surgical approach of cyanotic congenital heart disease

presented in LPS institute of cardiology on 15.2.17 by Dr Dibbendhu Khanra 2nd year DM resident

1. Surgical Approach of Cyanotic CHD Dr. Dibbendhu Khanra

2. Disclaimer No cong Acyanotic Heart diseases No Eisenmenger’s No clinical or Echo diagnosis No medical management No surgical details 2

3. Parts of Discussion Introduction History Fetal and Adult circulation Pulmonary artery and PBF Shunt Fontan & complications PAB and BAS ICR & ASO Surgeon’s perspective Individual defect and m/n 3

4. Cyanotic CHD PULMONARY STENOSIS Pulm ESM NO PULMONARY STENOSIS NO VSD VSD PULMONARY HYPERTENSION NO PULMONARY HYPERTENSION INCREASED PBF DECREASED PBF PULMONARY VENOUS HYPERTENSION ASD+PS ( Triology ) 1 Fallot’s Physiology 2 Transposition physiology 3 Eisenmenger’s physiology 4 Obstructive TAPVC 5 PAVF SV to LA 6 4

5. So many surgeries! ICR/ ASO Blalock- taussig Glenn/ Fontan Banding/ TCV repair Mustard/ senning Norwood- sano 5

6. Rome was not built in a day 6

7. 1945: BT shunt 7

8. 1958 Glenn shunt 1971 Fontan surgery 1973 Kreutzer 1983 Kawashima 8

9. 1954 Lillehei : TOF 1957 Kirkin : DORV 1959 Senning : TGA 1959 Mustard: TGA 1966 Rashkind : TGA 1975 Jatene : ASO 1958 Carpentier : TC repair 1983 Norwood HLH 2003 Sano HLH 9

10. 10

11. What we already know Disease Types Surgery Timing TGA NO VSD Rashkind / BAS If switch delayed Artreial switch 3-4 wk TGA VSD LV inadequate Atrial switch 3-6 m LV adequate Arterial switch 3 m TOF Uncontrolled spells BT shunt <3 m Stable Total repair 1-2 yrs TOF PA severe cyanosis BT shunt <3 m Post-shunt Total repair RV – PA conduit 3-4 yrs TAPVC Obstructive Total repair Urgently Non obstructive Elective repair 1-2 yr 11

12. What we already know (cont.) Disease Types Surgery Timing PTA CHF Total Repair If delayed Urgently PA banding NO CHF Total Repair 6-12 wks Ebstein Deep cyanosis RV inadequate Fontan pathway ASD enlargement Good RV TCV repair> replacement HLH Norwood Fontan pathway 3m 1-2 yr TOF like conditions Two ventr repair not possible Mild cyanosis Direct fontan Glenn 3-4 yrs 3-4 yrs TA, SV TGA OR DORV With non-routable VSD Significant cyanosis Glenn Fontan < 6m > 6m 12

13. A gap in understanding Philosophy behind the surgeries 13 Surgeon’s perspective

14. Necessity Innovation 14

15. The normal structure Two filling chambers Two pumping chambers Two septum Two great vessels Two coronary arteries 15

16. The fetal circulation % Cardiac output % saturation Pressure 16 RV is the main pump in Fetal life

17. Fetal vs adult heart Points Fetal heart Neonatal heart Implications Lungs Immatured Matured PBF not mandatory in fetus MPA Small Large PBF less in fetus PVR Very high less PVR falls with first cry RV Main pump Smaller RV large and thick in fetus PDA R-L L-R PDA closes by 2 wks FO R-L L-R PFO closes by birth Circulation parallel series Better O2 pickup & delivery 17 RV is well trained in Fallot

18. Normal relation 18 SVC/IVC – PA, PV – AO (CPB) PA – both Lung (collaterals, shunts ) LV-AO, RV-PA ( VSD routing/ switch) PA anterior and to the left of aorta (Le Compte ) Coronaries from Aorta (TGA, TOF)

