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Congenitally Corrected TranspositionOur Experience
W.J. BrawnCardiac Services
Joint conference in advances in Pediatric Cardiovascular Disease Management 2012
Historical illustration – Baron von Rokitansky
ccTGA Natural history is 50% survival to 40 years of age
66% of patients in heart failure by age 45
25% of ccTGA with NO assoc lesions will be in congestive heart failure by age 45
Rarely presents neonatally in severe CCF
Rarely survival to old age without symptoms
Not a benign condition with unsatisfactory results
from conventional repair
The problem of natural history
Conventional management
Conventional or physiological repair of ccTGA (RV systemic circulation)• Generally disappointing outcomes:
• Early mortality 10‐15% (up to 33% in infants)
• Continued attrition:• Mayo: 60% 10‐year survival (JTCVS 109:642, 1995)• Toronto: 75% 10‐year survival, 48% 20‐year survival (JTCVS
117:1190, 1999)• Brompton: 58% develop mod/sev TR during follow up (Br.
Heart J. 50:476, 1983)• Boston: 61% 15‐year survival. Ebstein malformation of TV or
TV replacement risk factors for death (Hraska V. JTCVS 2005)
TI
TV annulardilatation
RVdilatation
RVoverload
VSD shuntAbn. TV & TI
Vicious circle of increasing Tricuspid Valve regurgitation and increasing heart failure
Mechanism of right ventricular failure
Long term follow up post surgery for ccTGA with systemic RV
Our series confirms that in long term follow up, surgery in CCTGA with the right ventricle as systemic ventricle has a suboptimal survival and limited freedom of reoperation. There is an increased incidence of abnormal anatomy of the proximal coronary arteries. An important number of patients will need tricuspid valve replacement at either primary or later surgery. An important number of patients will need a pacemaker at any stage of observation. Death occurred mostly as a result of cardiac failure. (Bogers A.J.J.C. et al Jn. Cardiothoracic Surg. 2010)
Effect of morphologic LV pressure on RV geometry and TV regurgitation PAB (14 patients) – Decrease in TR from severe to moderate
• Severe TR decreased from 64% ‐ 18% of patients
• Sphericity index increased in LV, decreased in RV
LV to PA conduit (16 patients)
• TR increased from none to mild
Highlights the importance of septal shift in relation to TR in ccTGA
Kral Kollars CA. et al Am. J. Cardiol. 2010 March Ann Arbor
Impaired myocardial blood flow and coronary flow reserve of the anatomic RV• MBF assessed by dynamic PET scan with 13N ammonia
• 7 patients ccTGA
• 8 patients ccTGA plus associated anomalies
• 11 normal as controls
• Reduced RV function caused by mismatch between oxygen demand and supply
• RV remodelling with myocyte hypertrophy and fibrosis, with inadequate capillary growth may contribute to reduced nutritional supply and ventricular deterioration
Hauser M. et al Heart 2003 Munich
MRI evaluation of un‐operated ccTGA• Define patients with ccTGA who may need surgery
• Asymptomatic or mildly symptomatic patients may be selected by MRI Dobutamine stress scans for either operative or non‐operative management
• Allows more objective approach to selection for surgery
Dodge-Khatami A. et al Ann. Th. Surg. 2002 May Netherlands
• Natural History is 50% survival at 40 y
• Wide spectrum of morphology & associated lesions
• Anatomical Repair has become the benchmark
• Late outcomes are poorly understood
• Late morbidity poorly understood
• Do some groups perform better than others?
ccTGA – The problem
Concept developed as strategy to ‘train’ the LV in simpleTGA with a failing Mustard/Senning
Found that ccTGA patients are symptomatically improved due to reduction in TR
Extent of retraining assessed by RV:LV pressure ratios & LV wall thickness
Mee R. B. JTCVS 1986
Poirier N.C. JTCVS 2004
Winlaw D.S. Circulation 2005
Pulmonary artery banding
Winlaw D. Circulation 2005
BCH experience with PAB to train mLV
Winlaw D. Circulation 2005
Patient characteristics
TR no early or late improvement overall (p=0.26).
TR reduced in subset undergoing anatomic repair.
ccTGA TR no alteration in significant TR.
Patients >16 years not likely to achieve anatomic repair.
PAB can be an effective palliative procedure (? Reason).
