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Left to RightShunts
Left to RightShunts
William Herring, M.D. 2002
In Slide Show mode, to advance slides, press spacebaror click left mouse button
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7 yo acyanotic female
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Atrial Septal Defect
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Atrial Septal DefectFour Major Types
Ostium secundum
Ostium primum
Sinus venosus
Posteroinferior
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Atrial Septal DefectGeneral
4:1 ratio of females to males
Most frequent congenital heart lesioninitially diagnosed in adult
Frequently associated with Ellis-vanCreveld and Holt-Oram syndromes
Associated with prolapsing mitral valve
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Atrial Septal DefectOstium Secundum Type
Most common is ostium secundum(60%) located at fossa ovalis
High association with prolapse of
mitral valve
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Atrial Septal DefectOstium Primum Type
Ostium primum type usually part of
endocardial cushion defect
Frequently associated with cleft
mitral and tricuspid valves
Tends to act like VSD physiologically
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Looking throughostium primum defectat cleft mitral valve
Proximity of ostiumprimum defect totricuspid valve
Frank Netter, MD Novartis
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Atrial Septal DefectSinus Venosus Type
Sinus venosus type located high in
inter-atrial septum 90% association of anomalous drainage
of R upper pulmonary vein with SVC
or right atrium
Partial anomalous pulmonary venous return
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Frank Netter, MD Novartis
Right atrium open looking into left atrium through ASD
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Atrial Septal DefectPosteroinferior Type
Most rare type
Associated with absence of coronary
sinus and left SVC emptying into LA
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Atrial Septal DefectPulmonary Hypertension
Rare in ostium secundum variety (
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37 yo female with severe PAH 2ostium primum type of ASD
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Atrial Septal DefectX-Ray Findings
Enlarged pulmonary vessels
Normal-sized left atrium
Normal to small aorta
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Atrial Septal DefectComplications
Large shunts associated with
Pulmonary infections and cardiac arrythmias
Higher incidence of pericardial disease
with ASD than any other CHD Bacterial endocarditis is rare
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LA Ao
ASD
PDA
VSD
Differentiating ASD, PDA and VSD
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Atrial Septal DefectWhy the Left Atrium Isnt Enlarged
LARA
RV LV
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1 yo acyanotic female
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Ventricular SeptalVentricular SeptalDefectDefect
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Ventricular Septal DefectGeneral
Most common L R shunt Shunt is actually from left ventricle into
pulmonary artery more than into right
ventricle
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Ventricular Septal DefectTypes
Membranous
Supracristal
Muscular
AV canal
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Ventricular Septal DefectMembranous
Membranous = perimembranous VSD(75-80%most common)
Location: Posterior and inferior tocrista supraventricularis near right
and posterior (=non-coronary) aortic
valve cusps
Associated with: small aneurysms of
membranous septum
Right entricle opened
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Right ventricle opened
Cristasupraventrularis
Normal
Frank Netter, MD Novartis
Membranous VSD
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Aneurysm ofmembranousseptum
Normal
Frank Netter, MD Novartis
Frank Netter, MD Novartis
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Ventricular Septal DefectSupracristal
Supracristal = conal VSD (5%leastcommon)
Crista supraventricularis= inverted U-shaped muscular ridge posterior andinferior to the pulmonic valve high in
interventricular septum On CXR: right aortic valve cusp may
herniate
aortic insufficiency
LV open RV open
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LV open RV open
Frank Netter, MD Novartis
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Ventricular Septal DefectMuscular
Muscular VSD (510%)
Low and anterior within trabeculationsof muscular septum
May consist of multiple VSDs = swiss-cheese septum
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Swisscheese
Frank Netter, MD Novartis
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Ventricular Septal DefectAV Canal
Atrioventricular canal = endocardial
cushion type = posterior VSD (510%)
Location: adjacent to septal and
anterior leaflet of mitral valve
Large VSD pulmonary hypertension,
