Download Transposition Complexes

“Everything you need to know
about transposition”
Paediatric Cardiology for Paediatricians
Birmingham
Sept 2010
Dr Dirk G Wilson
Consultant Paediatric Cardiologist
University Hospital of Wales
Remit
“Simple” transposition of the great arteries (TGA)
Transposition of the great arteries with ventricular septal
defect and pulmonary stenosis (TGA/VSD/PS)
Congenitally-corrected transposition of the great arteries
(ccTGA)
• Natural history
• Operative approach
• Long-term outcomes and surveillance
Sequential Segmental Analysis
Systemic
veins
Pulmonary
veins
Atrial
arrangement
Atrio-ventricular
connection
Ventricular
arrangement
Ventriculo-arterial
connection
Arterial
arrangement
Terminology
• Connections may be
– Concordant
• The expected, appropriate connection
– e.g. the morphologic LA connects to the morphologic LV
– e.g. the morphologic LV connects to the aorta
– Discordant
• The connection is inappropriate
– e.g. the morphologic LA connects to the morphologic RV
– e.g. the morphologic LV connects to the PA
So…
• Atrial situs solitus, A-V concordance, V-A
discordance
Simple TGA
• Atrial situs solitus, A-V discordance, V-A discordance
Congenitally-corrected TGA (ccTGA)
Simple Transposition of the
Great Arteries
Case
• 41+1/40
• Cyanosis from birth – no response to
oxygen
• Taken to local NICU (Swansea)
– Intubated
– Ventilated
Case
• Found to be hypoxic, hypotensive and
acidotic
• Treated with
– Fluids
– Bicarbonate
– Inotropes (dopamine, dobutamine,
adrenaline)
Case
• Local echo performed
• Commenced on PGE
• Saturations remained low despite
escalating doses of PGE (up to 160
ng/kg/min)
Echo
Echo
Bifurcating PA
from LV
Echo
Ao
PA
LA
Parallel great arteries
long axis view
Echo
Ao
PA
Ao anterior and to R
of PA on short axis
view
PDA flow
Echo
Hmm
• What is your assessment?
– What is the underlying diagnosis
– Why is the baby so compromised?
– What is exacerbating the situation
• What needs to be done?
BAS
Septostomy
balloon filling LA
Post-BAS
Simple Transposition of the Great Arteries
• There is
– Normal atrial arrangement
– AV concordance with VA
discordance
• Also known as…
– TGA
– Complete transposition
– D-TGA (dextroposition of
the bulboventricular loop,
bringing the RV to the right
side, and the aorta usually
to the R and anterior to the
PA)
• Associations
– PFO/ASD
– Small VSD
– Coarctation of the aorta
TGA
• Natural history
– Average life expectancy for a newborn with
transposition is 0.65 years
– Survival
• At 1 year ~10%
• Dependent on adequate mixing between the
two circulations
– Atrial
– Ventricular
– Arterial
TGA – Modern Management
• Prostaglandin E administration
• Balloon atrial septostomy
• Arterial switch operation
– Described by Jatene (1975)
– Widely practised in most UK centres from
the early 1990s
• Survivors now entering ACHD services
Arterial Switch Operation
Arterial Switch Operation
From: Cardiol Young 2008; 18:124–34
ASO - Survival
• 30 day survival = 99.2%
• 1 year survival = 96.9%
• Complications
– RV outflow tract distortion
– Coronary artery stenosis or occlusion
– Reintervention for
•
•
•
•
Branch PA stenosis
Aortic root enlargement (“neo-aortic dilatation”)
Aortic regurgitation
Coronary complications
CCAD.org
Echo Post-Switch
Enlarged neoaortic root
CT Angiogram Post Switch
PAs draped across the Ao
Dilated Ao root
CT Angiogram Post Switch
CT Angiogram Post Switch
CT Angiogram Post Switch
Post Switch – Clinic Assessment
• History
–
–
–
–
Exercise performance
Chest pain
Palpitation
Syncope
• Examination
– Expect ESM ULSE referred to back
– Listen for AR murmur
Post Switch – Clinic Assessment
• ECG
• Echo
–
–
–
–
–
Function
Regional wall motion abnormalities
AR
Branch PAs
TR velocity (surrogate for branch PS)
• Exercise test
– Before high school sports participation
– To investigate symptoms
Post Switch – Clinic Assessment
• Consider need for
– MRI or CT angiogram
– Myocardial perfusion scan
– Coronary angiography
• 324 patients undergoing coronary angiography
post ASO
• ~7% had coronary obstruction (>50% of lumen)
Circulation. 2003;108:II-186
In Practice
• Investigate coronaries if
– Exertional chest pain
– Unexplained LV dysfunction
• Encourage “heart healthy” lifestyle
in all patients
Simple TGA – Historical Management
• Atrial switch procedure
– Senning operation (1958)
– Mustard operation (1963)
•
•
•
•
Systemic venous blood baffled to MV
Pulmonary venous blood baffled to TV
Senning – autologous material
Mustard – Dacron baffles
Atrial Switch
• Problems
– Extensive atrial
surgery
– Risk of rhythm
problems
– Risk of baffle leaks
and obstruction
– RV remains the
systemic ventricle 
late heart failure
Atrial Switch
• Only a small number of these patients
are in the paediatric age group
• Adult congenital clinics will have to deal
with this legacy
TGA with VSD and PS
TGA with VSD and PS
• Arterial switch not
possible
• Strategy
– BAS
– BT shunt if
needed
– Rastelli operation
(or similar)
Rastelli Operation
• VSD patch directs blood from LV to Ao
• PV is oversewn, PA is detached
• Valved conduit between RV and PA
MMCTS (January 23, 2009).
