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Adult Congenital Heart Centre & National Centre for Pulmonary HypertensionRoyal Brompton Hospital
National Heart & Lung Institute, Imperial College, London, UK
Cardiac Surgery and Congenital Heart Disease:Where are we in 2016 ?
Michael A. Gatzoulis
37th Hellenic Cardiac Society Meeting, Athens, October 2016
Congenital Cardiac Surgery in 2016
Historic Perspective
Magnitude of the problem
Native lesions
Re-operations
End-stage ACHD
Royal Brompton Contemporary ACHD Surgery Study
Robert Gross: Patent Arterial Duct for heart failure 1939; Coarctation Repair for severe hypertension 1945
Blalock-Taussig Shunt: For severe cyanosis 1945
Alfred Blalock Helen Taussig Vivien Thomas
Russel Brock: ‘Lord Brock of Wimbledon’ Closed Pulmonary Valvotomy/infudibulectomy 1948 For severe cyanosis
Walt Lillehei John Kirklin
Repair of Tetralogy: Lillehei 31st Aug 1954 Kirklin 1955 For severe cyanosis
Donald Ross Extracardiac Valved Conduit 1966
Senning: 1958 Mustard: 1964
Lillehei used ‘Human Cross-Circulation’ First Case 31st August 1954
The first 10 patients died – How could he carry on? The only operation described with a potential mortality of 200% A parent lost not only their child but also their spouse!
Congenital Heart Disease Interventions in the UK
http://nicor4.nicor.org.uk/CHD/an_paeds.nsf/WMortality?Openview
0
500
1,000
1,500
2,000
2,500
3,000
3,500
4,000
4,500
5,000
5,500
6,000
Number[n]
2000
2001
2002
2003
2004
2005
2006
2007
2008
2009
2010
2011
2012
Year
Surgery + 43%
Intervention +130%
0
500
1,000
1,500
2,000
2,500
3,000
3,500
4,000
4,500
5,000
5,500
6,000
Number
2000
2001
2002
2003
2004
2005
2006
2007
2008
2009
2010
2011
2012
Year
Paediatric Interv. +63%
Adult Interv. +600%
Brompton Activity: Financial Year 2013 - 2014
• Congenital Heart Disease Operations 565 (Children <16yrs – 420) • Interventional Cardiac Catheterisation Procedures 365 (Children <16yrs – 242) (Infants 0 – 12 months – 87) • Total 930
• Hybrid, Ablations, Pacing, Implantables not included (>200)
Webb & Gatzoulis Circulation 2007
Native CHD lesions presenting in adulthood: ASD
Closure of ASD: Reverse Remodelling
Kort et al JACC 2001
0
10
20
30
40
50
60
70
80
90
100
0 1 2 3 4 5
< 40 yrs at repair (n=110)
> 40 yrs at repair (n=103)
Time from repair (yrs)
Free
dom
from
AF/
Fib
% p
atie
nts
Gatzoulis et al NEJM 1999
Surgical closure of ASD: The Toronto Series
Murphy et al NEJM 1990
Surgical closure of ASD: The Mayo Clinic Series
Native CHD lesions presenting in adulthood: Coarctation of the aorta
Coarctation StentingRBH Experience 2004 to 2015
Patients (n) 147 (83 male) Age (yrs) 25 ± 15
Native CoA (n) 96/147 (65%) - Atresia (n) 24/96 (25%)
No deaths; low (13%) complication rate
Slides courtesy of Anselm Uebing
Aortic Valve Surgery Transcatheter Aortic Valve Implantation (TAVI)
Aortic Valve Replacement (AVR)
Aortic Valve Repair
Biological
Stentless
Homograft (Ross)
Stented
Mechanical
Transfemoral Xenograft Sutureless Conventional Transapical
Slide courtesy of Darryl Shore
Slide courtesy of Darryl Shore
The Ross Procedure
Slide courtesy of Darryl Shore
Exo-stentDeveloped by: Prof John Pepper
Prof Tom Treasure & Mr T Galsworthy
Criteria for PV replacment • CTR >50%
• QRS prolongation
• RVESV ⇓ with severe PR
• Change in MVO2
• Symptoms (SOB, fatigue, arrhythmia)
Re-operations in ACHD (and timing): PVR
Babu-Narayan et al Circulation 2007 & 2014
Babu-Narayan et al, Circulation 2014
Surgical Pulmonary Valve Replacement Late after TOF Repair
Bonhoeffer et al, JACC 2002
PVR late after repair of Tetralogy Transcatheter PV implantation
Major advance Size of the valve and PV “annulus” may be a problem for the adult patient RVOT aneurysms can not be addressed
ACHD Interventions at RBH
0
20
40
60
80
100[%]
2004/2005 2012/2013
ASDPFOVSDPDACoAPA stentPSPPVIrare
40 %
5 %
20 %
15 %
N=239 N=168
Slides courtesy of Anselm Uebing
Raissadati et al Circulation 2015
Long-term survival after surgery for CHD
13876 operations
1953 to 2009
FU 98% complete
Peri-op mortality from 7% ! 3%
Raissadati et al Circulation 2015
Long-term survival after surgery for CHD
Mortality in ACHD
Diller/Kempny et al., Circulation 2015
• 6,969 adult patients (age 29.9±15.4 years)
• FU between 1991 and 2013, mean of 9.1 years
• 524 patients died
• Patients with Eisenmenger syndrome, complex
CHD and Fontan physiology had much poorer
survival.