eventually shunt reversal Eisenmengers physiology
Very large VSD CHF soon after birth
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Frank Netter, MD Novartis
Large posterior VSD(AV canal)
Frank Netter, MD Novartis
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Ventricular Septal DefectNatural History
Natural history of VSD is affected by
two factors:
Location of defect
Muscular and perimembranous have high
incidence of spontaneous closure
Endocardial cushion defects have low rate of
closure
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Ventricular Septal DefectNatural History
Size of the defect
Larger the defect, more likely to CHF
Smaller the defect, more likely to be
asymptomatic
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Ventricular Septal DefectEisenmenger Physiology
Progressive increase in pulmonary
vascular resistance Intimal and medial hyperplasia
Reversal of L R shunt to R L shunt
Cyanosis
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Ventricular Septal DefectClinical Course
Neonates usually asymptomaticbecause of high pulmonary vascular
resistance from birth to 6 weeks Common cause of CHF in infancy
Bacterial endocarditis may develop Severe pulmonary hypertension
Eisenmengers physiology/cyanosis
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Ventricular Septal DefectX-ray Findings
Prominent main pulmonary artery
Adult
Shunt vasculature (increased flow tothe lungs)
LA enlargement (80%)
Aorta normal in size
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5 yo acyanotic male
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Ventricular Septal DefectWhy Left Atrium Is Enlarged
LARA
RV LV
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4 mos old acyanotic female
V t i l S t l D f t
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Ventricular Septal DefectPrognosis
Spontaneous closure occurs in
40% during first 2 years of life 60% by 5 years
V t i l S t l D f t
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Ventricular Septal DefectIndications For Surgery
Greater than 2:1 shunt, surgery requiredbefore pulmonary arterial hypertension
develops
CHF unresponsive to medical management
Failure to grow Supracristal defects because of their high
incidence of AI
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8 mos old acyanotic female
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Patent DuctusPatent DuctusArteriosusArteriosus
P t t D t A t i
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Patent Ductus ArteriosusGeneral
Higher incidence in
Trisomy 21
Trisomy 18
Rubella
Preemies
Predominance in females 4:1
P t t D t A t i
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Patent Ductus ArteriosusAnatomy
Ductus connects pulmonary artery to
descending aorta just distal to left
subclavian artery
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Ductus Arteriosus
Frank Netter, MD Novartis
Ductus Arteriosus
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Ductus ArteriosusPhysiology
In fetal life, shunts blood from
pulmonary artery to aorta
At birth, increase in arterial oxygenconcentration constriction of
ductus
Ductus Arteriosus
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Ductus ArteriosusNormal Closure
Functional closure
By 24 hrs of life Normal anatomic closure
Complete by 2 months in 90%
Closure at 1 year in 99%
Patent Ductus Arteriosus
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Patent Ductus ArteriosusPathophysiology
Ductus may persist Because of defect in muscular wall of ductus, or
Chemical defect in response to oxygen
Anatomic persistence of ductus
beyond 4 months is abnormal
Blood is shunted from aorta to
pulmonary arteries
Patent Ductus Arteriosus
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Patent Ductus ArteriosusClinical
Common cause of CHF in premature
infants
Usually at age 1 week (after HMD subsides and
pulmonary arterial pressure falls)
Wide pulse pressure Continuous murmur
Patent Ductus Arteriosus
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Patent Ductus ArteriosusX-ray Findings
Cardiomegaly Enlarged left atrium
Prominent main pulmonary artery(adult)
Prominent peripheral pulmonaryvasculature
Prominence of ascending aorta
Patent Ductus Arteriosus
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Patent Ductus ArteriosusWhy Left Atrium Is Enlarged
LARA
RV LV
Patent Ductus Arteriosus
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Patent Ductus ArteriosusCalcifications
Punctate calcification at site of closed
ductus is normal finding
Linear or railroad track calcification at
site of ductus may be seen in adults
with PDA
Patent Ductus Arteriosus
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Patent Ductus ArteriosusPrognosis
Spontaneous closure may occur
Patent Ductus Arteriosus
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Patent Ductus ArteriosusComplications
CHF
Failure to grow
Pulmonary infections
Bacterial endocarditis
Eisenmengers physiology with advancedlesions
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2 yo old cyanotic female