doi:10.1510/mmcts.2007.003046
Rastelli Operation
• Advantage: LV is the systemic ventricle
• Problems
– Conduit degeneration and stenosis
– Risk of atrial and ventricular arryhthmias
– Risk of sudden death, especially with
conduit degeneration
Réparation à l'Etage Ventriculaire
25 yr survival = 80%
REV Operation
• Elements
– LeCompte manoeuvre - brings the pulmonary trunk forward
– Resection of outlet septum – allows reasonable alignment
between LV and Ao
– Direct implantation of pulmonary trunk into RV (no conduit)
– Lower likelihood of reoperation
MMCTS (January 23, 2009).
doi:10.1510/mmcts.2007.003046
TGA with VSD and PS
• OPD Surveillance
–
–
–
–
–
–
Exercise capacity
Palpitations
ECG (QRS duration)
RV pressure, size and function
RVOT status (obstruction, PR)
Timing of conduit replacement
Congenitally Corrected
Transposition of the Great
Arteries
CCTGA
• Rare - <1% of CHD
• Heterogeneous population
– Clinical features are variable
– May present at any age
• Fetus
• Child
• Adult
ccTGA
• There is
– Normal atrial arrangement
– AV and VA discordance
Ao
• Also known as…
– cTGA
– Ventricular inversion
– L-TGA (laevoposition of the
bulboventricular loop,
bringing the RV to the left
side, and the aorta usually
to the L and anterior to the
PA)
– Double discordant
transposition
PA
mLA
mRA
mLV
mRV
ccTGA
• Associations
–
–
–
–
Dextrocardia
VSD (70%) – usually perimembranous
Pulmonary stenosis (40%) – often subvalvar
Tricuspid (systemic) valve abnormalities (90%)
• Significant leak in up to 90% of patients
• Valve may be “Ebstein-like” with displacement towards
the cardiac apex
– Rhythm
• AV node + His bundles displaced – course is elongated
• Dual AV nodes may be present
• Risk of heart block 1-2% per year (+ increased risk with
surgery)
ccTGA - Variants
ccTGA – Presentation
• Murmur ± cyanosis – due to associated
lesions
• CCF – due to
– VSD
– Systemic ventricular failure
• Heart block
• Incidental finding
• NB – may present from fetus to mature
adult
ccTGA – ECG
•
•
•
•
R&L bundles are inverted along with ventricles
Septal activation occurs R to L
Q waves in R precordial leads, but not in V5/V6
Heart block may be present
ccTGA – Echocardiogram
Apical
displacement of L
AV valve (TV)
ccTGA – Echocardiogram
ccTGA – Echocardiogram
Parallel great arteries
ccTGA – Clinical Challenges
• Systemic ventricular failure
• Systemic AV valve regurgitation
(SAVVR)
• Heart block
ccTGA – Heart Failure
• “Simple” ccTGA
– 1/3 have CCF by the 5th decade of life
• “Complex ccTGA (associated lesions)
– 2/3 have with CCF by 5th decade
despite medical and surgical
interventions
ccTGA – Heart Failure
• Factors
– RV performing systemic workload
– RV coronary supply – attenuated
coronary reserve
• Effect of volume load (shunt, AV
valve leak)
– Pacing
ccTGA – Heart Failure
• Treatment
– ACE inhibitor
– -blockers – use with caution (heart block)
– Surgery to improve SAVVR
ccTGA – SAVVR
• Contributing factors
– Tricuspid valve bears the systemic load
– Altered RV geometry (affecting leaflet
apposition)
– Ebstein-like malformation
ccTGA – SAVVR
• Treatment
–
–
–
–
ACE inhibitor
-blockers – use with caution (heart block)
Surgery
Repair or replacement of SAVV
• Intervention is needed before significant SV
dysfunction sets in
• High risk of heart block
ccTGA – Heart Block
• Risk is 1-2% per year
• Pacing may be required
• Risk of worsening SV function (?due to
shift of septum, ??V-V interaction,
→effect on TV)
ccTGA – Surgical Strategy
• ccTGA with no associated lesions
– Consider double switch in younger patients –
requires “training” of LV with PA band – needs to
be a clear indication for intervention
• ccTGA with VSD
– Consider VSD closure
– Consider double switch
• ccTGA with VSD and PS
– Consider Rastelli–Senning operation
ccTGA – Surgical Intervention
•
Double Switch – Paris Group (Planché) – 20 patients
– Actuarial survival at 10 years was 100%
– Freedom from reoperation at 5 and 10 years were 93% and 77.4%,
respectively
EJCTS 2009;35:879-884
– All patients class I or II
Transposition Complexes
• Summary
– Varied anatomical spectrum
– Varied management
– Simple TGA
• Modern management should produce good
outcomes
• Concern about atrial switch outcomes
– TGA with VSD and PS
• “Like a bad ToF with a conduit”
– ccTGA
• “Like MR with a bad ventricle” and a high risk of
heart block