcath.
Ventricular Septal Defect
pathology
Ventricular Septal Defects (VSD)
MOST COMMON DEFECT OF CONGENITAL HEART DISEASE!!!!
Locations of Pathology: Left to Right shunt & mutiple locatios on the septum
- MC = perimembranous: conoventricular near tricuspid valve
- Muscular: usually see multiple “swiss cheese” holes
- inlet & subpulmonic possible too
Pathology of VSD
- blood flows from the LV to the RV, thus immediately going back into the pulmonary arteries to the lungs
- volume overload in the pulmonary artery
- defect sizee and PVR play a role in how much is shunted
- as the pulmonic vascualr resistance falls, increased flow to it
VSD
Clinical Findings
Symptoms
Clinical Findings
- small shunts: can have asymptomatic murmurs : restrictive (small hole) will have normal pressure between the two ventricles
- larger shunts: will affect CO and oxygen delivery: non-restrictive: no pressure difference btween left and right will impact the delivery of oxygen to thebody
Compensatory Mechanisms will start: since there is decrease perfusion via decreased CO and O2 delivery
- RAAS system activaation & Catechoamines to increase CO
___________________________________________
Symptoms
Tachcardic (trying to compensate)
tachypena
diphoresis: extreme with feeding (due to increase SNS because of decreased CO)
fatigue with feeding: failure to thirve
PE
- loud high pitched, HARSH HOLOSYSTOLIC murmur at LLsternal boarder
How is congestive heart failure difference that heart failure noramlly
Congestive heart failure: is not a pump problem; is the inability to compensate problem
this is thought of as “high output heart failure”
thus no periphearl edema as a result since its not a backup issue, its an overworking issues and failure to compensate
VSD
Diagnosis and Imaging
Diagnosis and Imaging
CXR: cardiomegaly, right ventricualr hypertrophy
echo: the perferred method of imaging
EKG
- combined RVH/LVH (large, equiphasic waves in >50% of precordial leads)
MRI: only if echo is not diagnostic
Cardiac Cath: only if other modes arent diagnostic or you’re concerned for pulmonary HTN
VSD
Surgical Indications
Surgical Indications
- heart failure
- anatopic complications (aortic valve prolaspe)
- endocarditis risk
need to prevent eisenmenger physiology
Eisenmenger Physiology
what is it and what happens
a complication of VSD if unoperated on
What is it
- a medial hypertrophy of the pulmonar artery and its branches
- this results in pulmonary hypertension and eventually RV hypertension
overtime: this can become a RIGHT TO LEFT SHUNT since there is NOW so much pressure in teh right side of the heart: it flows the opposite way
RIGHT TO LEFT = CYANOSIS
VSD Management
small defect: could close spontaneously
Decongestive Thearpy
- diuretics
- digoxin
- afterload reduction (ACE inhib.)
- beacuse of the risk of pulonary HTN here
Surgery (usually 2-4 months)
- a patch closure
- most repaired by 2 years old to prevent the pulmonary hypertension
if you cant operate: palliation with pulmonary artery banding to decrease the flow to the lungs until you can operate
Atrioventricualr canal
pathology & population
AV Canal
- essentially, one big valve (a combo tricuspid and mitral)
- with an ASD (hole between atria) & VSD (hole in ventricles)
- most commonly seen in those with trisomy 21
Pathology
- a primum ASD
- inlet VSD
- a common 5-leadflet valve
- thus, as a result blood shunts at both the atrial and ventricle levels (resulting in regurgitaion)
Atrioventricular Canal
Management
Management
- decongestive therapy
- Surgical repair: pathc closure of ASD and VSD
- AV valve plasty to create new tricuspid and mitral valves
- downside is due to the prostehci nature of the new valves, regurgitation on both is a common complications
Patent Ductus Arteriosus (PDA)
why is the ductus arteriosus naturay there
pathologically what is the flow
PDA
Ductus arteriosus
- normally in fetal development, shunts blood from the pulmonary artery out to the aorta to bypass the lungs: since the lungs at that point had a higher PVR
- it should spontaneously close by days 1-4 of life due to the absence of prostoglandins
Pathological
- when the ductus arteriosus fails to close, it results in an ability for blood to go from the aorta BACK into the heart via the PDA, directly into the pulmonary arteries
- since high pressure aorta = to the lower pressure right side of the heart/pulm, artery
- a LEFT to RIGHT shunt
PDA
who is at risk
murmur & clinical Findings
PDA
- Highest riks: premature babies; those with perinatal distress, hyopxia, rubella infection in the 1st trimester
- the result of teh PDA: increased pressure in the pulm artery, resulting in volume overlaod of the LEFT heart since the blood goes lungs, left, aorta, lungs, left, aorta, etc.
