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Transcript
LONG-TERM RESULTS OF
ATRIAL SWITCH IN TGA
MARKO TURINA
UNIVERSITY HOSPITAL
ZURICH, SWITZERLAND
Åke Senning, 1915-2000
First description of atrial correction of TGA
Senning, Opuscula Medica (!) 1958
Correction of TGA at the University
Hospital Zurich, 1962-2000 (493 pts.)
Atrial vs. arterial repair
35
No. Patients
30
25
20
15
10
5
0
Atrial repair
Arterial repair
Correction of TGA at the University
Hospital Zurich
Age at the time of correction
140
months
120
100
80
60
40
20
0
1962
1966
1970
1974
1978
1982
1986
1990
1994
Genoni et al., 1999
TGA: 32 years of atrial repair
Actuarial survival in hospital survivors
(follow-up 95.3 % complete)
%
100
80
60
40
20
0
0
5
overall
10
15
before 1978
after 1978
20
years
Genoni et al., 1999
TGA: 32 years of atrial repair
Actuarial survival in hospital survivors
% survival
100
80
60
40
20
0
0
5
10
15
20
25
years
overall
simple
complex
Genoni et al., 1999
ATRIAL CORRECTION OF TGA
Cause of late death
University Hospital Zürich, 1962 - 1987 (33/239
patients)
Malignancy
3.1%
Pulmonary
embolism
3,1%
Accident
6,3%
Heart failue
62,5%
Unknown
3,1%
Sudden
21,9%
FAILING RIGHT VENTRICLE AFTER
ATRIAL CORRECTION OF TGA
•  Does this disease truly exists?
•  Is it inevitable in majority of atrial
repair survivors?
•  Which are predictors of RV failure?
•  Possible causes?
•  Treatment options?
•  Magnitude of the problem?
FAILING RIGHT VENTRICLE AFTER
ATRIAL CORRECTION OF TGA
Time of onset (after 1st operation)
5
7.7 % of all
corrections
Patients
4
3
2
1
0
2
4
6
8 10 12 14 16 18
Years after 1st operation
20
0
Zurich University Hospital, 17/220 patients operated
1964-1985, follow-up 95.3 % complete
RV failure after atrial correction for
TGA as function of age at correction
hazard function
5
Differences are not
significant!
% / YEAR
4
3
2
1
0
2
1
2
1
3
1
2
0
1
2
1
2
4
0 2
4
1
3
6
0
T
I
M
E
(
M
O
N
T
H
S
)
FAILING RIGHT VENTRICLE AFTER
ATRIAL CORRECTION OF TGA
Surgical options
•  Correction of all residual anomalies.
•  Restoration of AV synchrony (DDDR or
biventricular pacing).
•  Repair of systemic AV valve.
•  Banding + arterial switch (Mee’s technique)
•  Damus-Stansel-Kaye procedure (PA-Aorta
anastomosis, RV- PA homograft)
•  Heart transplantation
FAILING RIGHT VENTRICLE AFTER
ATRIAL CORRECTION OF TGA
Boston experience: Chang et al, Circulation 1992;86:II-140-9
•  10 patients after Mustard or Senning repair
•  Anatomic correction in 5 pts. (Arterial
switch in 3 and Damus-Stansel-Kaye in 2)
•  Heart transplantation in 5 pts.
•  Results: 1 early death (switch), 90%
survival @ 27 months
•  Complications: 3 AI in switch group with 1
AVR; 1 lymphoma in TX group
SWITCH CONVERSION LATE AFTER
ATRIAL REPAIR FOR TGA
Cochrane et al, Ann Thor Surg 1993;56:854-62
•  24 patients after Mustard or Senning repair
•  Direct conversion in 4 pts. with 1 early
death
•  PA banding in 20 pts. : 3 deaths, 2
unsuitable for correction, 15 suitable.
