Congenital Heart Disease

Question 1

Pediatric Functional Murmurs
what are they
characteristics of the sound

Answer 1

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

Question 2

Pediatric Murmurs: Still’s Murmur
- what is it
- chacteristics of it

Answer 2

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

Question 3

Pediatric Murmurs: Venous Hum
- what is it
- chacteristics of it

Answer 3

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

Question 4

Pediatric Murmurs: Pulmonary Ejection Murmur
- what is it
- chacteristics of it

Answer 4

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

Question 5

Congenital Heart Disease
definitions
etiology

Answer 5

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

Question 6

Fetal Circulation
what are the three critial structures
how does blood flow

Answer 6

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

Question 7

Role of PVR and the closure of the ductus arteriosus

Answer 7

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

Question 8

Acyanotic Congenital Heart Diseases
left to right shunts
4 different ones

Answer 8

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)

Question 9

Symptoms of all congenital heart diseases in children

Answer 9

Commonly….
- poor weight gain
- poor feeding, decreased PO intake
- lethargy
- diphoresis with feeds

Question 10

Explain the physiology behind Left to Right Shunts

Answer 10

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

Question 11

Atrial Septal Defects
pathology
Ostium locations

Answer 11

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

Question 12

Atrial Septal Defects
Symptoms

Answer 12

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

Question 13

Atrial Septal Defects
PE finiding
Murmur
Imaging & Diagnositcs

Answer 13

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.

Question 14

Atrial Septal Defects
Management

Answer 14

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.

Question 15

Ventricular Septal Defect

pathology

Answer 15

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

Question 16

VSD
Clinical Findings
Symptoms

Answer 16

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

Question 17

How is congestive heart failure difference that heart failure noramlly

Answer 17

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

Question 18

VSD

Diagnosis and Imaging

Answer 18

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

Question 19

VSD
Surgical Indications

Answer 19

Surgical Indications
- heart failure
- anatopic complications (aortic valve prolaspe)
- endocarditis risk

need to prevent eisenmenger physiology

Question 20

Eisenmenger Physiology
what is it and what happens

Answer 20

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

Question 21

VSD Management

Answer 21

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

Question 22

Atrioventricualr canal
pathology & population

Answer 22

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)

Question 23

Atrioventricular Canal
Management

Answer 23

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

Question 24

Patent Ductus Arteriosus (PDA)
why is the ductus arteriosus naturay there
pathologically what is the flow

Answer 24

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

Question 25

PDA
who is at risk
murmur & clinical Findings

Answer 25

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

Question 26

PDA
Diagnostics and Imaging
Managment

Answer 26

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

Question 27

When might the PDA remaining open be of benefit???

Answer 27

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)

Question 28

Role of Prostoglandin E1 and maintaining the PDA

Answer 28

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

Question 29

Obstructive Lesions
- 2
- what do they result in
- murmur heard
- EKG

Answer 29

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

Question 30

Pulmonic Stenosis
what is it
results in
PE findings
Treatment

Answer 30

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

Question 31

Aortic Stenosis
what is it
results in waht
PE findings
Treatment

Answer 31

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

Question 32

Coarctation of the Aorta
what is it
what commonly occurs with it
Pathology

Answer 32

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

Question 33

Coarctation of the Aorta
locations

Answer 33

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

Question 34

Coarctation of the Aorta
PE and Clincial Findings

Answer 34

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

Question 35

Coarctation of the Aorta
Management

Answer 35

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

Question 36

What conditions are considered critical congential hear diseases

Answer 36

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

Question 37

Prinicples of Cyanosis

hyperoxia test

Answer 37

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

Question 38

Tetraology of Fallot
what is it
Pathology

Answer 38

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

Question 39

ToF
clinical manifestation
dx. and imaging

Answer 39

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

Question 40

ToF with Pulmonary Atresia

Answer 40

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

Question 41

Clinical presentation of Left herat obstrutive lesions

Answer 41

Left Heart Obstrutive: decreased systemic out flow

grey/ashen color pt.
tacypnea
poor perfusion
decreased/differential pulses
single sencond heart sound

Question 42

Hypoplastic Left Heart Syndrome (HLHS)
what is it

Answer 42

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

Question 43

HLHS
management

Answer 43

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

1. Norwood procedure
   - creates a shunt to all blood to mix * makes the RVfunctio as the LV for a while
2. Hemi-Fontan Procedure
   - connect SVC to pulm artery and deteach SVC from RA
3. Fontan Procedure
4. • IVC reconnected to the pulmonary artery and bypass the entire right heart as a reuslt

Question 44

Transposition of the Great Arteries
waht is it
pathology

Answer 44

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

Question 45

D-TGA
pathology
clinical manifestations

Answer 45

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

Question 46

D-TGA
dx. and imaging
management

Answer 46

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

Question 47

Trucus Arterious
what is it
pathology

Answer 47

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

Question 48

Truncus Arterious
Dx. and Imaging
management

Answer 48

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

Question 49

TAPVR
total anomalous pulmonary venous return

Answer 49

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)

Question 50

Tricuspid Atresia
what is it
pathology

Answer 50

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

Question 51

the 5 Ts of Cyanotic Heart Disease

Answer 51

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