Clincial Findings
- continuous machine like souning murmur throughout cycle
- freqeunt repiratry infections, failure to thrive
- bounding pulses with widened pulse pressure
PDA
Diagnostics and Imaging
Managment
Dx. + Imaging
- CXR: normal or cardiomeg.
- EKG: LVH with left atrial enlargement
- echo: Gold standard
Management
- infant: give prostoglanding inhibitors: since prostoglandins are keeping it open!!!
- IV indomethacin preferred (or ibuprofen)
- surgical ligation is 2nd choice;
in children: surgical correction via coiling or ligation
- usually if symptomatic and continued to have issues
When might the PDA remaining open be of benefit???
if there is a blocked outflow of the pulmonary artery OR blocked flocw from teh LV into the aorta- the PDA can be the ONLY source of blood supply to either the lungs or to the body!!!
Obstructed Pulmonary Artery Flow
- no way to get blood into the lungs, so the only way is for backflow from teh PDA
- seen in Tetrology of Fallot due to pulmonary atresia
Obstructed Aortic flow
- no way to get blood to the rest of the body; so they only way is for the PDA to supply
- seen with severe cyanosis, but alive
- hypoplastic left heart syndrome or coarctation of aorta(SeverE)
Role of Prostoglandin E1 and maintaining the PDA
PGE1
the duct will close on its own beginning at birth
- PGE1 will allow the duct to remain patent at birth during newborn period: given to help stabilize the duct if it is dependent upon for pulmonary or sytemic flow
- prostogladin E1 analongs: alprostadil can be given to reduce cyanosis and improve circulation until surgical correction can be done
SE: apena: may neeed to stimulatre respiratory drive via intubation or just NC
IN SUM
a newborn with circulatory shock +/- cyanosis HAS A PROSTOGLANDIN DEPENDENT SYSTEM UNTIL PROVEN OTHERWISE: need alprostadil
START PGE AND GET ECHO STAT
need to blow open PDA and maintain patency
Obstructive Lesions
- 2
- what do they result in
- murmur heard
- EKG
Obstructive Lesions
- result in an inability to allow blood to leave the heart: create compensatory hypertrophy in attempt to push harder
Two ways
- pumonic stenosis: RV hypertrophy results
- aortic stenosis: LV hypertrophy
Systolic ejection murmurs with post-stenotic dilation
- systolic ejection murmur
- ejection “click”
- ventricualr heave & thrill: during flow through
- EKG: hypertrophhy and heart strain
Pulmonic Stenosis
what is it
results in
PE findings
Treatment
Pulm. Stenosis
- inability for blood to flow smoothly from the RV into the Pulmonary artery
- reuslts in; hypertrophic RV
PE findings
- systolic ejection murmur in pulmonic area; radiation to the lungs
Treatment
- balloon valvulopasty
Aortic Stenosis
what is it
results in waht
PE findings
Treatment
What is it
- a difficult for blood to flow from the LV into the aorta
- results in LV hypertrophy
- most commonly due to a bicuspid aortic valve
- results in : POOR PERFUSION since if it cant get to aorta…. compromised Cornary artery perfusion since the ostium sit right there outside the leaflet
PE
- systolici ejction murmur in aortic area with diminisehd pulses
Treatment
- balloon valvuloplasty
Coarctation of the Aorta
what is it
what commonly occurs with it
Pathology
Coarctation of Aorta
- abnormal congential narrowins of the decending thoracic aorta
- results in: non-cyanotic: still blood flow
Occurs with
- commonly with a bicuspid aortic valve
- +/- other left sided heart defects
Pathology
- increased LV afterload in a low cardiac output setting: increased sympatheic activity & RAAS activation thinks there is not flow, attempts to ramp it up
- leads to: left verntricular hypertrophy & secondary hypertension and eventaul congestive heart failure
Symtpoms as a result
- bilateral claudication
- yncope
- DOE
- infants with failure to thirve, shock!!! (since CO is low) cant perfuse
Coarctation of the Aorta
locations
Locations
- Periducatl: cinches off around the level of the ductus arteriosus when it closes
- creates “posterior shelf” on Echo
- if the coarction is preductal: PDA can play a vital role in providing systemic flow
- postductal: no role for the PDA
differ from an inturrupted arch where there is disconnect between teh entier system and therefore pda is the only flow possible
Coarctation of the Aorta
PE and Clincial Findings
PE Findings
- systolic murmur: radiation to the bacl, scapula and chest
- UPPER EXTERMITIY BP WILL BE GREATER THEN THE LOWER!!!!!
