The Double Switch Using Bidirectional Glenn and Hemi-Mustard. Frank Hanley

Transcription

1 The Double Switch Using Bidirectional Glenn and Hemi-Mustard Frank Hanley

2 No relationships to disclose

3 CCTGA

4 Interesting Points for Discussion What to do when. associated defects must be addressed surgically: anatomic (DS) vs physiologic repair RV and TV begin to fail: medical management and Tx vs TVR vs anatomic repair Anatomic repair is under consideration but LV must be trained A neonate presents with no associated defects and has good RV function

5 The Big 4 Ventricular septal defect Pulmonary stenosis Systemic tricuspid valve abnormality and regurgitation Complete heart block

6 Other Anatomic Details of importance Positional anomalies in 45% (26/58) [S,L,L] 50 Dextrocardia: 13 Mesocardia: 5 [I,D,D]

7 Surgical Management of CCTGA using anatomic repair ( double switch ) Objective of Surgery -- Place the morphological left ventricle (once adequately trained) in the systemic circulation -- Address coexisting lesions (VSDs, PS/PA, TV, CHB)

8 Surgical Management of CCTGA The Surgical Objective is accomplished by: -- switching the atrial outflow and switching the ventricular outflow to create AV and VA concordance -- Addressing coexisting lesions (VSDs, PS/PA, TV, CHB)

9 Surgical Management of CCTGA Options for switching ventricular outflow: -- arterial switch -- Rastelli LV-AO baffle + RV-PA conduit -- aortic translocation + RV-PA conduit

10 Surgical Management of CCTGA Options for switching atrial outflow: -- Senning Baffle -- Mustard Baffle -- Bidirectional Glenn + hemi-mustard

11 Evolution of use of hemi-mustard and BDG Initially used in pts with positional abnormalities : dextrocardia / apicocaval juxtaposition, and IDD Then extended to all pts with CCTGA/PS who required a Rastelli and RV-PA conduit Finally extended to pts with arterial switch and no positional anomalies

12 Anatomic correction of CCTGA is facilitated by use of the Bidirectional Glenn (BDG) is several ways Unloads RV Prolong life of RV-PA conduit Decrease tricuspid regurgitation Lessen impact of RV dysfunction Simplifies atrial baffle Shortens cross clamp time Reduce systemic and pulmonary venous pathway obstruction Reduce sinus node and atrial dysrhythmias Particularly useful when cardiac position anomalies are present, such as situs solitus w dextrocardia, and situs inversus

13 RVOT revision hemi-mustard and BDG vs conventional atrial switch Hemi-Mustard group: 100% 10-yr freedom from RVOT intervention Conventional atrial switch: 75% 5-yr and 50% 10-yr Hemi-Mustard lower risk of RVOT reintervention (p=0.019)

14 TR grade Tricuspid regurgitation following double switch p= Pre-DS Post-DS

15 Contraindications to using BDG Neonate or very young infant Any patient with elevated PVR

16 Hemi-Mustard results No baffle obstruction No sinus node dysfunction or sinus arrhythmias 1 BDG complication due to circular shunt 1 neurologic complication possibly related to BDG

17 Surgical Management of CCTGA Indications for intervention Decreased morphologic right ventricular function Progressive tricuspid regurgitation -- Correct associated structural defects (exception: IVS, severe PS) ? Management of neonates/infants with no structural defect and no physiologic abnormalities (TR)

18 Patient Profile 2.5 yo 10 kg male presented with SLL levocardia CCTGA, IVS, no PS, and severe TR PA band x 2 over the course of 1 year LV met criteria for placement into systemic circulation (5 point scoring system) Underwent takedown of PA band, arterial switch, hemi-mustard, and BDG

