SURGERY FOR TGA AND LVOTO - shaconferences · DYNAMIC LVOTO IN TGA TGA AND LVOTO DYNAMIC LVOTO IN...

SURGERY FOR TGA AND LVOTOLVOTO

Thomas L. Spray, M.D.

Chief, Cardiothoracic SurgeryAli L d W E d d Ch iAlice Langdon Warner Endowed ChairThe Children’s Hospital of Philadelphia

Professor of SurgeryProfessor of SurgeryUniversity of Pennsylvania

TGA WITH LVOTO-TGA AND LVOTO

TGA WITH LVOTODEFINITION

• TGA/IVS Or VSD With PV Abnormalities• TGA/IVS Or VSD With Subvalvar

LVOTO• TGA/IVS With Dynamic LVOTO• TGA/IVS With Dynamic LVOTO• TGA/VSD With Coarctation/Arch

HypoplasiaHypoplasia• Taussig-Bing Heart (Arch Obstruction)

DYNAMIC LVOTO IN TGA

TGA AND LVOTO

DYNAMIC LVOTO IN TGA• Caused By Septal Shift Seen Only In• Caused By Septal Shift – Seen Only In

TGA/IVS• More Common When Aorta Anterior And• More Common When Aorta Anterior And

Leftward• SAM Can Contribute To Gradient• SAM Can Contribute To Gradient• ASO Alone Reliably Relieves Obstruction

M b Si ifi t L t P bl Aft• May be Significant Late Problem After Atrial Switch

FIXED LVOTO IN TGA

TGA AND LVOTO

FIXED LVOTO IN TGA

P l V l Ab liti• Pulmonary Valve Abnormalities• Subvalvar Fibrous Membrane• Subvalvar Fibromuscular Tunnel• Septal Malalignment• Accessory Mitral Or Tricuspid Valve

Tissue• Anomalous Insertion Of MV On

Infundibular Septum (Straddle)

LVOTO IN TGA SURGICAL

TGA AND LVOTO

LVOTO IN TGA-SURGICAL OPTIONS

• ASO, Resection Of LVOTO • Atrial Switch Resection Of LVOTO• Atrial Switch, Resection Of LVOTO

Or LV-PA Conduit• Rastelli Or REV Procedure• Nikaidoh-Bex Procedure• Nikaidoh-Bex Procedure• Single Ventricle Repair (Fontan-

Kreutzer)

PULMONARY VALVE

TGA AND LVOTO

PULMONARY VALVE ABNORMALITIES

• May Occur With IVS Or VSD• Unequal Cusp Sizes And Bicuspid Valves, q p p ,

Dysplasia, Commissural Fusion• Abnormal PV May Not Be Obstructivey• May Result In Neoaortic Insufficiency/

Root Dilation• Rare For PS To Be Isolated; Usually In

Association With Complex SubAS, PV p ,Annular Hypoplasia (Rastelli)

TGA AND LVOTO

ASO WITH PV HYPOPLASIA AND

The image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.

ASO WITH PV HYPOPLASIA AND BICUSPID PV

From: Park, C S, et al. JTCVS 2010;139:135-8

TGA AND LVOTO

ASO WITH PV HYPOPLASIA AND BICUSPID PVThe image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.

ASO WITH PV HYPOPLASIA AND BICUSPID PV

From: Park, C S, et al. JTCVS 2010;139:135-8

SUBVALVAR FIBROUSTGA AND LVOTO

SUBVALVAR FIBROUS MEMBRANE

• Seen In IVS And VSD• Seen In IVS And VSD• Usually Resectable

SUBVALVAR FIBROMUSCULAR TUNNEL• Seen In IVS And VSD• Often Associated With Hypoplastic PVOften Associated With Hypoplastic PV• Difficult To Resect• Rastelli/REV Nikaidoh-Bex (Konno)Rastelli/REV, Nikaidoh Bex (Konno),