Outcome of PA banding to train the mLV
Algorithm for the management of Congenitally Corrected Transposition
No TRNo RVF
No Associated Cardiac Anomalies Associated Cardiac Anomalies
AdultAlive and well
TVDysplasia
CCFPHT
PSVSDPulmonary
Atresia & VSD
TRRVF
Repair orReplace TV
DoubleSwitch
? CardiacTransplantation
? below 15 years PAB to train LV
and/or reduce TRPAB
DebandVSD closure
DoubleSwitch
VSD &PS
BalancedCirculation
IncreasingCyanosis
SystemicShunt
Cyanosis
RastelliSenning
Systemic shuntIn infancy
VSD closureLimiting LV>PA
conduit
PAB toTrain LV
No PHTPHT
Usually severeTR with CCF
PS resectionVSD closure
? Indication for Fontan Procedure
CP shuntPs resectionVSD closure
Delay interventionuntil symptomatic
(increasing cyanosis)
DoubleSwitch
? CardiacTransplantation
Key
TV – Tricuspid valve.
TR – Tricuspid regurgitation.
RVF – Right ventricular failure.
PAB – Pulmonary artery band
LV – Left ventricle.
CCF – Congestive cardiac failure.
PHT – Pulmonary hypertension.
VSD – Ventricular septal defect.
PS – Pulmonary stenosis.
CP shunt – Cavopulmonary shunt.
ccTGAMorphology and the Double Switch
ccTGA – Study Objective• Analysis of a complete cohort of anatomical repair of ccTGA over
a 19 year period
• Focus on:
• Late morbidity and re‐intervention
• Performance of the morphologic LV (mLV)
• Fate of the neo‐aortic valve
• Outcomes in high‐risk groups
ccTGA – Study Methods• 1991 to 2011
• Retrospective
• Single centre
• n = 113
• High risk = requiring intubation, ventilation and inotropic support pre‐operatively (n = 17)
• Exclusions:
• 13 awaiting repair
• 14 not suitable for repair
ccTGA – Associated lesions
* All in D/S group
Characteristic Number (%)Number of patients 113
Male / Female 73/40Median age at repair (years) 3.2 (25d ‐ 40y)Median weight at repair (kg) 14.3 (3.2 ‐ 61.4)
Usual atrial arrangement 100Situs inversus 10
Azygous continuation of IVC 4Left atrial isomerism 2
DORV 7Levocardia 88
Dextrocardia 23Mesocardia 2
VSD 89Ebsteinoid anomaly of TV 14
Severe TR 6*Pulmonary hypertension 6*
Coarctation / Arch hypoplasia 20Interrupted aortic arch 1Bicuspid Ao V/Pulm V 2/3
Quadricuspid PV 1Double orifice TV 2 (1 with straddling)
Heart block 15
ccTGA – Patient pathways
ccTGA – ResultsEarly Mortality: 5/113 (4.4%)
Late survival
RS(0/45) (0.0%)
Years post repair
Sur
viva
l
0 2 4 6 8 10 12 14 16 18 20
0.0
0.2
0.4
0.6
0.8
1.0
68 46 41 37 27 22 18 12 5 2 double_switch
42 38 37 29 23 13 11 6 4 rastelli_senning
Survival (Death) Post ccTGA repair
double_switchrastelli_senning
DS(5/68) (7.4%)
DS
RS
All low risk(2/96) (2.1%)
High risk(3/17) (17.5%)
p = 0.98
9 month old with profound heart failure
ccTGA – Poor mLV function at follow upn = 16 (14.8%)
Excluding early deaths
Mod/Good LV Poor mLVDS 42/63 (75%) 16/63 (25%)RS 45/45 (100%) 0/45 (0%)High Risk Gp 12/14 (80%) 3/14 (20%)≥Mod AR or AVR 7/92 (6%) 3/16 ( 19%)Previous PA Band 53/92 (58%) 7/16 (44%)Duration PA Band 554 days 579 days
P=ns
P=ns
P<0.01
P=ns
ccTGA – Freedom from death, transplantation or poor mLV function
DS
RS
p = 0.03Years
Sur
viva
l
0 2 4 6 8 10 12 14 16 18 20
0.0
0.2
0.4
0.6
0.8
1.0
68 42 36 33 22 18 14 7 3 2 double_switch
42 38 37 28 22 12 10 5 3 rastelli_senning
Freedom from death, transplantation or poor mLV function
ccTGA – Aortic regurgitation at follow up
DS RS≥ Mild AR 40/58 (70%) 8/38 (21%)≥ Mod AR 6/58 (10%) 0/38 (0%)AV Replacement 6/ 58 (10%) 1/38 (3%)
ccTGA – Impact of Aortic Root annuloplasty
Annuloplasty n=13
No Annuloplasty n=55
Freedom from AVR or > mild AR
Years
Sur
viva
l
0 2 4 6 8 10 12 14 16 18 20
0.0
0.2
0.4
0.6
0.8
1.0
56 35 30 27 22 18 14 7 3 2 not_repaired
12 7 6 6 repaired
ccTGA Re‐interventionsDS RS
