William Herring, M.D.

2003

Left to Right
Shunts
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Whats the diagnosis?

7 yo acyanotic female

Atrial Septal Defect

Atrial Septal Defect

Four Major Types

Ostium secundum

Ostium primum

Sinus venosus

Posteroinferior

Atrial Septal Defect

General
l

4:1 ratio of females to males

Most frequent congenital heart lesion

initially diagnosed in adult

Frequently associated with Ellis-van

Creveld and Holt-Oram syndromes

Associated with prolapsing mitral valve

Holt-Oram Syndrome
Absence or hypoplasia of the radial ray

Atrial Septal Defect

Ostium Secundum Type

Most common is ostium secundum

(60%) located at fossa ovalis

High association with prolapse of

mitral valve

Normal

Right atrium open looking into left

atrium through ASD

Frank Netter, MD Novartis

Atrial Septal Defect

Ostium 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

Looking through
ostium primum defect
at cleft mitral valve

Proximity of ostium
primum defect to
tricuspid valve

Frank Netter, MD Novartis

Atrial Septal Defect

Sinus 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
l

Partial anomalous pulmonary venous return

 Frank Netter, MD Novartis

Right atrium open looking into left atrium through ASD

Atrial Septal Defect

Posteroinferior Type

Most rare type

Associated with absence of coronary

sinus and left SVC emptying into LA

Atrial Septal Defect

Pulmonary Hypertension

Rare in ostium secundum variety (<6%)

n

Low pressure shunt from LA RA

More common in ostium primum variety

n

Behaves physiologically like VSD

37 yo female with severe PAH 2

ostium primum type of ASD

Atrial Septal Defect

X-Ray Findings

Enlarged pulmonary vessels

Normal-sized left atrium

Normal to small aorta

Prominent
MPA

Prominent
pulmonary
vessels

Normal left
atrium

ASD

Atrial Septal Defect

Complications

Large shunts associated with

n

Pulmonary infections and cardiac arrythmias

Higher incidence of pericardial disease

with ASD than any other CHD

Bacterial endocarditis is rare

Differentiating ASD, PDA and VSD

LA

Ao

ASD

PDA

VSD

Atrial Septal Defect

Why the Left Atrium Isnt Enlarged

RA

LA

RV

LV

Auckland MRI

Ostium Secundum
ASD
Amersham

Auckland MRI

SCMR

Discontinuity in the atrial septum with systolic signal void consistent with L->R shunt
on atrial level
Right atrium is mildly dilated; RV, LV and LA size are normal

Whats the diagnosis?

1 yo acyanotic female

Ventricular Septal Defect

General

Most common L R shunt

Shunt is actually from left ventricle into
pulmonary artery more than into right
ventricle

Ventricular Septal Defect

Types

Membranous

Supracristal

Muscular

AV canal

Ventricular Septal Defect

Membranous

Membranous = perimembranous VSD

(75-80%most common)
Location: Posterior and inferior to
crista supraventricularis near right
and posterior (=non-coronary) aortic
valve cusps
Associated with: small aneurysms of
membranous septum

Auckland MRI

SCMR

Discontinuity in the atrial septum with systolic signal void consistent with L->R shunt
on atrial level
Right atrium is mildly dilated; RV, LV and LA size are normal

Right ventricle opened

Crista
supraventricularis

Normal

Membranous VSD
Frank Netter, MD Novartis

Aneurysm of
membranous
septum

Normal

Frank Netter, MD Novartis

Ventricular Septal Defect

Supracristal

Supracristal = conal VSD (5%least

common)
Crista supraventricularis= inverted Ushaped muscular ridge posterior and
inferior to the pulmonic valve high in
interventricular septum
Right aortic valve cusp may herniate
aortic insufficiency

Ventricular Septal Defect

Muscular

Muscular VSD (510%)

Low and anterior within trabeculations
of muscular septum
May consist of multiple VSDs = Swisscheese septum

Swiss
cheese

Frank Netter, MD Novartis

Ventricular Septal Defect

AV 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

Large posterior VSD

(AV canal)

Frank Netter, MD Novartis

Ventricular Septal Defect

Natural 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

Ventricular Septal Defect

Natural History

Size of the defect

Larger the defect, more likely to CHF

Smaller the defect, more likely to be
asymptomatic

Ventricular Septal Defect

Natural History

Size of the defect

Larger the defect, more likely to CHF

Smaller the defect, more likely to be
asymptomatic

Ventricular Septal Defect

Clinical Course

Neonates usually asymptomatic

because 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

Ventricular Septal Defect

Why Left Atrium Is Enlarged

RA

LA

RV

LV

VSD

Ventricular Septal Defect

Prognosis

Spontaneous closure occurs in

40% during first 2 years of life
60% by 5 years

Ventricular Septal Defect

Indications For Surgery

Greater than 2:1 shunt, surgery required

before pulmonary arterial hypertension
develops

CHF unresponsive to medical management

Failure to grow

Supracristal defects because of their

high incidence of AI

Auckland MRI

Membranous VSD
Amersham

Whats the diagnosis?

