Management of complex CHD in adults

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1 Management of complex CHD in adults Victor Tsang Society of Thoracic Surgeons of Thailand 2016

2 The impact of infant cardiac surgery

3 Over 90 % of infants born with CHD will reach adulthood By 2010, adults accounted for 60 % of patients with complex CHD Khairy P et al. J Am Coll Cardiol. Marelli et al. Circulation 2014

4 Pablo Avilà et al. Canadian Journal of Cardiology 2014

5 Complex morphology tetralogy of Fallot, truncus arteriosus, transposition complexes, AVSD and univentricular hearts Complex Substrates Risky patients, late diagnosis, co-morbidities and age related illnesses

6 Risky patients Heart failure Arrhythmias Pulmonary and systemic hypertension Tromboembolic events Endocarditis Pregnancy

7 Some defects may be diagnosed late in adult life ESC guidlines 2010 Brickner et al. NEJM 2000

8 Patient s comorbidities People with CHDs have increased risk for diabetes, chronic renal disease, epilepsy and cardiovasular comorbidities A simple morphological defect becomes complex! Billet et al. Heart 2008;94:

9 Surgical treatment in adulthood Multiple re-interventions Transplantation Even a minor non-cardiac surgery may carry a high risk!

10 NON-SURGICAL NEEDS Education Employment Insurance Sport Scars Etc..

11 ACHD Surgery GOSH/HH Number of Patients 1221 Age at Surgery (y) Median 28 Mean 32 Range Gender Male (%) 53,3 Female (%) 46,6 Post Op Hospital Stay Median 7 Mean 10,1 Range Days Mortality 1,64%

12 Fourth or more operations 7% Operative sequence Third operation 17% First operation 48% Second operation 28% First operation Third operation Second operation Fourth or more operations

13 Survival 100% Mortality per Operative Sequence ACHD % 96% 94% 92% 90% 88% 86% First Oiperation Second Operation Third Operation Fourth or More Operations Months

14 120 Operation Sequence per year GOSH/HH First Operation Second Operation Third Operation Fourth or More Operations

15 Pulmonary procedures Aortic operations ASD closure AVSD Mitral valve operations Other Tricuspid procedures Ross procedure Tetralogy of Fallot repair Pulmonary vein procedures VSD repair Replacement of cardiac conduit Systemic-to-PA shunt / BT shunt Coronary artery procedure Heart transplant ACHD OPERATIONS First Operation Second Operation Third Operation Fourth or More Operations

16 Age > 40 years in ASD repaired patients NYHA 1 NYHA 2 NYHA 3 NYHA 4 Number of cases Length of stay, days (F) Length of stay, days (M) 13,7 8,4 16,7 15,5 Mean 10,5 8,2 15,0 19,3

17 RV PA Valve conduit Longevity Size Position Choice of valve conduit

18 Longevity of 405 Homografts Stark et al. JTCVS 1998 Stark homograft slides

19 Help from interventional cardiologists? Signh et al. Nat Rev Cardiol. 2014

20 Or just storing up the problems? Surgical approach is more challenging after catheter interventions

21 Patient s Background 17 years old male, 60 kg VA discordance Pulmonary atresia VSD Right sided arch Right PAPVC

22 Previous history Modified BT shunt 1997 Rastelli repair, RV to PA conduit 17 mm aortic homograft 1999 Melody valve (18 mm) implantation 2007 Stent ballooning 2013 Melody valve endocarditis 2014

23

24 Pulmonary Valve Reoperations post stenting Pulmonary homograft Gore-Tex graft

25

26 Life time management of pulmonary homograft starts again

27 Ebstein`s anomaly RA RA`

28 Ebstein`s anomaly Laminated and dysplastic Septal leaflet Redundant Antero-superior leaflet Deficient Inferior leaflet

29 Grading of Ebstein s severity: Carpentier Type A: adherence of septal and posterior leaflets but without restrictive volume of functional RV Type B: RV atrialised with normal anterior leaflet Type C: Severe restriction of anterior leaflet (which may cause RVOTO) Type D: Almost complete atrialisation of RV with just small residual infundibular component Carpentier A et al. J TCS 1988

30 Ebstein`s anomaly Displacement vs Rotation Rotational displacement around inner curve...rather than strict downward displacement Courtesy A Cook & RH Anderson

31 Ebstein`s anomaly : Cone Repair Antero-Superior and Inferior leaflets mobilised and detached from their position in RV and rotated clockwise and sutured to the septal margin of detached leaflet (=cone) Septal leaflet (if present) delaminated and incorporated in Cone Annuloplasty Right atrium plicated and ASD closed (if present) Da Silva JP, Arq Brasil Cardiol 2004, JTCVS 2007

32 Ebstein`s anomaly: Cone operation N=100, hospital mortality 3.0% No tricuspid valve replacement Da Silva et al, Semin Thorac Cardiovasc Surg Pediatr Card Surg 2012

33 Surgical indications We operate on patients who are symptomatic or have few symptoms when tricuspid valve regurgitation is severe and exercise tolerance is measurably diminished

34

35

36 Ebstein`s anomaly: Cone operation GOSH experience: N=27 Age Weight 22 days to 56 y (mean age 15 yr) 5 to 75 Kg CPB time 156 ± 46 CX Time 92 ± 27 Ablations 3 Deaths 0 Failure of Repair 1 Reoperation 1 Length of Follow-up 2.3 ± 0.9 years Number of patients Age at Operation

37 Dehiscence of the Inferior annuloplasty

38 Number of patients Cone operation reduces TR TR Pre-Op Post-Op 0 Trivial Mild Moderate Severe Effect of cone operation on functional status Number of patients NYHA Pre-Op Post-Op 0 NYHA 1 NYHA 2 NYHA 3 NYHA 4

39 .but, despite clinical improvement, markedly reduced RV function!

