congenitally corrected transposition our...

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


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


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

congenitally corrected transposition our...

Documents