19. Target for surgery Priority wise Systemic blood flow (Norwood, VSD routing) PA maturation/confluence (AP shunt, RV-PA conduit, PDA stent) Pulmonary blood flow (BDG/ Fontan ) (PA banding) Managing collaterals ( embolization / unifocalization ) VA switch ( atrial / ventricular/ artreial ) Aorta/ PA relation (Le Compte ) Shunt repair/ closure (ASD/ VSD/ PDA/ AP shunt/ conduit) Take care of coronaries 19

20. The right heart SVC – RA (passive) IVC – RA (passive) RA – RV (RA = flowing reservoir) RV – RVOT (active pump) RVOT – MPA MPA – LPA – LT LUNG MPA – RPA –RT LUNG L/O ENERGY 20 Classic Fontan Bypasses RV With Intact RA

21. PBF PA growth PA in-confluent In Pulm atresia / absent PA P annular hypoplashia Collaterals Aorto -pulmonary shunt (few wks) PDA stenting RV – PA conduit Active flow Lung maturation Makes PA adequate Complete venous drainage RV not functional TA SV PA IVS small RV Ebstein with small RV Cavo -pulmonary shunt SVC – PA = Glenn (3-6m) IVC – PA = Fontan (1-2yr) Passive flow/ PVR low Only when PA adequate 21

22. Aortopulmonary shunt Central shunt: - CHF - PAH - Distorted PA - Difficult to close Classical BT Modified BT Connection End to side Side to side Material Rt SA Gore tex (Lt SA) Upper limb Less Growth Normal growth PA Rt PA (I/L) Lt PA (I/L) Arch Opposite side Same side Age >3m <3m Thrombosis High in <3m Common Size mismatch - + 22 Surgeon’s choice: Mod BT shunt Side which PA is smaller Aspirin for 3-6m Size mismatch Thrombosis/ obstruction If IL Subclavian if <2.5mm Common carotid can be used

23. Cavopulmonary shunt (SVC) Classic Glenn Modified Glenn (BDG) Hemi Fontan Classic Glenn BDG /BDCPA Connection End to end End to side Flow unidirectional Bidirectional Left lung Deprived Normal growth Cavopulmonary shunt IVC blood bypasses lung No Hepatic vasoconstrictor PG PAVF remain cyanotic Passive (low PVR) 23 Surgeon’s choice: BDG If VSD not repairable

24. Cavopulmonary shunt (IVC) BDG To Fontan HemiFontan to Fontan Passive (low PVR) Fontan patient: Swollen face Pulsations in head / neck veins PAVF IJV approach not possible 24 Surgeon’s choice: BDG to Fontan Fenestration relieves RA pressure At the cost of cyanosis

25. Fontan (TCPC) Total cavo -pulmonary connection Physiologically flawed Cyanosis RA overloaded Chronic low CO Syst ven congestion Exercise intolerance Arrythmia Thromboembolism Pulm vein compression PLE CLD No Heart transplant Obstructed FONTAN 25

26. Complications Prevalence Timing Reasons Prevention Thrombo embolism ( rarely PVOD) 20% 1 st yr After 10 yrs Dilated RA Stasis in RA Low CO Arryhtmia Aspirin preferred + Warfarin (INR >2) (high risk cases) Arrythmia SVT 20-35% MC A flutter As long as 20 yrs surgical scar High RA pressure RA distension sinus node injury Acute DC shock Chronic Amiodarone Chronic Fatigue Exercise Intolerance Low CO Arrythmia / CMP Syst congestion Myo remodelling PLE ACEI Digoxin Avoid – ve ionotrops LVF Pulm vein compression by dilated RA More in classic Fontan Fontan conversion TCPC Fontan complications 26

27. Fontan complications Complications Prevalence Timing Reasons Prevention Prolonged pl eff PLE/ ascitis Neutr deficinecy Immuodeficiency Thrombogenecity 3% Bronchitis 1% 3 yrs High SVC pressure Lymphatic drainage impaired Interstitial Leakage L/o α 1AT in stool Loss of ATIII High protein diet AB/ vaccine MLCFA Somatostatin Octeotride Heparin Hepatopathy Ascitis ALI CLD Diuretics Spiranolactone NO heart transplantation Cyanosis Fenestration leak Microemboli PVOD PAVF Pulm dis Abnormal SVC 27

