Lt or Shunts

8/10/2019 Lt or Shunts

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Left to RightShunts

Left to RightShunts

William Herring, M.D. 2002

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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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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


Membranous VSD


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Aneurysm ofmembranousseptum

Normal




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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



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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



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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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Large posterior VSD(AV canal)



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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


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



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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



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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



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Patent Ductus ArteriosusWhy Left Atrium Is Enlarged

LARA

RV LV



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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



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Patent Ductus ArteriosusPrognosis

Spontaneous closure may occur



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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


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


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


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

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Lt or Shunts