ASO All Options

ACCESSORY TV TISSUETGA AND LVOTO

ACCESSORY TV TISSUE• May Be Present With VSD, Usually Not

Outlet Typeyp• Prolapses Into LVOT• Usually Resectable Or Excluded By VSDUsually Resectable Or Excluded By VSD

PatchACCESSORY MV TISSUECC SSO V SSU

• Usually Seen With IVS, Rarely VSD (ECD Tissue)(ECD Tissue)

• Mitral Tissue Tags/ Accessory Tissue• Usually Resectable Without Damage ToUsually Resectable Without Damage To

Valve

TGA AND LVOTO

SEPTAL MALALIGNMENT

• Deviation Of Outlet Septum Into LVOTLVOT

• Seen Only In TGA/VSDy• Difficult To Resect

i i O G O i• Nikaidoh-Bex Or REV Good Options

ABNORMAL VALVETGA AND LVOTO

ABNORMAL VALVE INSERTION

Ab l Att h t MV Ch d O• Abnormal Attachment MV Chordae Or Pap Muscle To Outlet Septum

• Straddling TV• May Not Preclude ASO, VSD Closure, But

Resection May Not Be Possible• Various Techniques Such As Nikaidoh-Bex q

May Be Necessary• Single Ventricle Approach If Severe MV• Single Ventricle Approach If Severe MV

Abnormalities

ARTERIAL SWITCH/LVOTO TGA AND LVOTO

OPTIONS

From: Sohn, et al. Ann Thorac Surg 1998;66:842-8

ASO WITH LVOTOTGA AND LVOTO

ASO WITH LVOTO• Sohn, et al. Ann Thorac Surg

1998 66 842 81998;66:842-826 Pts. Mean Age 69 Days Mean Wt. 4.5

Kg.Bicuspid PV - 4, Dysplastic PV – 5LVOT Abnormalities – 17Preop Gradient 30 mm Hg (0-93)p g ( )Death – 2 (7.7%), No Late DeathsFreedom From Reop 87% @ 130 mo.Freedom From Reop 87% @ 130 mo.Gradient At Followup 0 mm Hg.

TGA AND LVOTO

ARTERIAL SWITCH/LVOT RESECTION-CAUSE OF OBSTRUCTION

From: Sohn, et al. Ann Thorac Surg 1998;66:842-8

TGA AND LVOTO

ARTERIAL SWITCH/LVOT RESECTION-GRADIENT ON FOLLOWUP

From: Sohn, et al. Ann Thorac Surg 1998;66:842-8

ASO WITH RESECTION LVOTO

TGA AND LVOTO

ASO WITH RESECTION LVOTO

• Sharma et al Ann Thorac SurgSharma et al. Ann Thorac Surg 2002;74:1986-91

23 i A 90 4 323 Pts., Median Age 90 Days Wt. 4.3 Kg.2 Early Deaths8 Pts. Mild AR At DischargeMean Followup 60 Months4/8 (50%) AR Progressed To Moderate Or Severe At Followup

RASTELLI PROCEDURE FOR TGA AND LVOTO

TGA/LVOTO• Kreutzer, et al. JTCVS 2000;120:211-23

101 Pts. Median Age 3.1 Yr., Wt. 12.6 Kg.PS - 73, Pulm. Atresia – 18 7 Early Deaths (7%)Risk factors For Death – Straddling TV, Long X-

ClampMedian FU 8.5 Yr. – 17 Late Deaths, 1 TransplantLate Reop/Arrhythmias/Sudden Death Common