28. Age above 4 years Adequate size of right atrium Normal systemic venous return mean pulmonary artery pressure (below 15 mmHg) Low PVR No atrio -ventricular valve regurgitation Normal ventricular function No distortion of pulm art from prior shunt/ band Normal sinus rhythm Adequate pulmonary artery size Ten commandments (Fontan and Baudet ) 28

29. Fontan Evolution Classic Fontan SVC – RPA (end to end) RAA – RPA (outlet Valve) IVC-RA (inlet valve) ASD closure MPA ligated Kreutzer modification RAA – MPA ASD closure Bjork modifications RAA – RVOT ASD closure RAA – RPA ASD closure No valve inlet/ outlet valve RA RV RA 29 No RV

30. Fontan Evolution Kiwoshima modifications IVC cont of hemiazygous vein Total venous return into RPA NO RA Classic Glenn BD Glenn Modified Fontan 30

31. Fontan Evolution Intracardiac tunnel Extracardiac conduit Fenestration 31

32. Fontan Classic Fontan RAA - RPA Lateral Tunnel Intra- atrial Baffle PTFE Extra cardiac conduit Intracardiac baffle Extracardiac conduit Pleural effusion ++ +++ Thromboembolism ++ + SVT +++ + Age I year > 3yr Exercise intolerance ++ +++ 32 Surgeon’s choice: 1-3 yr: intracardiac >3 yr: extracardiac

33. Fenestration right-to-left shunt pop-off valve prevent rapid volume overload to the lungs Limit caval pressure Increase preload to the systemic ventricle Increase cardiac output Cyanosis decrease pleural effusions Less hospital stay Can be closed (if required) 33 Surgeon’s choice: Fenestrated Fontan

34. The left heart PV – LA (abnormality=TAPVC) LA – LV LV – LVOT LVOT – AO (active pump: high pressure) AO – BRAIN/ ARMS/ LEGS Late presenting TGA LV is not trained 34 BT shunt Upper limb is deprived Surgeon’s choice PAB

35. Cyan CHD with increased PBF Anatomical repair overcomes RV failure Qp:Qs = 1:1 35

36. PA banding PBF/ CHF PAH/ PVOD IPPR/ NO CPB Pulm Dysfunction cyanosis anatomic distortion Asym LVH 36

37. PA banding How tight? Diamater 50% reduction - TRUSLAR FORMULA NRGA : 20mm+1mm/ KgBW TGA: 24mm+1mm/ KgBW mPAP 50% reduction Maintaining SPO2 to 93% Where to band? MPA (not annulus) If too high - branch PA stenosed If too low - coronary reimplntation difficult Not reliable in TGA Needs multiple banding 37 Surgeon’s choice Proper size hegar should pass Often PBF reduces At the cost of Asymmetric LVH Subaortic AS

38. PA banding: Indicatons Very sick neonate on IPPR can not tolerate CPB chance of early PVOD (TGA, ECD) Complex congenital CHD e.g. criss cross heart, swiss cheese VSD small fetal heart Biventricular repair not possible Preparation for Glenn/ Fontan PVR needs to be low for passive forward flow Preparation for ASO Late presenting TGA with CHF HLHS: stage I Hybdrid procedure Bilateral PA banding 38 Surgeon’s choice High risk of PVOD And not in a state of repair

39. VA relation establishment: switch Atrial level Ventricular level Great arterial level Le Compte (PA anterior to Ao ) Coronary artery manipulation RV systemic ventricle LV systemic ventricle Physiological repair Anatomical repair 39

40. Atrial switch Mustard Intracardiac Baffle Senning Pericardial patch SVC/IVC - LA – LV – PA PV – RA – RV - AO 40

41. Atrial switch Arrythmia 50% Baffle leak 20% RV dysfunction / TR 10 % SVC obstruction 5% Pulm Venous occlusion 3% Dense adhesion: transition to ASO difficult 41

