Path Block 2- Congenital Heart Disease

Front 1

congenital heart disease

Back 1

-abnormalities of heart and/or great vessels
-present from birth
-6-8 per 1000 livebirths
-can result in stillbirth, present at birth or remain undetected until later

Front 2

CHD etiology

Back 2

-most arise during weeks 3-8 of embryogenesis
-genetic, environmental, idiopathic

Front 3

fetal circulation

Back 3

-placenta--> umbilical vein --> liver--> ductus venosus--> IVC--> RA--> foramen ovale--> pulm artery--> ductus arteriosus--> aorta--> body--> umbilical artery --> placenta

Front 4

L-R shunts

Back 4

-initially oxygenated blood flowing into R circulation --> no cyanosis
-increases pulm blood flow beyond its designed capacity --> pulm HTN and RV hypertrophy
-increases R pressure and REVERSES the blood flow and becomes a R-->L shunt
-LATE cyanosis

Front 5

increase in pulm blood flow

Back 5

-well tolerated by pulm vessels
-ie ASD

Front 6

increase in pulm blood pressure

Back 6

-not well tolerated by pulm vessels
-ie VSD

Front 7

plexogenic pulm HTN

Back 7

-medial hypertrophy
-intimal proliferation
-plexiform lesions (irrev)

Front 8

plexogenic pulm HTN most often happen with what anomalies?

Back 8

-VSD>>PDA>>ASD

Front 9

atrial septal defect

Back 9

-interatrial opening
-present throughout the cardiac cycle
-secundum (90%), primum (5%), sinus venosus (5%)

Front 10

location of atrial septal defects

Back 10

-secundum-- at fossa ovalis (90%)
-primum-- adjacent to AV valves (5%)
-sinus venosum-- near SVC entrance (5%)

Front 11

ASD features

Back 11

-may be asymptomatic until adulthood
-can allow paradoxical embolism
-<10% lead to pulm HTN

Front 12

patent foramen ovale

Back 12

-present in 1/3 of people
-small remnant opening
-no shunting (back pressure closes it) except rarely
-can cause paradoxical emboli, decompression sickness, migraines

Front 13

ventricular septal defect

Back 13

-most common heart anomaly
-interventricular opening between LV and RV
-usually assoc with other anomalies (30% in isolation)

Front 14

VSD locations

Back 14

-90% at membranous septum

Front 15

membranous VSD

Back 15

-defect is usually large
-spontaneous closure by septal TV leaflets 10% of the time
-or requires surgical closure at around 1 yo

Front 16

muscular VSD

Back 16

-defect is usually small
-spontaneous closure by fibrous adhesions in >60% by 1yo
-most do not need surgery
-multiple= swiss cheese septum

Front 17

normal ductus arteriosus closure

Back 17

-functional: 12h
-anatomical: 3 months
-delayed by PGE2
-closes late in premies and at high altitude

Front 18

PDA exam findings

Back 18

-harsh, continuous, machine murmur

Front 19

PDA features

Back 19

-usually seen in isolation (90%)
-necessary for survival in AV or PV atresia, others

Front 20

atrioventricular septal defect

Back 20

-deficient AV septum
-assoc with MV and TV anomalies
-partial or complete

Front 21

what CHD is seen in 40% of babies with Downs?

Back 21

-complete AVS defect

Front 22

types of AVS defects

Back 22

-partial: primum ASD and cleft MV and MR
-complete: AVSD and common AV valve

Front 23

R-->L shunts

Back 23

-dump de-ox blood into systemic circulation
-dec pulm blood flow
-early cyanosis
-paradoxical emboli and decompression sickness

Front 24

R-L shunts symptoms

Back 24

-cyanosis
-digital clubbing
-polycythemia (compensatory RBC production)

Front 25

anomalies causing R-L shunts

Back 25

-tetrology of fallot
-transposition of the great arteries
-truncus arteriosus
-tricuspid atresia
-total anomalous pulm venous connection

Front 26

tetralogy of fallot

Back 26

-anteriorsuperior displacement of the infundicular septum
-subPulmonary stenosis
-RV hypertrophy
-Overriding aorta
-VSD

requires surgery

Front 27

what is the most common form of cyanotic congenital heart disease?

Back 27

-tetralogy of fallot

Front 28

what do the clinical outcomes of tetralogy of fallot depend on?