8 mos old acyanotic female

Patent Ductus
Arteriosus

Patent Ductus Arteriosus

General

Higher incidence in

Trisomy 21

Trisomy 18

Rubella

Preemies

Predominance in females 4:1

Patent Ductus Arteriosus

Anatomy

Ductus connects pulmonary artery to

descending aorta just distal to left
subclavian artery

Ductus Arteriosus

Frank Netter, MD Novartis

Ductus Arteriosus
Physiology

In fetal life, shunts blood from

pulmonary artery to aorta
At birth, increase in arterial oxygen
concentration constriction of

ductus

Ductus Arteriosus
Normal Closure

Functional closure

Normal anatomic closure

By 24 hrs of life

Complete by 2 months in 90%

Closure at 1 year in 99%

Patent Ductus Arteriosus

Pathophysiology

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

Clinical

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

X-ray Findings

Cardiomegaly

Enlarged left atrium

Prominent main pulmonary artery (adult)

Prominent peripheral pulmonary vessels

Prominence of ascending aorta

PDA

Patent Ductus Arteriosus

Why Left Atrium Is Enlarged

RA

LA

RV

LV

Patent Ductus Arteriosus

Calcifications

Punctate calcification at site of closed

ductus is normal finding
Linear or railroad track calcification at
of ductus may be seen in adults

site

with PDA

Patent Ductus Arteriosus

Prognosis

Spontaneous closure may occur

Patent Ductus Arteriosus

Complications

CHF

Failure to grow

Pulmonary infections

Bacterial endocarditis

Eisenmengers physiology with advanced

lesions

 Jet of signal loss showing continuous flow from the aorta to the MPA
consistent with sizeable PDA
MPA is severely dilated at level of PDA

SCMR

Auckland MRI

Auckland MRI

Whats the diagnosis?

2 yo old cyanotic female

Partial or Total
Anomalous Pulmonary
Venous Return

Cyanosis With Increased

Vascularity
Truncus types I, II, III
TAPVR
Tricuspid atresia*
Transposition*
Single ventricle

* Also appears on DDx of Cyanosis with Inc Vascularity

CHF In Newborn

Impede Return of Flow to Left Heart

Infantile coarctation
Congenital aortic stenosis
Hypoplastic left heart syndrome
Congenital mitral stenosis
Cor triatriatum
Obstruction to venous return from
lungs

TAPVR from below diaphragm

Two Types

Partial (PAPVR)

Mild physiologic abnormality

Usually asymptomatic

Total (TAPVR)

Serious physiologic abnormalities

Return of
blood from
lungs is by
four pulmonary
veins to LA
RA

LA

RV

LV

PA

Normal heart

Ao

PAPVR
General

One or two of four pulmonary veins may

drain into right atrium

Mild or no physiologic consequence

Associated with ASD

Sinus venosus or ostium secundum types

Return of
blood from
lungs is mostly
to LA
One vein
abnormally
connected to
right heart

RA

LA

RV

LV

PA

Ao

Frequently
associated with
sinus venosus
or secundum
ASD

Partial Anomalous Pulmonary Return

Auckland MRI

PAPVR
Korean Journal of Radiology

TAPVR
General

All have shunt through lungs R heart

All must also have R L shunt for
survival

Obligatory ASD to return blood to the systemic

All are cyanotic

Identical oxygenation in all four chambers

side

TAPVR
Types

Supracardiac

Cardiac

Infracardiac

Mixed

TAPVR

Supracardiac TypeType I

Most common (52%)

Pulmonary veins drain into vertical
vein (behind left pulmonary artery)
left brachiocephalic vein SVC
DDx: VSD with large thymus

Left Brachiocephalic vein

Left
superior
vena cava

Right
superior
vena
cava

Vertical
vein
Right
atrium
Pulmonary
veins
Frank Netter, MD Novartis

TAPVR-Supracardiac Type 1

 Frank Netter, MD Novartis

TAPVRSupracardiac
Type 1

TAPVR

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

TAPVR-Supracardiac Type 1

Blood from
lungs drains
into left
vertical vein
to L SVC

Blood moves
through L
brachiocephalic v
to R SVC

ASD provides R
L shunt to
allow
oxygenated
blood to reach
body (moderate
cyanosis)

RA

LA

RV

LV
Increased

PA

Ao

return to right
heart overloads
lungs
shunt vessels

TAPVRType ISupracardiac type

TAPVR

Cardiac TypeType II

Second most common: 30%

Blood from lungs coronary sinus or RA

Coronary sinus more common

Overload of RV CHF after birth

Increased pulmonary vasculature

20% of Is and IIs survive to adulthood

Remainder expire in first year

Coronary
sinus
Frank Netter, MD Novartis

TAPVR-Coronary Sinus-Type II

Pulmonary veins

Frank Netter, MD Novartis

TAPVR

Blood returns
from lung to RA
or coronary sinus

ASD provides R
L shunt to
allow
oxygenated
blood to reach
body (moderate
cyanosis)

RA

LA

RV

LV

PA

Ao

Increased

return to right
heart
overloads
lungs
shunt vessels

TAPVRType IICardiac Type

TAPVR

Infracardiac TypeType III

Percent of total: 12%

Long pulmonary veins course down along
esophagus
Empty into portal vein (more

common) or IVC

Vein constricted by diaphragm as it passes

through esophageal hiatus

Pulmonary
veins

Frank Netter, MD Novartis

Portal vein

TAPVR-Type III-Infradiaphragmatic

TAPVR

Infracardiac TypeContinued

Severe CHF (90%) 2 obstruction to

venous return
Cyanotic 2 right left shunt through
ASD
Associated with asplenia (80%), or
polysplenia
Prognosis=death within a few days

Blood returning
from lungs
pulmonary
veins which are
constricted by
diaphragm CHF

CHF vasculature

ASD provides
R L shunt to
allow
oxygenated
blood to reach
body (cyanotic)

RA

LA

RV

LV

PA

Ao

To portal v IVC RA

TAPVRType IIIInfracardiac type

TAPVR

Mixed TypeType IV

Percent of total: 6%

Mixtures of types I III

University of Minnesota

Frank Netter, MD Novartis

TAPVR