42. Switch at ventricular level VSD closure LV – AO tunnel RV – PA conduit Le Compte (PA brought anterior to Ao ) No Coronary reimplantation VSD routing SBF PBF 42 Surgeon’s choice VSD PS (non TOF) TGA/DORV Not correcting the abnormal great artrey relation

43. RV-PA conduit Rastelli VSD routing Long tunnel Subaortic AS Aneurysm Operative mortality 30% 20 year survival 50% VSD closure 43 Extracardiac conduit Not suitable for neonate Occlusion high

44. REV ( Réparation à l'Etage Ventriculaire ) ) VSD routing RV-PA conduit Operative mortality 20% Incision above coronaries LeCompte VSD closure 44 Short VSD-AO tunnel Intacardiac conduit Surgeon’s choice For VSD PS REV

45. Nikaidoh VSD routing RV-PA conduit Operative mortality 10% Incision below coronaries LeCompte VSD closure 45 Not suitable for anomalous coronaries Limited Experience

46. Arterial switch operation (ASO) LeCompte Coronary reimplantation LV function Must be normal Difficult Post atrial baffle Dense adhesion LV dysfunction: PA Band – ASO not enough for TGA PS ( fallot ) TGA AS (PAB) Coronary anomalies Complications Supravalvular PS (12%) Neoaortic regurgitation Coronary artery obstruction 46 Surgeon’s choice ASO for TGA Surgeon’s choice for TGA+VSD+PS ASO +REV

47. Coronary anomalies in TGA 47

48. Damus Kaye Stensel No Coronary reimplantation Subaortic stenosis Often after PAB AP shunt MPA – Asc aorta 48 Surgeon’s choice TGA VSD PS subaortic AS Abnormal coronaries DKS+RV-PA = YASUI procedure

49. CCTGA Atrial switch Arterial switch Double switch 49

50. HLH HLH 50 Norwood AP shunt MPA – Asc aorta Sano RC-PA conduit

51. Raskind : Balloon atrial septostomy 51

52. The right ventricle PA without VSD Normal RV Inflow Trabecule Infandibulum (outflow) O T I I I I O O T Tripartite RV (Z score >-2.5) Inflow Trabecule Infandibulum (outflow) Bipartite RV (Z score -2.5 to -5) Inflow Infandibulum (outflow) Monopartite RV (Z score <-5) Inflow Biventricular repair Univentricular repair 52

53. Tricuspid annular Z score Z score = observed value – expected value/ SD RV size and function: CMRI 53 Z score <-2.5 Small RV size RV-coronary communications RV dependent circulation

54. High RV pressure PA without VSD - RV myocardial fibrosis, ischaemia or infarction - RV decompressed through RV – coronary connections - If prox coronary art absent – RV dependent coronaries ( Hhb ) - However, presence of TR or VSD or RV-PA conduit decompresses RV pressure - RV decompression leads to coronary steal 54

55. Coronary abnormalities 55

56. So, What to do? 56

57. Cardiopulmonary Bypass (CPB) PUMP Cross- clapms Cardioplegia Hypothermia Ischaemia ECMO for neonates 57

58. Surgical approach Total repair Definite / desired Anatomical repair CPB required VSD repair RVOTO relief ASO/ DKS Collateral closure unifocalization Palliation Total repair not possible Anatomical reasons CPB not tolerable AP shunt/ RV PA conduit Glenn/ Fontan PAB BAS ASO/ DKS 58

59. TOF Palliative AP shunt RVOT stenting MAPCA embolzation Definitive ICR VSD closure RVOTO relief TAP for hypoplastic annulus Intact PV/ FU for PR/RV dysfunction Confluence of PA Unifocalization Avoid injury to coronaries Any other defect - repair Lowest morbidity 3-12 months of age 59

60. Cath study before ICR Pulmonary artery assessments (CT, MRI) Mascular VSD (Echo) Abnormal coronaries Collaterals and embolisation Previous shunt patency 60 Surgeon’s choice: To see Collaterals Coronaries Shunts