- if the narrowing is preductal: RUE will be greater tha LUE
- delayed or weak femoral pulses
- can be diaphoretic, in shock, gery and pale
Dx. and Imaging
- CXR: RIB notching & #3 sign where the narrowing of aorta is seen
- EKG: LVH
- ANGIOgram is gold standard
- if pt unstable in real pracitce: echo will work
Coarctation of the Aorta
Management
Management
KEEP PDA Open: especailly if the coarctaion is preductal; thats the only flow to the rest of body (lower)
- give PGE to keep it open
Surgical Correction
- balloon angioplasty with stent
What conditions are considered critical congential hear diseases
tetraolgoy of fallot
pulmonary atresia
coraction of the arta
hypoplastic left hear
transportaion of great vessesl
total anomalous pulmonary venous return
turcus arteriouss
tricuspid atresia
the 4 Mechanisms of Critical Congential Heart Disease
- decreased pulmonary blood flow
- left heart obstruction
- inadequate mixing of deoxy and oxy blood
- inadequate gas exchange
Prinicples of Cyanosis
hyperoxia test
Cyanosis
- blue discoloring of skin, nais and mucous membranes
- can be observed when there is a clincal desat. of 4-5 grams of hgb in the capillary bed
- greay: indicated anemia: not enough actaul blood to look blue
- Hypoexmia: hen the arterial satuation falls below 90%
Hyperoxia Test
- helps determine if the hypoxia is a result of the lungs not being able to get O2 or the heart not delivering it
- test & determine the PaO2 of the room
- then administer high FiO2 for 10 minutes
- repeat PaO2 reading
- if PaO2 >150 = suggests lung issue
- if PaO2 < 150 = suggests heart issue
can test in teh hand and foot to see if its pre or post ductal flow thats the issue
Tetraology of Fallot
what is it
Pathology
Tetraology of Fallot
most commony cyanotic heart condition congenital
What is it
- Right ventricular Outflow tract obstruction (either a valve or subvalve issue)
- right ventricular hypertrophy
- Ventricular septal defect
- Overriding Aorta: taking blood from both R and L ventricle
Pathology
- the degree of right ventricualr outflow obstruction determines the level of cyanosis
- pt has Tet Spells: episodes of hypercyanotic states
- increased cyanosis, loss of murmur and LOC
- older kids: will decreased the tet spell by squatting down
- squatting: inreased SVR, therefore blooe will flow to the PVR (less resistant) and flows there
- the cyanosis get WORSE with age
ToF
clinical manifestation
dx. and imaging
Clinical manifestation
- harsh holosystolic murmur at LUSB : sounds lik epulmonary stenosis, because it is
- digital clubbing = cyanosis
- right ventricualr heave: trying to push
Dx
- CXR: boot shaped heart: prominent RV
- EKG: RVH and RA enlarged
- echo: GOLD STANDARD
Management
- repair ToF with patch closure of the VSD
- transmular patch of teh RV outflow at the pulmonary valve
ToF with Pulmonary Atresia
Pulmonary Atresia = lack of flow: because the pulmmoanry artery is not connected to teh RV
Therefore, the only reason blow flow cna occur is due to the PDA remainig open to supply pulmonary flow
therefore, they NEED PGE1 !!!
Treatment
- the can do a shunt! from the aortic branches to the pumonary arterie s
Clinical presentation of Left herat obstrutive lesions
Left Heart Obstrutive: decreased systemic out flow
grey/ashen color pt.
