Cardiac Surgery and Congenital Heart Disease: Where are we...

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

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Year

Surgery + 43%

Intervention +130%

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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

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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

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60

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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