40 .but, despite clinical improvement, markedly reduced RV function! ECHO: eyeballing Pre-OP Post-OP RV Function (TAPSE) 50 P < *** ECHO: TAPSE 5 0 Good Mild Moderate Severe 0 Pre-Op Post-Op

41 Why does RVEF decrease after surgery? Pre-operative Diastole Systole Post-operative Stroke Volume Competent valve -Decreased stroke volume & ejection fraction -Increased afterload Dyskinetic re-ventricularised myocardial wall Readaptation or Intrinsic Cardiomyopathy?

42 Reduced EF but Increased Forward Flow Indexed Forward Flow in MPA Indexed Forward Flow in MPA 60 p < 0.01 Pre-Op Post-Op

43 400 p = p = RVESI (ml/m 2 ) RVEDI (ml/m 2 ) Pre-Op Post-Op 0 Pre-Op Post-Op p = p < LVESI (ml/m 2 ) LVEDI (ml/m 2 ) Pre-Op Post-Op 0 Pre-Op Post-Op Better LV filling

44 Improving VO2 max

45 . any evidence of improvement 2 weeks post 2 years post

46 Cone repair for Ebstein s Encouraging surgical experience with Cone repair Not an easy operation Late RV geometry, function and arrhythmia is unknown long term results awaited

47 ACHD:Transition of Care yrs 18 yrs Paediatric Cardiology Transition Clinic ACHD ACHD specialist and paediatric cardiologist Multidisciplinary team and nurse specialists Management plans established and discussed with patients

48 How to train ACHD surgeons? Increasing ACHD population and complexity bring new challenges Establishment of specialist centres responsible for training

49 Thank you

50 EBSTEIN ANOMALY Luu et al. Heart, Lung and Circulation 2015

51 Fontan circulation Fontan et al. Circulation 1990

52 Fontan circulation The procedure will never be curative in nature Currently, improved surgical techniques are likely to lead improved late outcomes

53 Fontan circulation after decades Ventricular failure Atrial arrhrythmia Thromboembolic issues This unusual cardiac physiology is confronting adult surgeons and cardiologists!

54 EBSTEIN ANOMALY IN ADULTS Re-do tricuspid valve surgery Arrhythmias

55 Tetralogy of Fallot Risk for late PVR optimal timing? Heart failure Hickey et al. Eur J Cardiothorac Surg. 2009

56 Tetralogy of Fallot Hickey et al. Eur J Cardiothorac Surg. 2009

57 Most challenging patients! High proportion of re-operations ICU/anaesthetic issues Careful preoperative planning required How to train ACHD surgeons?

58 ESC guidelines* for organisation of ACHD care Level I Exclusive care in ACHD unit Level II Shared care with adult cardiac services Level III Care in the non-specialist units (with access to specialist care if required)

59 ESC guidelines 2010 Level of care: No spesific categorisation by diagnosis Well-working network is highly important

60 congenital heart disease > 60 yr age: a new challenge in the care of adults with congenital heart disease? Baumgartner. Eur Heart J 2014

61

62 Special features Higher morbidity More frequent hospital admissions and interventions Acquired cardiac diseases Additional comorbidities (e.g Alzheimer disease) The need for integrated care in adult medicine Turatel et al. Eur Heart J. 2013

63 Number of eldery patients with CHD is increasing There is an urgent need for ACHD surgery training

64 Fifth Operation Outcome Aortic operations (5) Reoperated (2) Systmic to PA- shunt/ BT shunt (2) Died late (2) Double chambered RV repair (1) Pulmonary procedures (4) Replacement of cardiac conduit (2) Removal of aortic pressure line (1) Mediastinal procedure (1) Alive Alive Died late (1) Died in hospital (previous TCPC and RPA repair) Alive (previous TOF repair)

65 Sixth Operation RV-PA conduit construction Outcome Died late Tricuspid annuloplasty Died in hospital Aortic valvar replacement (mechanical) Alive Pacemaker procedure Reoperated (seventh operation)

66 120 Outcome per year GOSH/HH Alive Died in Hospital Died late Reoperated

67 Hospital stay of ASD repaired patients NYHA 1 NYHA 2 NYHA 3 NYHA 4 Average Number of cases Length of stay, days (F) 7,9 7,5 10,8 23,0 8,4 Length of stay, days (M) 9,1 8,0 13,5 15,5 9,4 All in total 8,3 7,7 11,6 19,3 8,8

68 Thank You

69 ACHD at Heart Hospital 1020 operations cases of PVR (25%) Mortality 1.7%

70 Melody Valve Implantation

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