19 CCTGA,IVS

20 CCTGA,IVS s/p PA Band

21 Removal of distally placed PA Band

22 CPB initiated with bicaval canulae

23 Cardioplegia

24 CCTGA arterial switch, hemi-mustard incisions

25 CCTGA arterial switch

26 CCTGA arterial switch completed

27 Arterial switch aortic transection

28 Arterial switch main PA transection at PA band site

29 LeCompte Maneuver

30 Anterior Coronary Dissection

31 Trimming of PA band site scar on proximal neo-aortic root. Note distal position of band site

32 Trimming of PA band site scar on proximal neo-aortic root

33 Coronary implantation

34 Aortic Anastomosis

35 Patching of coronary explantation site on neo-pulmonic root

36 Completed PA reconstruction

37 CCTGA arterial switch completed right atriotomy for Hemi-Mustard, with intact atrial septum

38 Right atrial free wall incision stops at least 1cm above IVC

39 CCTGA arterial switch completed atrial septectomy

40 Atrial Septectomy

41 Atrial Septectomy Near CS

42 CCTGA arterial switch completed atrial septectomy completed

43 Hemi-Mustard pathway as an hour glass TV Atrial septal plane IVC

44 Waist of hour glass atrial septal plane PTFE patch RL pul vein Coronary sinus Atrial septal ridge

45 Re-endothelialization of IVC rim of atrial septectomy

46 coronary sinus unroofing

47 Superior enlargement of atrial septectomy

48 Re-endothelialization of superior enlargement of atrial septectomy

49 Re-endothelialization of superior enlargement of atrial septectomy, 2

50 Size of atrial septostomy after superior enlargement

51 CCTGA arterial switch completed measuring hemi-mustard PTFE patch

52 Measure TV anulus - IVC length, to determine Hemi-Mustard patch diameter

53 Measure TV anulus - IVC length, to determine Hemi-Mustard patch diameter,2

54 Creating Hemi-Mustard PTFE patch

55 Measure Hemi-Mustard patch diameter

56 Marking Hemi-Mustard patch

57 CCTGA arterial switch completed hemi-mustard PTFE patch partially sewn in

58 Initiation of Hemi-Mustard patch suture line at most superior aspect of TV anulus

59 Identify left pulmonary veins in relation to patch

60 Identify right lower pulmonary vein

61 Hemi-Mustard patch posterior suture line near right lower pulmonary vein

62 Hemi-Mustard patch posterior suture line completion

63 Anterior Hemi-Mustard patch suture line near MV and conduction

64 Hemi-Mustard patch suture line along lip of unroofed coronary sinus

65 Completion of Hemi-Mustard patch suture line near right atrial incision and away from IVC

66 CCTGA arterial switch completed hemi-mustard PTFE patch completed

67 Completed Hemi-Mustard PTFE patch

68 Right atrial closure near Hemi-Mustard patch suture line

69 Arterial Switch Hemi-Mustard with BDG

70 Right PA clamped and opened in preparation for BDG

71 BDG back suture line

72 Completed operation, off pump

73 CCTGA, VSD,PS s/p central shunt

74 Rastelli Double Switch with Hemi-Mustard and BDG

75 END

76 Anatomic repair 58 patients between Jan 1993 to Sept 2011 Rastelli-atrial switch (RAS): 30 Pulmonary atresia: 23 (5 with PA/MAPCAs) Severe subpulmonary stenosis: 7 Arterial-atrial switch (AAS): 28 PAB required: 19 PAB tightened: 8

77 58 cc-tga 19 PAB 9 AAS 30 RAS 19 AAS

78 Morphology n=58 PS: 30 VSD: 48 CHB: 4 TV: 25 Anatomic Details Moderate or greater TR: 20 Ebsteinoid valve: 10

79 Age distribution < 1 yr 1-5 yr 5-10 yr yr > 15 yr Median age 3.0 years Range 3.9 months to 24.0 years

80 Pre-DSO Procedures Arterial-atrial group 28 Rastelli-atrial group 30 Pulmonary artery band 19 Modified Blalock-Taussig Shunt 20 PA Band tightening 8 Bidirectional Glenn 2 VSD repair 1 Second Modified Blalock-Taussig Shunt Aortopulmonary window prior to unifocalization Unifocalization of MAPCAs to central shunt Pacemaker implantation 3 Bidirectional Glenn 7 Pacemaker implantation 1

81 Glenn/Hemi-Mustard Atrial Switch Performed in 40/58 (69%) patients Conventional atrial baffle performed in 18 pts BDG avoided due to elevated PVR 6 Anatomic considerations 4 -- Evolution of management 8

82 Outcomes Hospital mortality: 1.7% (1/58) One late death One late transplant Postoperative ECMO support required in 2 patients Postoperative heart block: 21% (12/58)

83 Midterm results Median follow-up 6 y, range 7 m - 16 y Biventricular function preserved in 87% of survivors LV dysfunction: moderate 4 severe 2 NYHA functional class I in 43/47 All acyanotic One cardiac transplantation

84 Outcomes: Reoperation AAS-higher earlier need for reoperation RAS-late reoperation due to RV to PA conduit replacement

85 AAS Outcomes Coronary complications: 2/23 Reop LeCompte/LCA release performed Severe proximal RCA stenosis Neo-Aortic insufficiency: 2/23 AVR required in one patient 30 At higher risk for decreased NYHA functional status compared to Rastelli group (p=0.013) RAS AAS IV III II I

86 Concerns BDG In cases of elevated PVR runs risks of impaired cerebral venous drainage, impacting cerebral perfusion pressure (CPP=MAP-CVP) Interventional access for electrophysiologic procedures limited

87 Concerns LV training had occurred in all pts with: -- late reduced functional status -- late decreased LV function -- late death -- transplant

88 LV mass (g/m2) LV Training PAB 17, Tightened 8 Mean pre-op LV mass: 58.0 ± 15.1 g/m 2

89 Conclusions Rastelli pts, in general, do better than arterial switch patients. The only issue is conduit failure, which can be delayed significantly by using the BDG Arterial switch patients are often subjected to the uncertainty of LV training, and suffer complications related to PA banding (neo AI)

90 Conclusions Hemi-Mustard/BDG: Decreases volume across RVOT and prolongs life of RV to PA conduit Unloads the failing right ventricle Reduces volume load on dysplastic tricuspid valve Baffle complications minimized Sinus node dysfunction minimized Simplifies atrial technique, which is particulary important in pts with positional anomalies (SLL dextrocardia, IDD)

91 CCTGA,VSD,PS

92 Arterial Switch Hemi-Mustard with BDG

93 CCTGA, PS Rastelli, hemi-mustard incisions

94 CCTGA,PS Rastelli completed

95 The issues AV and VA discordance In theory the circulation is normal, but this is rarely the case, because of the high incidence of associated structural defects. The Big 4 Even if there are no associated structural defects, the right ventricle and tricuspid valve in the systemic circulation eventually cause problems

96

97 Arterial-Atrial Switch (AAS) Rastelli-Atrial Switch (RAS) The Hemi-Mustard patch is always a simple circle, which bends appropriately around the TV anulus and IVC orifice

98 Modified Double Switch Rastelli procedure Bidirectional Glenn IVC to Tricuspid valve

99 Hemi-Mustard PTFE patch suture line

100 Identify right lower pulmonary vein in relation to patch