Download The Adult with Transposition

3/5/2015
Forms of Transposition
The Adult with
Transposition
•
L-Transposition of the Great Vessels
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D-Transposition without pulmonary stenosis
Do two wrongs make a right?
•
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Atrial Repair
−
Anatomic repair (arterial switch)
D-Transposition, VSD, pulmonary stenosis
−
Figure 3. Relative survival of patients and patient groups.
Nieminen H et al. Circulation 2001;104:570-575
Rastelli repair
L-transposition of the Great
Vessels
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Rare: Less than 1 % of congenital heart disease
•
Atrioventricular discordance and ventriculoarterial
discordance
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If no major associated anomalies, survival to
adulthood without intervention is common
•
High frequency of major anomalies that can
dominate early history
Copyright © American Heart Association
L-TGV: Two wrongs
Normal vs. L-TGV
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L-transposition: tricuspid valve closer to apex
L-transposition: mitral-pulmonary valve continuity
L-transposition
•
L-transposition
Associated anomalies
−
VSD in 70%
−
PS 40%
−
Systemic AV valve abnormalities
Ebstein’s Anomaly
Progressive Tricuspid Regurgitation
−
•
Conduction system abnormalities
−
Dual AV node anatomy and abnormal His
bundle
−
Progression to AV Block estimated at 2% per
year
Progressive dysfunction of systemic right ventricle
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L-TGV: ECG
L-TGV: Echocardiography
•
Tricuspid valve closer to cardiac apex
−
•
Mitral-pulmonary valve continuity
•
Aorta usually anterior and to the left of pulmonary
artery
L-transposition: tricuspid regurgitation
L-transposition: right ventricular function
RV failure
D-TGV
•
•
In those without associated lesions, some degree
on CHF in about 1/3 by fifth decade
−
•
Importance of tricuspid regurgitation
Role of tricuspid regurgitation
Frequency greater in patients with previous heart
surgery
Ventriculoarterial discordance
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D-loop, RV on right side of heart
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Ao tends to be on right and
anterior
•
Cyanosis
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Associated lesions
•
Role of PS
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Atrial Repair
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Atrial repair
Senning 1958, Mustard later
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Baffle atrial blood to contralateral AV valve
Atrial repair
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Good early results
•
Late complications
−
−
Sinus node dysfunction;
sinus rhythm in 77% at 5
years, 40% in 20 years
Atrial flutter may be
marker of sudden death
Atrial repair
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Late ventricular dysfunction and tricuspid regurgitation
•
Actuarial survival 80% at 20 years
•
Evaluation of RV function difficult due to different
architecture of RV
•
Baffle leaks and obstruction (SVC>IVC>pulmonary
venous)
•
Pulmonary hypertension more frequent in those
operated at > 2 years of age
Atrial repair
•
Actuarial survival
•
Mortality 1-5 %
•
Late sudden death
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High frequency of good functional class (80% symptom
free with NYHA Class I)
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Progressive increase in RV dysfunction, TR, arrhythmias
−
Atrial repair
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Loss of sinus rhythm
•
Increase in atrial
arrhythmias
•
Late sudden death
2.5% early mortality, 2.5% late mortality
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Atrial repair of D-TGV
Atrial Repair: Echo
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•
Evaluate right (systemic) ventricular function
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RV size and contractility (often subjective)
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Tricuspid regurgitation
Evaluate for baffle obstruction
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Systemic venous (more common)
−
Pulmonary venous
Atrial repair: Pulmonary venous baffle
Atrial repair: Pulmonary venous baffle
Atrial repair: Tricuspid regurgitation
Arterial switch
•
Introduced by Jatene in 1976
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Transect great arteries,
transpose them and move
coronaries
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Distortion of branch
pulmonary arteries, aortic
insufficiency, coronary
occlusion early
postoperatively
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Arterial switch
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Advantage: Left ventricle is the systemic
ventricle
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Substantial morbidity and mortality in early
experience
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Recent results much improved, but long term
results are still evolving
Surgical mortality
•
Only studies are
retrospective
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High early mortality
with arterial switch,
probably better long
term mortality
•
Increased mortality
with single or
intramural coronaries
Figure 1. Top, Actuarial survival in 1200 patients who had ASO. Numbers indicate number of patients
observed at beginning of interval.