61. Surgeon’s view 61

62. Pulmonary infandibulum assessment RA incision routinely VSD repair with Dacron patch A Hegar dilator (as per Z table) pass through TCV If passes freely thru RVOTO, no resection needed If does not passes, resection of RVOT done Sewed back with Dacron or PTFE patch Patch is always kept subannular to avoid PV injury 62 Surgeon’s choice: transRA+transpulm approach Hegar passage Subannular patch

63. Pulmonary annulus assessment MC GOON RATIO Diameter RPA+LPA/DA N = 2-2.5 <1.5 : BT shunt >1.8: Fontan <1.5 : TAP NAKATA INDEX (mm2/m2) Area RPA+LPA/BSA N = 330 +/- 30 <200 : BT shunt >250: Fontan <200 : TAP 63 Z score<-3: TAP Z score Surgeon’s choice: Z score <-3 Transannular patch

64. Pulmonary valve assessment In subannular patch Pulm valve not injured In transannular patch Pulm valve Is injured Mild to moderate PR develops But RV is trained so no RV dysfunction FU for more than severe PR or RV dysfunction PVR(bovine jugular, monocusp , porcine valve) PVR must be done in absent or dysplastic PV 64 Surgeon’s choice: Mild to mod PR is normal PVR only if PV dysplastic or absent

65. Pulmonary artery assessment 3-6m 1-3yr MPA/ LPA/RPA MPA/ LPA/RPA Not Discernable RV – PA conduit RV – PA conduit Collateral arteries anastomosis Collateral arteries anastomosis 65 Uni focalization

66. Pulmonary artery confluence TAP MPA stenosis LPA/ RPA stenosis near branch RV-PA conduit MPA atresia Distal branch PS 66 BT shunt in sick babies Absent PA unifocalize the collaterals

67. Embolization of collaterals TOF Pulm atresia – more than 3yrs Routine CAG for collaterals Embolize if >2.5mm pre-operatively More chance of bleeding Pulmonary edema Intraoperative embolization also done 67

68. Embolization vs unifocalization Embolization Only the large collaterals Unifocalization In nonconfluent / absent PA 68 Surgeon’s choice: Cath backup: Preoperaitve embolization No cath backup: Intraoperative embilization Surgeon’s choice: Unifocalization Multiple sitting

69. Coronary anomalies in TOF 69

70. Coronary anomaly assessment Long conus artery crossing RVOT RVOT resection is risky in infandibular stenosis Try RVOT stenting by total atrial approach RV to PA conduit Sometimes BT shunt is the only palliation 70 Surgeon’s choice: RV PA conduit

71. BTT shunts Only to buy time for ICR Wt <2 kg or very sick newborn MPA atresia (RV –PA conduit) Hypoplastic Pulm Annulus ( Transannular patch) Unfavourable Coronaries Uncontrollable cyanosis Distal branch PA stenosis Too small for surgery Too sick for CPB AP shunts: pitfalls Cyanosis I/L Radial pulse absent Less growth of upper limb High PBF Chronic LVF PVOD Focal PA stenosis Rib notching 71 Surgeon’s choice: Take down the BT shunt When CPB is established To have blood-free surgical field/ pulm edema

72. Outcome of ICR Long-term Sequale of ICR PR Residual RVOTO Residual VSD/ ASD Arrythmia (QRS>160 ms) TR LV dysfunction PA stenosis RVOT aneurysm Results of severe PR RV dilation RV failure TR Arrythmia Sudden death 72

73. CMRI: one stop shop RV function Coronary artery anomalies Pulmonary artery & branches Collaterals VSD routability Earlier shunts Venous drainages 73 Surgeon’s choice: RV failure

74. Severe PR ECHO MRI Moderate or more PR PLUS:2 or more of RVEDV ≥ 160 ml/m2 (Z-score >5) RVESV ≥ 70 ml/m2 LVEDV ≤ 65 ml/m2 RV EF ≤ 45% RVOT aneurysm PR PHT>100ms Severe PR plus New onset VT Severe exercise intolerance Right heart failure Late repair PVR 74