AVR 6 1MV repair 1 0TV repair 3 0
RF ablation for Aflutter 4 0Multi‐site pacing 3 0
Residual VSD 3 0
LVOTO resection 0 4Senning Pathways 7 (3 balloon) 5 (4 balloon; 1 stent))
Pulmonary Arteries 11 (3 balloon/stent) 8(5 balloon)
RVOT enlargement 2 0RV‐PA conduit n/a 14 (2 balloon)
ccTGA – Freedom from re‐intervention
Years post repair
Sur
viva
l
0 2 4 6 8 10 12 14 16 18 20
0.0
0.2
0.4
0.6
0.8
1.0
68 37 33 30 24 19 15 9 2 double_switch
42 33 31 24 19 9 6 4 2 rastelli_senning
Freedom from Re-intervention Post ccTGA repair
double_switchrastelli_senning
p = 0.44
Freedom from re‐intervention
Years
Sur
viva
l
0 2 4 6 8 10 12 14 16 18 20
0.0
0.2
0.4
0.6
0.8
1.0
50 29 28 27 18 15 12 6 3 2 lecompte
18 12 8 6 5 4 3 1 posterior
ccTGA – Freedom from PA re‐intervention post Double Switch
p = 0.25
P=0.25
Freedom PA re‐intervention
Years
Sur
viva
l
0 2 4 6 8 10 12 14 16 18 20
0.0
0.2
0.4
0.6
0.8
1.0
50 29 28 27 18 15 12 6 3 2 lecompte
18 12 8 6 5 4 3 1 posterior
No Lecompte n=18
Lecompte manoevre n=50
P=0.25
Hemi‐Mustard – Bidirectional Glenn in the Double Switch
• 1994 – 2009 48 patients (1 hospital death)• Risk low – no late deaths• Prolonged conduit life• Reduced baffle sinus node complications• Technically simple• Long term outcome unknown• Pacing access is a problem
Malhotra S.P. et al J. Th. CVS Jan. 2011
Midterm results of cavopulmonaryanastomosis and hemi‐mustard
Anatomic Correction ccTGA
2004‐2011 8 patients (median 2.9 years)
Rastelli‐Senning ‐ 6
Arterial switch – 2
Hospital deaths – 2
Late 6 years – 1 conduit change and one patient with LV dysfunction
CP – can unload the RV & technically easier
Sojak V. – Hazekamp M. – E.J.C.Th.S. March 7th 2012
Concludes: “mid” term satisfactory results
Early prophylactic PAB in isolated ccTGA – PAB in infancy2001 ‐ 2009• 11 asymptomatic infants
• (7 neonates)
• PAB – 1.5 months (mean)
• Hospital deaths – 0
• Late deaths – 1
• Double switch – 1
Metton O. - Vouhe´ P. E.J.C. Th. S. 38. 2010
Aims: • Stabilise or improve TR• Allow for double switch
Determinants of LV function after anatomic repair
23 Rastelli – atrial switch
21 Double switch
2 Early deaths (4.5%)
0 Cardiac related late deaths
8 Deterioration in LV function (18%)
LV function deterioration related to pacing and prolonged QRS
? Value of re‐synchronization Bautista – Hernandez V. Am. Th. Surg. 2006 May Boston
Health related quality of life in patients with ccTGA
38 ‐ Anatomic repair
13 ‐ Conventional or no procedure
• Similar quality of life outcomes.
• Prolonged hospitalization – pacemaker risk for lower quality of life in anatomic repair group.
Gaies MG et al Jn. Th. CVS. 2011 July (Ann Arbor)
• Late Follow‐up in ccTGA reveals a significant morbidity
• The DS group do less well than the RS Group.
• Late mLV dysfunction and aortic regurgitation are important factors in the DS group
• High Risk Groups have particularly rewarding outcomes
• Currently 85% survival
• The majority (>75%) of survivors remain well and free of heart failure at 10 years
Birmingham – Conclusions 2011
Double Switch (an aside) – Shone’s Syndrome
• Patient 1• Died 2 years post operatively at cardiac transplantation
• Patient 2• Alive, well on medication 5 years post operatively
• Patient 3• After reversal of double switch died 6 months later, stroke whilst
on Berlin heart
• Patient 4• Well thriving 18 months post operatively
Outcome
Long term outcomes of surgically treated patients
Conventional repair
123 patients (1963 – 1996)
68% survival at 10 years
100% survival – Fontan (17 patients)
• Long term outcomes unsatisfactory
• TVR gave worse outcomes
• Consider Double Switch / Rastelli – Senning / Fontan
Hraska V. J. Th. CVS. 2005 January Boston