Back 28

-severity of subpulmonary stenosis
-stenosis here is good because prevents lung damage that could be caused by the RV hypertrophy that is seen

Front 29

shape of heart in tetralogy of fallot

Back 29

-boot shaped dt RV hypertrophy

Front 30

tetralogy of fallot and lungs

Back 30

-subpulmonary stenosis prevents lung damage
-pulmonary outlet doesn't grow with the child so effects are worse with age

Front 31

transposition of the great vessels

Back 31

-aorta arises from RV, pulm artery arises from LV
-separate pulm and systemic circulations
-RV hypertrophy and pulm HTN
-can be with intact ventricular septal or with VSD (more stable)

Front 32

transposition of the great vessels and intact vent septum

Back 32

-65%
-unstable
-need prompt surgical intervention

Front 33

transposition of the great vessels with VSD

Back 33

-35%
-stable
-will need surgery

Front 34

truncus arteriosus

Back 34

-origin of aorta and pulm artery from truncal artery
-failure of separation of embryologic truncus into aorta and pulm artery
-causes mixing of blood
-inc pulm blood flow, pulm HTN, cyanosis

Front 35

what is often seen with truncus arteriosus

Back 35

-large VSD

Front 36

variations seen with truncus arteriosus

Back 36

-patterns in pulm artery origin
-truncal valve cusps (merged aortic and pulm valves, variable # cusps, etc)
-can be seen with DiGeorge's

Front 37

tricuspid atresia

Back 37

-complete occlusion of the tricuspid valve orifice
-from unequal division of AV canal-- mitral valve is enlarged
-causes RV hyPOplasia (seeing less blood)
-need coexisiting ASD/PFO and VSD

Front 38

total anolamous pulm venous return

Back 38

-pulm veins do not directly drain into LA
-LA hypoplasia
-connect via innominate vein or coronary sinus
-need ASD/PFO

Front 39

obstructions

Back 39

-aortic coarctation
-pulm stenosis/atresia
-aortic stenosis/atresia

Front 40

aortic coarctation

Back 40

-narrowing of the aorta
-tubular hypoplasia with PDA (infants) or ridgelike infolding without PDA (adult)

Front 41

aortic coarctation clinical presentation

Back 41

-bicuspid AV (50%)
-rib notching from extra collateral arteries
-symptoms depend on degree of narrowing
-surgically treatable HTN

Front 42

pulmonary stenosis

Back 42

-pulm valve obstruction dt hypoplasia, dysplasia, or abnormal number of cusps
-can be isolated or with VSD

Front 43

isolated PV stenosis

Back 43

-RV dilation and hypertrophy
-post-stenotic injury to PA
-may be asymptomatic until adulthood

Front 44

PV atresia with intact VS

Back 44

-hypoplastic RV and TV
-PDA needed to get blood to lungs

Front 45

aortic stenosis

Back 45

-aortic valve obstruction dt hypoplasia, dysplasia or abnormal number of cusps

Front 46

isolated AV stenosis

Back 46

-80%
-LV hypertrophy and LA dilatation
-mild to critical
-systolic murmur

Front 47

hypoplastic L heart syndrome

Back 47

-aortic valve atresia with intact VS
-hypoplastic mitral valve and LV
-depend on PDA for survival
-requires staged surgical correction

Front 48

Ebstein anomaly

Back 48

-inferiorly displaced and adherent septal and posterior leaflets
-redundant anterior leaflet
-dilated annulus with TR

Front 49

secondary effects of Ebstein anomalies

Back 49

-RV and RA dilatation

Front 50

features of Ebstein anomalies

Back 50

-arrhythmias inc WPW syndrome
-may be asymptomatic until adulthood

Front 51

dextrocardia

Back 51

-R-sided heart
-usually assoc with situs invertus
-5-10% have other heart anomalies, usually TGA

Front 52

ectopia cordis

Back 52

-anterior thoracic wall fails to close properly
-can present as part of pentalogy of cantrell

Front 53

late effects of CHD

Back 53

-endocarditis
-hyperviscosity
-pulm HTN and shunt reversal
-childbearing risk
-residual post-op pathology

Front 54

least worrisome CHD

Back 54

-ASD

Front 55

where do obstructive lesions exert their damage

Back 55

-upstream of obstruction