75. Surgeon’s thoughts Is VSD repairable? How is the RV? Is VSD routable? Are the great arteries normally related? 5. Is there PS? need of patch? 6. How are the pulmonary arteries? ( unifocalization ? MAPCA embolization ) 7. How is the pulmonary valve? Are coronaries crossing over RVOT? Any other repairable defects/ or lesions? Previous shunt or conduit or bands? 75

76. DORV 76

77. Surgeon’s approach for DORV 77

78. TGA Condition Surgery TGA IVS Atrial switch 2WKS Artreial switch 1YR PA banding – switch TGA IVS If LV func poor PA banding - switch Two stage/ high mortality TGA VSD Switch + VSD repair If unfavourable coronary anatomy DKS Instead of ASO TGA+VSD+PS BT shunt initially ASO+Rastelli ASO+REV ASO+Nikaidoh TGA+VSD + subaortic stenosis DKS TGA +VSD Straddled TCV ( RV small ) BT+ASO BDG – Fontan TGA+PVOD No repair Sx not possible early BAS 78

79. CCTGA BT shunt 79 Surgeon’s choice Double switch Surgeon’s choice Senning + REV

80. Single Ventricle VA Concordant VA Discordant (Aorta anterior) Holmes Heart (PS) LV type RV type (DORV) Non Inverted (D- TGA) Inverted (L- TGA) % 15 25 35 5 Aorta Right Left Side/ ant Outlet chamber + + - 80 Surgeon’s choice SV FONTAN

81. TA 81 Surgeon’s choice SV FONTAN

82. PA IVS Dilated RV Small RV Vulvotomy ( Ballon / open) PV atretic BT RV –P A connection Infandibulum atretic Residual RVOTO Vulvotomy ( Ballon / open) PGEI RVOTR ASD closure BT BDG Fontan ASD closure RV coronary connections Left alone TV closure ( starnes Op) 82

83. Ebstein’s Adult - severe progressive cyanosis - RVOTO - NYHA 3-4 poor activity - paradoxical embolus - arrythmia - RV dysfunction Neonate: CHF/ cyanosis Biventricular repair (Knott Craig approach) Single ventricular repair ( strane’s TC closure – Fontan ) 83 Ebstein Danielson Carpentier De silva’s Cone repair Surgeon’s choice Cone repair

84. HLH MBT Sano Connection SCA – IL PA RV - MPA Supply One lung Both lung DBP Lesser Higher Coronary steal + - SBF PBF 84 Surgeon’s choice Sano shunt Within 2 weeks of life High surgical risk

85. HLH Surgeon’s choice Hybrid Process B/L PAB PDA stent (1 st week: NO CPB) Norwood sano Removal of PAB, PDA stents (3-6m: CPB) Fontan 1-2 yr + BDG BAS may be required 85

86. TAPVR LT Innominate LT vertical Supracardiac 50% RA Coronary sinus Intracardiac 20% Infracardiac 20% IVC Esophageal hiatus Mixed 10% ASD PV obstruction Results in PAH End to end Com PV - LA Patch in ASD All PV to LA Unroofing End to end Com PV - LA Ligation Ligation 86

87. Truncus Arteriosus TYPE I VSD repair RV – PA conduit TYPE A2 Dacron patch Anastomosis 87

88. A long presentation.. 88

89. Take home messages AP shunts are only time buying Always Modified BT Repair when repairable Subannular patch. TAP causes PR. Long term RV dysfunction Collaterals – embolize or unifocalize Fontan is only when repair not possible Fontan complicated! PAB/ BAS has fallen out of grace except special indication ASO is the choice for TGA/ REV in PS/ DKS in AS RV plays a big role. CMRI is gold starndard PA IVS: ventriculo -coronary connections Ebstein : Cone Reconstruction CT angio : coronary abnormalities 89

90. Acknowledgement: Dr. Neeraj Prakash Dr. Sandip Chandra Dr. Kaushik Chatterjee 90 Thank you