Download CONOTRUNCAL ANOMALIES A Case Based Review

CONOTRUNCAL
ANOMALIES
A Case Based Review
Beverley Newman M.D.
Professor of Radiology
Lucile Packard Children’s Hospital at
Stanford University
Thanks to Frandics Chan for supplying
• The outflow tract ( conotruncus) of the
tubular embryonic univentricular heart
begins as a common outlet
• Spiral flow through the arteriovenous
connection and differential conal growth
result in the creation of separate left and
right ventricular outflow tracts which
normally connect to the aorta and main
pulmonary artery respectively
Images from John
Partridge – cardiac
embryology
Conotruncal Anomalies
 Transposition
of the Great Arteries
 Double Outlet Right Ventricle
 Truncus Arteriosus
 Tetralogy of Fallot
Aberrant coronary arteries associated with conotruncal anomalies
R
L

Surgically relevant

Ao

Aberrant LAD anterior to
RVOT (4% of TOF)
Aberrant coronary in TGA
PA
PA
LAD
Courtesy Frandics Chan
Q1. The most common plain film
radiographic appearance of complete
transposition of the great arteries in the
newborn is:
1. Normal
2. Cardiomegaly and shunt vascularity
3. Egg on a string
4. Bulge of left superior heart border
5. Coeur en Sabot
Complete Transposition of the great Arteries
(D-transposition)
Congenitally Corrected Transposition
(L-Transposition) ( dextrocardia 25%)
Double Outlet Right Ventricle
Truncus Arteriosus
Tetralogy of Fallot
Q2 This configuration of the aortic arch is characteristic of
which of the following congenital cardiac anomalies
1.Truncus Arteriosus
2.Transposition of the Great Arteries
3.Congenitally Corrected Transposition
4.Double Outlet Right Ventricle
5.Tetralogy of Fallot
RV
RV
LV
LV
LV
RV
Transposition of the Great
Arteries

Discordant connections of the ventricles and
great arteries
 CCTGA- also atrioventricular discordance:
associated with VSD, ASD, tricuspid abnormality
(Ebstein’s),pulmonic stenosis, conduction
abnormalities
 TGA – associated with VSD, ASD, PDA,
pulmonary stenosis/atresia, Ao
coarctation/interruption, AV valve abnormalities
and RV hypoplasia or single ventricle
RV
RV
LV
LV
TGA with VSD and PDA
Q3 Which of the following surgical procedures are
used to repair Double Outlet Right Ventricle
1. Arterial switch
2. Rastelli
3. VSD closure and PA outflow tract patch
4. RV to PA conduit
5. Fontan
6. All of the above
Double Outlet Right Ventricle
Subaortic VSD
( 50%)
Doubly
Committed
VSD
Subpulmonic
VSD (30%)
( Taussig-Bing)
Remote/
uncommitted
VSD
Frank et al
Radiographics :
30;2010
Double Outlet Right Ventricle

Both great arteries arise predominantly from the
right ventricle. Both great arteries have
subvalvular conal muscle
 Associated with VSD, pulmonary stenosis or
atresia,TGA, mitral stenosis or atresia,
hypoplastic LV, straddling AV valves,
subaortic/aortic valve obstruction/coarctation
 May physiologically resemble TOF ( subaortic
VSD and PS); Transposition ( Subpulmonic
VSD); VSD( subaortic VSD); Hypoplastic or
univentricular heart
Double Outlet Right Ventricle
 Surgery:





Subaortic VSD – Rastelli tunnel through VSD
connecting LV with Ao
RVOT obstruction may necessitate patch similar
to TOF repair or RV – PA conduit
Subpulmonary VSD – Switch and VSD patch
Doubly committed VSD- Rastelli or switch
depending on LV /Ao relationship
Remote VSD – may need single ventricle
palliation and eventual Fontan
DORV with
subaortic VSD and
PS – like TOF
DORV with Transposition
and subpulmonic VSD
Ao
PA
Outlet
Septum
Aortic
Valve
VSD
Muscular
Septum
Mus.
VSDs
PA
Conal Muscle
DORV WITH
SUBAORTIC VSD AND
PS – RASTELLI REPAIR
DECR RV FX, TR
Gaca, A Radiology 2008. 248:1;44
FONTAN- COMMON PATHWAY FOR
VERY COMPLEX HEART DISEASE
13yo Asplenia, abnormal situs,
dextrocardia, unbalanced AVC with
hypoplastic LV, DORV, Lt sided
Glenn/Fontan
Q5 Which of the following syndromes are
most often associated with Truncus
Arteriosus
1. Di George Syndrome
2. Heterotaxy with Asplenia
3. Alagille Syndrome
4. Williams Syndrome
5. Down syndrome
TRUNCUS ARTERIOSUS
Collett Classification
Van Praagh Classification
TRUNCUS ARTERIOSUS
 Single
arterial vessel arises from the heart
which gives rise to aortic, pulmonary and
coronary arteries
 Association with VSD, truncal valve
abnormality, RAA, interrupted aortic arch
(11-14%), abnormalities of the mitral valve
and anomalous pulmonary venous
connections
Truncus Arteriosus Type 1- 22q11deletion
(~ 35% of cases)
2dy old Type 3 Truncus
LT
Alagille Syndrome
WILLIAMS SYNDROME
3d old
Heterotaxy with right
IsomerismAsplenia
Q4 Which of the four features of Tetralogy
of Fallot is the most consistently present
but also the most variable in severity
1.Overriding Aorta
2. Ventricular Septal Defect
3. Right Ventricular Hypertrophy
4. Pulmonary Outflow Obstruction
Tetralogy of Fallot
 Most
common cyanotic congenital heart
defect
 Anterior malposition of the conal septum
results in :overriding aorta; pulmonary
outflow obstruction; ventricular septal
defect and right ventricular hypertrophy
 Associated with VSD, Right aortic arch
( 25%), pulmonary atresia, PDA, major
aorticopulmonary collaterals, ASD, CAVC
Systole
Down S
CAVC
TOF
Diastole
RVOT
RV
LV
TOF post repair
RPA 2.1cm
LPA 1.3*1 – 2.5cm
TOF PA MAPCA’S
Tetralogy of Fallot,
absent pulmonary valve
Conotruncal Anomalies
Summary

Transposition of the Great Arteries
The aorta moves upward and to the right instead of
pulmonary artery
 Truncus Arteriosus
Truncal Septum fails to develop
 Double Outlet Right Ventricle
Arterial trunk divides but stays over on the right side
 Tetralogy of Fallot
Truncal septum deviates anteriorly
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