The Double Switch Using Bidirectional Glenn and Hemi-Mustard Frank Hanley
No relationships to disclose
CCTGA
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
The Big 4 Ventricular septal defect Pulmonary stenosis Systemic tricuspid valve abnormality and regurgitation Complete heart block
Other Anatomic Details of importance Positional anomalies in 45% (26/58) [S,L,L] 50 Dextrocardia: 13 Mesocardia: 5 [I,D,D] 8 8 26
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)
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)
Surgical Management of CCTGA Options for switching ventricular outflow: -- arterial switch -- Rastelli LV-AO baffle + RV-PA conduit -- aortic translocation + RV-PA conduit
Surgical Management of CCTGA Options for switching atrial outflow: -- Senning Baffle -- Mustard Baffle -- Bidirectional Glenn + hemi-mustard
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
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
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)
TR grade Tricuspid regurgitation following double switch 4 3 2 1 p=0.006 0 Pre-DS Post-DS
Contraindications to using BDG Neonate or very young infant Any patient with elevated PVR
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
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)
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
CCTGA,IVS
CCTGA,IVS s/p PA Band
Removal of distally placed PA Band
CPB initiated with bicaval canulae
Cardioplegia
CCTGA arterial switch, hemi-mustard incisions
CCTGA arterial switch
CCTGA arterial switch completed
Arterial switch aortic transection
Arterial switch main PA transection at PA band site
LeCompte Maneuver
Anterior Coronary Dissection
Trimming of PA band site scar on proximal neo-aortic root. Note distal position of band site
Trimming of PA band site scar on proximal neo-aortic root
Coronary implantation
Aortic Anastomosis
Patching of coronary explantation site on neo-pulmonic root
Completed PA reconstruction
CCTGA arterial switch completed right atriotomy for Hemi-Mustard, with intact atrial septum
Right atrial free wall incision stops at least 1cm above IVC
CCTGA arterial switch completed atrial septectomy
Atrial Septectomy
Atrial Septectomy Near CS
CCTGA arterial switch completed atrial septectomy completed
Hemi-Mustard pathway as an hour glass TV Atrial septal plane IVC
Waist of hour glass atrial septal plane PTFE patch RL pul vein Coronary sinus Atrial septal ridge
Re-endothelialization of IVC rim of atrial septectomy
coronary sinus unroofing
Superior enlargement of atrial septectomy
Re-endothelialization of superior enlargement of atrial septectomy
Re-endothelialization of superior enlargement of atrial septectomy, 2
Size of atrial septostomy after superior enlargement
CCTGA arterial switch completed measuring hemi-mustard PTFE patch
Measure TV anulus - IVC length, to determine Hemi-Mustard patch diameter
Measure TV anulus - IVC length, to determine Hemi-Mustard patch diameter,2
Creating Hemi-Mustard PTFE patch
Measure Hemi-Mustard patch diameter
Marking Hemi-Mustard patch
CCTGA arterial switch completed hemi-mustard PTFE patch partially sewn in
Initiation of Hemi-Mustard patch suture line at most superior aspect of TV anulus
Identify left pulmonary veins in relation to patch
Identify right lower pulmonary vein
Hemi-Mustard patch posterior suture line near right lower pulmonary vein
Hemi-Mustard patch posterior suture line completion
Anterior Hemi-Mustard patch suture line near MV and conduction
Hemi-Mustard patch suture line along lip of unroofed coronary sinus
Completion of Hemi-Mustard patch suture line near right atrial incision and away from IVC
CCTGA arterial switch completed hemi-mustard PTFE patch completed
Completed Hemi-Mustard PTFE patch
Right atrial closure near Hemi-Mustard patch suture line
Arterial Switch Hemi-Mustard with BDG
Right PA clamped and opened in preparation for BDG
BDG back suture line
Completed operation, off pump
CCTGA, VSD,PS s/p central shunt
Rastelli Double Switch with Hemi-Mustard and BDG
END
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
58 cc-tga 19 PAB 9 AAS 30 RAS 19 AAS
Morphology n=58 PS: 30 VSD: 48 CHB: 4 TV: 25 Anatomic Details Moderate or greater TR: 20 Ebsteinoid valve: 10
Age distribution 20 18 16 14 12 10 8 6 4 2 0 < 1 yr 1-5 yr 5-10 yr 10-15 yr > 15 yr Median age 3.0 years Range 3.9 months to 24.0 years
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 5 3 5 Pacemaker implantation 3 Bidirectional Glenn 7 Pacemaker implantation 1
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
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)
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
Outcomes: Reoperation AAS-higher earlier need for reoperation RAS-late reoperation due to RV to PA conduit replacement
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) 25 20 15 10 5 0 RAS AAS IV III II I
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
Concerns LV training had occurred in all pts with: -- late reduced functional status -- late decreased LV function -- late death -- transplant
LV mass (g/m2) LV Training 90 80 70 60 50 40 30 20 10 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 PAB 17, Tightened 8 Mean pre-op LV mass: 58.0 ± 15.1 g/m 2
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)
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)
CCTGA,VSD,PS
Arterial Switch Hemi-Mustard with BDG
CCTGA, PS Rastelli, hemi-mustard incisions
CCTGA,PS Rastelli completed
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
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
Modified Double Switch Rastelli procedure Bidirectional Glenn IVC to Tricuspid valve
Hemi-Mustard PTFE patch suture line
Identify right lower pulmonary vein in relation to patch