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Partial or TotalPartial or Total
Anomalous PulmonaryAnomalous PulmonaryVenous ReturnVenous Return
Cyanosis With Increased
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Cyanosis With Increased
Vascularity
Truncus types I, II, III
TAPVR
Tricuspid atresia*
Transposition*
Single ventricle
* Also appears on DDx of Cyanosis with Inc Vascularity
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Two Types
Partial (PAPVR)
Mild physiologic abnormality Usually asymptomatic
Total (TAPVR) Serious physiologic abnormalities
Return ofbl d f
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Normal heart
blood from
lungs is byfour pulmonary
veins to LA
RA LA
RV LV
PA Ao
PAPVR
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PAPVRGeneral
One of the four pulmonary veins may
drain into right atrium
Mild or no physiologic consequence
Associated with ASD Sinus venosus or ostium secundum types
Return ofblood from
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Partial Anomalous Pulmonary Return
blood from
lungs is mostlyto LAOne vein
abnormally
connected toright heart
Frequentlyassociated withsinus venosus
or secundumASD
RA LA
RV LV
PA Ao
TAPVR
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General
All have shunt through lungs to R side
of heart
All must also have R L shunt for
survival
Obligatory ASD to return blood to the systemic
side All are cyanotic
Identical oxygenation in all four chambers
TAPVR
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Types
Supracardiac
Cardiac Infracardiac
Mixed
TAPVR
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Supracardiac TypeType I
Most common (52%)
Pulmonary veins drain into verticalvein (behind left pulmonary artery)
left brachiocephalic vein SVC DDx: VSD with large thymus
Left Brachiocephalic vein
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Leftsuperiorvena cava
Rightsuperiorvena
cava
Vertical
vein
Pulmonaryveins
Frank Netter, MD Novartis
Rightatrium
TAPVR-Supracardiac Type 1
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TAPVR-Supracardiac
Type 1
TAPVR
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Supracardiac Type 1X-ray Findings
Snowman heart = dilated SVC+ left
vertical vein
Shunt vasculature 2 increased return
to right heart
Enlargement of right heart 2 volume
overload
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TAPVR-Supracardiac Type 1
Blood movesBlood from
lungs drains
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RA LA
RV LV
PA Ao
TAPVRType ISupracardiac type
Blood moves
through Lbrachiocephalic v
to R SVC
g
into left verticalvein
to L SVC
Increasedreturn to rightheart overloadslungsshunt vessels
ASD provides R L shunt to
allowoxygenatedblood to reachbody (moderate
cyanosis)
Snowman Heart
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TAPVRType ISupracardiac type
TAPVR
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Cardiac TypeType II
Second most common: 30% Drains into coronary sinus or RA
Coronary sinus more common
Increased pulmonary vasculature
Overload of RV CHF after birth 20% of Is and IIs survive to adulthood
Remainder expire in first year
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Coronarysinus
Frank Netter, MD Novartis
TAPVR-Coronary Sinus-Type II
Blood returnsfrom lung to RA
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TAPVRType IICardiac Type
from lung to RAor coronary sinus
ASD provides R L shunt toallow
oxygenatedblood to reachbody (moderatecyanosis)
Increasedreturn to rightheartoverloads
lungsshunt vessels
RA LA
RV LV
PA Ao
TAPVR
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Infracardiac TypeType III
Percent of total: 12% Long pulmonary veins course down
along esophagus Empty into IVC or portal vein (more
common)
Vein constricted by diaphragm as it
passes through esophageal hiatus
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PulmonaryveinsPortal vein
Frank Netter, MD Novartis
TAPVR-Type III-Infradiaphragmatic
TAPVR
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Infracardiac TypeContinued
Severe CHF (90%) 2 obstruction tovenous return
Cyanotic 2 right left shuntthrough ASD
Associated with asplenia (80%), or
polysplenia
Prognosis=death within a few days
CHF vasculature Blood returningfrom lungs
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TAPVRType IIIInfracardiac type
ASD providesR L shunt toallowoxygenatedblood to reachbody (cyanotic)
RA LA
RV LV
PA Ao
gpulmonary veinswhich areconstricted bydiaphragm CHF
To portal v IVC RA
TAPVR
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Mixed TypeType IV
Percent of total: 6%
Mixtures of types I III
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Unknowns
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ASD (primum) with PAHASD (primum) with PAH
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TAPVR from below diaphragmTAPVR from below diaphragm
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VSDVSD
ASDASD
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The End