•  Staged switch in 12 with 2 deaths
•  Late survival of switch conversion 80 % @
1 year, majority with improved LV function
Roger Mee’s results in patients with previous
atrial correction of TGA
Poirier N. C. et al.; J Thorac Cardiovasc Surg 2004;127:975-981
FAILING RIGHT VENTRICLE AFTER
ATRIAL CORRECTION OF TGA
Possible causes
•  Inherent inability of RV to support systemic
circulation for the whole life span
•  Damage to the RV due to long-standing
cyanosis and volume overload (“late
corrections”)
•  Perioperative damage to the right ventricle
(deficient myocardial protection)
•  Atrial dysrhythmias
•  Tricuspid valve incompetence
PROBLEM AREAS OF ATRIAL
SWITCH FOR TGA
Related to surgical technique:
•  SVC or IVC stenosis
•  Pulmonary vein stenosis
•  Atrial dysrhythmias
Related to the method:
•  Tricuspid valve incompetence
•  Failure of systemic (right) ventricle
Prevention of pulmonary vein stenosis after atrial
correction:
Augmentation of pulmonary venous atrium with
autologous in-situ pericardium
Systemic
AV valve
SVC
IVC
Pulmonary atrium
opened
Prevention of pulmonary vein stenosis in atrial
correction:
Augmentation of pulmonary venous atrium with
autologous in-situ pericardium
SVC
Autologous in-situ
pericardium
attached to its
blood supply
IVC
LATE RESULTS IN ADULT SURVIVORS
OF ATRIAL TGA CORRECTION
Puley et al, Am J Card 1999;83:1080-4
•  86 patients >18 years old
•  Late deaths: 8 pts. (9 %)
•  Late heart failure in 9 pts. (10 %)
•  Atrial arrhythmia's in 73 % of survivors
•  Pacemaker implants in 22 %
Authors’ conclusion: these patients remain at
risk for premature death, supraventricular
tachycardia, and congestive heart failure
(a) Freedom from severe systemic ventricular dysfunction after surgical repair of TGA. (b)
Freedom from severe AV-valve insufficiency after surgical repair of TGA. TGA, transposition
of the great arteries.
Görler H et al. Interact CardioVasc Thorac Surg
2011;12:569-574
Published by European Association for Cardio-Thoracic Surgery. All rights reserved.
ATRIAL CORRECTION OF TGA
University Hospital Zürich, 1962 - 1997
231 patients, average follow-up 13.4 years, (158
patients @10, 22 @ 20 years)
NYHA III/IV
5%
NYHA II
29%
NYHA I
66%
Genoni et al., 1999
ATRIAL CORRECTION OF TGA:
INTELLECTUAL DEVELOPMENT
University Hospital Zürich, 1962 - 1997
205 patients, average follow-up 10.1 years
SPECIAL
CLASSES
13%
RETARDED
2%
NORMAL
SCHOOL
85%
Genoni et al., 1999
ATRIAL CORRECTION OF TGA:
Present occupation
University Hospital Zürich, 1962 - 1997
(82 adults)
Clerical work
44%
University
graduates
13%
No profession
1%
Manual labor
41%
Marital status and births: comparison of women who had undergone atrial repair with a
control group consisting of 26 year old women living in Switzerland in 1993.
10 live births in TGA survivors, all free of heart malformations
Genoni M et al. Heart 1999;81:276-277
Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.
ATRIAL CORRECTION OF TGA:
INCIDENCE OF PACEMAKER IMPLANTS
University Hospital Zurich, 1962 1987 (239 Patients)
Incidence of implants
12
10
8
6
4
2
0
0
1
2
3
4
5
6
7
8
9
Years postop.
10 11 12 13 14 15
(a) Freedom from loss of sinus rhythm after surgical repair of TGA. (b) Freedom from
pacemaker implantation after surgical repair of TGA. (c) Freedom from right bundle branch
block after surgical repair of TGA. TGA, transposition of the great arteries.
Görler H et al. Interact CardioVasc Thorac Surg
2011;12:569-574
Published by European Association for Cardio-Thoracic Surgery. All rights reserved.
Conduction system in
Senning’s correction of TGA
Avoid sutures
in the
vicinity of AV
and SA node
TRANSPOSITION OF THE GREAT
ARTERIES
Why has arterial correction replaced the atrial
method for total correction of TGA?
•  Total correction can be performed at
neonatal stage
•  No “interval mortality” after Rashkind
•  Lower operative mortality and smoother
postoperative course
•  Technically less demanding
•  Left ventricle in systemic circulation
“Double switch” in corrected TGA: Senning atrial
correction and arterial switch, to restore appropriate
ventricles to systemic and pulmonary circulation.
ARGUMENTS FOR A (LIMITED) UTILIZATION
OF ATRIAL CORRECTION
•  Senning’s atrial correction has accumulated > 30
years experience; long-term outlook for arterial
switch is less well known (neoaorta, coronaries,
reoperations).
•  Technical problems of atrial correction (stenosis
SVC or pulmonary veins) are avoidable.
•  Failure of systemic ventricle is not obligatory.
•  Atrial correction was performed in older children with
long-standing cyanosis which might have caused
late heart failure.
•  Dysrhythmias remain a problem in atrial correction
Present Limited Role Of
Atrial TGA Correction
•  TGA presenting later in life with normal PA
pressure. Long-term advantages of
banding + AP shunt followed by arterial
switch are unproven.
•  Double switch in some patients with
corrected transposition (VSD or PS).