Losay J et al. Circulation 2001;104:I-121-I-126
Copyright © American Heart Association
ASO: Late complications
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Late death relatively rare
•
Branch pulmonary stenosis
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Progressive aortic insufficiency
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Evaluate for coronary occlusion
Aortic insufficiency
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Figure 3. Actuarial survival free from grade II or more AI (Ao insufficiency) for 1095 survivors.
Losay J et al. Circulation 2001;104:I-121-I-126
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19 year-old S/P arterial switch
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Asymptomatic but with increasing left ventricular size
Copyright © American Heart Association
Arterial Switch: Aortic Insufficiency
Arterial Switch: Aortic Insufficiency
Arterial switch
“Complex” D-TGA
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Probably lower risk of late death, but population is
growing older
•
Often asymptomatic in presence of progressive
disease
•
Increased risk of coronary findings later in life
which are difficult to visualize echocardigraphically
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Most frequent complex
lesion is with ventricular
septal defect and
pulmonary (valvar and
sublalvar) stenosis
•
Most common mode of
repair is the Rastelli
procedure
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Rastelli repair
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Mean age (initially) age 4 years
•
Current survival 93% at 20
years, but early was 52% at 20
years
−
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Rastelli
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Late sudden death
Reoperation due to progressive
stenosis of RV-PA conduit
Rastelli: reintervention
“Complex” D-TGA: Rastelli
Surgical issues—recurrent
LVOTO, conduit
obstruction, arrhythmias,
late mortality
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Perioperative heart block
−
Straddling tricuspid valve
Rastelli: Echo
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Evaluate for both LV and RV outflow obstruction
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Evaluate for ventricular dysfunction
•
Investigate for residual VSD
“Complex” D-TGA: Rastelli
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“Complex” D-TGA: Rastelli
“Complex” D-TGA: Rastelli
Transcatheter valve
replacement
“Complex” D-TGA: Rastelli, residual VSD
Transcatheter valve
replacement
“Complex” D-TGA: Rastelli
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“Complex” D-TGA: Rastelli
Suprasternal notch view for branch pulmonary arteries
“Complex” D-TGA: Rastelli
Prognosis in Adults
Good
Atrial Septal
Defect
Patent Ductus
Arteriosus
Pulmonary
Stenosis
Ventricular Septal
Defect
Intermediate
Uncertain/Poor
Aortic Stenosis
D-Transposition
(arterial switch)
Tetralogy of Fallot L-Transposition
D-Transposition
(Senning/Mustard
)
Ebstein’s
Anomaly
Single Ventricle
Coarctation
Mortality-->Insurance
Lesion
Late Mortality
(%)
Mortality
Ratio
Underwriting
ASD/PDA/PS
VSD
CoA
Aortic Sten.
ToF
Senning
Single Vent.
5-12
20
16
15
14
24
85
70-200
667
320
375
350
480
>2800
100
100-200
100-300
225-400+
200-400
Declined
Declined
Cognitive issues
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Cognitive function probably diminished
•
Lower than average scores on
neuropsychological testing as adolescents
•
Many receive remedial academic or behavioral
services during school years
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Pregnancy in TGA
Pregnancy
Lesion
Success
rate
Complications
Reference
L-TGV
83%
CHF with valve
replacement after
delivery
JACC 33:1692 (1999)
Atrial
Switch
82%
Arrhythmias 22%,
other 80%
Eur Ht J 26:2588 (2005)
Arterial
Switch
13/17
(76%)
VT, valve
thrombosis
AJC 106:417 (2010)
Rastelli
50%
Increased subaortic stenosis
Aust NZ Obstet Gyn 45:243
(2005)
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Prognosis dictated by ventricular function and
arrhythmias
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Thorough evaluation of rhythm particularly in
atrial switch and L-TGV patients
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Complications of valve anticoagulation
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Increased risk of congenital heart disease in
offspring
There are more adults than children
with congenital heart disease in the
U.S…
…boldly going where no group
of patients has gone before.
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