Fontan: TCPC Conversion/Transplantation ? Indications and timing
Mavroudis et al J Thorac Cardiovasc Surg 2001
‘Fontan population’
Model assumptions: stable annual number of Fontan candidates, exponentially increasing deployment of surgical techniques within the first ten years after publication; stable annual mortality of 2%; annual AP to TCPC conversion rate of 1%. Age at first Fontan procedure of 2 years. Dashed area: patients after TCPC conversion.
Slide courtesy of Aleksander Kempny
Conclusions - ‘Ideal Fontan, 2016’
HR for mortality P-value SourceOperated >2001 (ECC) 0.15 0.04 0.61 0.008 KP, JACC, 2015Age at Fontan ≤7y 0.37 0.83 0.18 0.012 YD, Circ, 2015Female 0.40 0.77 0.22 0.004 YD, Circ, 2015
NYHA class I/II 0.10 0.37 0.03 <0.001 RC, JACC CV Img, 2016Satisfactory CPEX - - - - SMF, Congenit Heart Dis, 2011
ComplicationsºLiver cirrhosis 13.00 6.23 27.10 <0.001 KP, JACC, 2015Ventricular impairment* 4.40 1.20 16.90 0.03 RC, JACC CV Img, 2016AVV regurgitationº - - - - RC, JACC CV Img, 2016Renal failure 3.35 2.45 4.47 <0.001 KP, JACC, 2015Plastic bronchitis - - - - KRS, JAHA, 2014PLE 1.97 1.48 2.63 <0.001 KP, JACC, 2015Arrhythmia - - - - -
(*) >mild; (º) HR depending on severity, clinical setting;
95% CI
Slide courtesy of Aleksander Kempny
Transplant Assessments and Transplants for Failing Single Ventricle
Freeman Hospital, Newcastle, UK 2010 - Present
Slide courtesy of Asif Hasan
Survival: Era Effect
Timeline for Fontan Failure
1982 rBTS 1984 lBTS
2011 Transplant assessment
2006 Enalapril
2010 Sildenafil
Tx?Tx? Tx?
Tx?
- Renal failure - Liver impairment - Stroke
Slide courtesy of Darryl Shore
Bethesda Classification
Total Cohort:
•1090 ACHD patients •583 male/ 507 female •Mean age 35.3 +/- 14.9
1130 consecutive heart operations •30 non sternotomy •525 first sternotomy •559 Redo sternotomy
1089 (97.6%) CPB
Royal Brompton Contemporary Cardiac Surgery Study (2000-2014)
Operative data
• 97.3% had a median sternotomy • 53.2% (580) had > 2 haemodynamic lesions • Mean CPB time = 112 (+64.9) mins • Mean Clamp time = 62 (+48.6) mins
Results.
Royal Brompton Contemporary Cardiac Surgery Study
Royal Brompton Contemporary Cardiac Surgery Study
Study population according to type of surgery and surgical approach.