tacypnea
poor perfusion
decreased/differential pulses
single sencond heart sound
Hypoplastic Left Heart Syndrome (HLHS)
what is it
Left heart: ventricle doesnt develop/too small, no valve to get there
Thus
blood flows into right heart into lungs
lungs into LA, but cant go LV
so: patenet formane ovale exisits to allow blood to flow back to the right atrium
then from teh right atrium back to the pulmonary artery
and then into the PDA
Thus,
- cadiac output and systemic perfusion relie directly on the PDA to reamined open AND the foramen ovale to remain open
HLHS
management
Management
- keep the ducts open: GIVE PGE1
- no supplemental oxygen:it will drop PVR and decrease ability to perfuse at all
SURGERY ASAP
- allow adequate mixing
- limit pulmonary flow
- augmnet systemic flow
- Norwood procedure
- creates a shunt to all blood to mix * makes the RVfunctio as the LV for a while - Hemi-Fontan Procedure
- connect SVC to pulm artery and deteach SVC from RA - Fontan Procedure
- IVC reconnected to the pulmonary artery and bypass the entire right heart as a reuslt
Transposition of the Great Arteries
waht is it
pathology
Transposition of teh Great Arteries (TGA)
what is it
- the systemic and pulmonary systems work in parellel not in series, thus their only connect point is the PDA, VSD or ASD
L-TGA & D-TGA
L-TGA = congenitally corrected
- in that the RV and LV “morpholocially swapped spots, but physically, swapped positions
- the flow of blood remains the same (“righ heart’ to pulm., and “left heart” to body)
D-TGA
- the aorta comes from the RV — so literally no ocygen
- and the pulmonary artery comes from the LV so its literally reoxygenated the same
SEVERE CYANOSIS
D-TGA
pathology
clinical manifestations
D-TGA
- deoxy. systemic blood comes to the RA, but goes to RV, then directly back out to the systemic via aorta – never reaches lungs
- the oxygenated blood drains from the pulmonary veins into the LA, LV and back to the pulmanry artery – never leaving lungs
this is incompatable with life UNLESS there is a mixing present: PDA, PFO, ASD or VSD (mostly on the atrial level there is mixing)
Clinical Manifestations
- less mixing: more severe cyanosis and hypoxia
- reverse cyanosis: aka the RUE is LOWER O2 stat than the LUE since teh Right is beind fed by deoxy. blood all day long
- if VSD present: less severe cyanosis because blood cna mix, just have CHF with volume overload
- if VSD is not present: cyanosis + tachpnea
D-TGA
dx. and imaging
management
Dx and Imaging
- CXR: egg on a string; all the great vessels in line, so it looks like one string holding heart as egg
- Echo: primary dx. sees mixing and shunting
- cardiac cath with anigogram: not to dx. bu t used for balloon placement
Management
- promote mixing between systemic and pulmonic circuits
- admin PGE1 to continue the ability to mix through PDA
- balloon atrial septosomy: balloon and KEEP OPEN the ASD/PFO
- they they perform arterial switch: roots of the pulmonary artery and aortia swap
Trucus Arterious
what is it
pathology
Tructus Arteriosus
what is it
- one common arterial trunk that supplies both the systemic and pulmonary circuits
- tyically one valve, four leaflets
- overiding a large VSD that allows mixing and input from both ventrilces
Pathology
- complete mixing at the ventricular level
- influence by systeic and pulmonary system
- if there is LOW PVR: blood with flow to here, creating symptoms of heart failure & compromised cardiac output
- if there is high PVR: blood will flow to systemic,but without going to lungs = cyanosis
- pulmnary vascualr disease andpulmnary artery HTN can occurbecause high pressures
Truncus Arterious
Dx. and Imaging
management
Dx and Imaging
- Echo: GS
- CXR: extreme cardiomegaly: wall to wall heart!!!!!!
- EKG: right atriacl enlargement
- cardica cath: to eval pulmo-veno disease
Managemnet
- surgical repair: VSD closure with truncal valve pushed to the left side of heart
- excise pulm arter from the trucus
- placement of RV to PA and anastomsos
TAPVR
total anomalous pulmonary venous return
when teh confluence of the pulmonary veins do NOT drain to the left atrium….
can go
- suercardiac: to SVC
- intracardia: to cornary sinuse
- infracardiac: to teh intrahepatic
severity depends on where they drain to (the above)
Tricuspid Atresia
what is it
pathology
what is it
- no connection from the RA to the hypoplastic right ventricle
- the variants depend on the size and where the great arteries are palliation by Fontan urgery
the 5 Ts of Cyanotic Heart Disease
Trucus Arterious: 1 vessle
Transportation fo Great Arteries: 2 vessels swithced positions
Tricuspid Atresia: no tricuspid: hypoplastic right heart: need ASD/VSD to transfer
Tetraolgoy of Fallot: 4 porblems
Total anomouals pulmonar venous return
all four pulm viens connent somewhere other thatn teh right atrium