Royal Brompton Contemporary Cardiac Surgery Study
Results.• Early Mortality 1.77%
• Re-operations 79 (7%) – Post-op bleeding 4.5% – Drainage of effusion 1.3% – Pacemaker insertion 0.4% – Wound discharge 0.8% – Repair of peripheral vls 0.2% – Residual lesions 0.5%
Royal Brompton Contemporary Cardiac Surgery Study
Results. Complications:
• Permanent pacing 3.3% • Endocarditis (within 12/12) 0.4% • Pneumonia 2.3% • Prolonged intubation 2.9% • Renal Failure 4.1% • Neurological 1.2% (5 CVA)
Royal Brompton Contemporary Cardiac Surgery Study
Global Operative Mortality: 1.77%
525 383 135 41
Royal Brompton Contemporary Cardiac Surgery Study
Baseline predictors of early mortality (n=20).
Royal Brompton Contemporary Cardiac Surgery Study
Overall Survival
Royal Brompton Contemporary Cardiac Surgery Study
NYHA
0.00 730.50 1461.00 2191.50 2922.0005
101520253035404550556065707580859095
100
Time
Surv
ival
pro
babi
lity
(%)
Number at riskGroup: NYHA 1
431 294 203 156 121 88 62 43 26 15Group: NYHA 2
414 270 214 158 118 88 62 41 24 16Group: NYHA 3-4
119 69 56 45 36 23 15 10 7 3
Preop NYHANYHA 1NYHA 2NYHA 3-4
(Years) 1 2 3 4 5 6 7 8 9 10
p < 0.001
Royal Brompton Contemporary Cardiac Surgery Study
TAPSE
0.00 730.50 1461.00 2191.50 2922.00 3652.5005
101520253035404550556065707580859095
100
Time
Surv
ival
pro
babi
lity
(%)
Number at riskGroup: <15mm
151 102 77 66 48 39 20 11 7 2 0Group: 15 to 25mm
420 269 205 143 99 61 45 26 14 6 0Group: >25mm
178 115 76 54 41 27 16 12 5 3 0
TAPSE<15mm15 to 25mm>25mm
(Years) 1 2 3 4 5 6 7 8 9 10
p < 0.001
Royal Brompton Contemporary Cardiac Surgery Study
Operative Priority
Royal Brompton Contemporary Cardiac Surgery Study
Kaplan-Meyer survival curves to number of risk factors.
> 2
Relative survival curves after ACHD cardiac surgery according to number of risk factors (Non-elective surgery, NYHA >3 and TAPSE < 15mm).
> 2 risk factors (HR 16.1)
Royal Brompton Contemporary Cardiac Surgery Study
NYHA Functionnal class ImprovesNYHA class baseline and at the latest follow up among 1044 survivors of ACHD surgery
Latest FUBaseline
NYHA class baseline in non survivors (n=46/1090)
Royal Brompton Contemporary Cardiac Surgery Study
Kaplan–Meier curves depicting freedom from reoperation, other intervention and/or new onset arrhythmia (accumulatively).
10yrs Re-operation = 73% Other intervention = 80% New onset arrhythmia = 82%
5 years = 78% 10 years = 58%
Follow-up (years)
Slide courtesy of Darryl Shore
1. Choice of Procedure Must be thoughtful and informed
2. Complete surgical repair Residual defects are poorly tolerated TOE and expert intervention is mandatory
3. Haemostasis is key Post op stability Diagnosis of low post op C.O Morbidity associated with re-exploration 4. Re-sternotomy Planning and contingency planning
5. Communication with intensivists Discussion about post-op management (e.g. RV dysfunction)
Tips
Royal Brompton Contemporary Cardiac Surgery Study
Royal Brompton Contemporary Cardiac Surgery Study
Post op blood loss = 330ml
!/?
A MDT approach is paramount
Adult Congenital Cardiac Surgery in 2016: Conclusions
➢ Despite high complexity, cardiac surgery for ACHD performed in a single tertiary reference centre with a multidisciplinary approach is associated with low early and late mortality and improved functional class.
➢ The predictors of outcome of NYHA class >III right, ventricular dysfunction and non-elective surgery support a proactive approach and earlier intervention.
➢ More work is required to prevent, delay and or treat end-stage disease. ➢ A multidisciplinary and tertiary enviroment with a proactive approach
and early patient engagement are essential for securing best late outcomes.
Joseph Perloff, UCLA
Alexander Nadas, Boston
Jane Somerville, London