TGA AND LVOTORASTELLI PROCEDURE-

PATIENT CHARACTERISTICS

From: Kreutzer, et. Al. JTCVS 2000;120:211-23

TGA AND LVOTO

RASTELLI PROCEDURERASTELLI PROCEDURE

From: Kreutzer, et. Al. JTCVS 2000;120:211-23

TGA AND LVOTO

RASTELLI PROCEDURE-RASTELLI PROCEDURESURVIVAL

From: Kreutzer, et. Al. JTCVS 2000;120:211-23

TGA AND LVOTO

RASTELLI PROCEDURE-RASTELLI PROCEDURESURVIVAL BY ERA

From: Kreutzer, et. Al. JTCVS 2000;120:211-23

TGA AND LVOTO

RASTELLI PROCEDURE-RASTELLI PROCEDUREFREEDOM FROM REINTERVENTION

From: Kreutzer, et. Al. JTCVS 2000;120:211-23

TGA AND LVOTORASTELLI PROCEDURE-

LVOT, RVOT REINTERVENTION

From: Kreutzer, et. Al. JTCVS 2000;120:211-23

TGA AND LVOTO

RASTELLI AND VSD ENLARGEMENTRASTELLI AND VSD ENLARGEMENT

From: Navabi, MA et al. JTCVS 2009;138:390-6

TGA AND LVOTO

RASTELLI AND VSD ENLARGEMENTThe image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.

RASTELLI AND VSD ENLARGEMENT

From: Navabi, MA et al. JTCVS 2009;138:390-6

TGA AND LVOTO

RASTELLI AND VSD ENLARGEMENTThe image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.

RASTELLI AND VSD ENLARGEMENT

From: Navabi, MA et al. JTCVS 2009;138:390-6

TGA AND LVOTO

RASTELLI AND VSD ENLARGEMENTThe image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.

RASTELLI AND VSD ENLARGEMENT

From: Navabi, MA et al. JTCVS 2009;138:390-6

REV PROCEDURETGA AND LVOTO

(REPARATION A L’ETAGE VENTRICULAIRE)

• Extensive Resection Of Conal Septum +/• Extensive Resection Of Conal Septum +/-VSD Enlargement

• Direct Reimplantation Of Pulmonary• Direct Reimplantation Of Pulmonary Trunk On RV

• LeCompte Maneuver• LeCompte Maneuver• VSD Size And Abnormal TV Attachments

To Septum Not Limiting LessTo Septum Not Limiting – Less Recurrent LVOTO

• No Conduit Reoperation Less Likely• No Conduit – Reoperation Less Likely Despite PR

TGA AND LVOTO

RASTELLI VS LECOMPTE(REV)RASTELLI VS LECOMPTE(REV)

From: Lee, et al. Eur JCTS 2004;25:735-41

RASTELLI VS LECOMPTE(REV)

TGA AND LVOTO

RASTELLI VS LECOMPTE(REV)

From: Lee, et al. Eur JCTS 2004;25:735-41

TGA AND LVOTO

RASTELLI VS LECOMPTE(REV)( )

From: Lee, et al. Eur JCTS 2004;25:735-41

TGA AND LVOTO

RASTELLI VS LECOMPTE(REV)RASTELLI VS LECOMPTE(REV)

From: Lee, et al. Eur JCTS 2004;25:735-41

TGA AND LVOTO

AORTIC TRANSLOCATION VS RASTELLIThe image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.

AORTIC TRANSLOCATION VS. RASTELLI

From: Morrell, VO et al. Ann Thorac Surg 2005;79:2089-93

TGA AND LVOTO

AORTIC TRANSLOCATION (NIKAIDOH-BEX)AORTIC TRANSLOCATION (NIKAIDOH BEX)

From: Bautista-Hernandez, V, et al. JACC 2007;49:485-90

TGA AND LVOTO

AORTIC TRANSLOCATION (NIKAIDOH-BEX)AORTIC TRANSLOCATION (NIKAIDOH-BEX)

From: Bautista-Hernandez, V, et al. JACC 2007;49:485-90

TGA AND LVOTO

AORTIC TRANSLOCATION (NIKAIDOH-BEX)AORTIC TRANSLOCATION (NIKAIDOH-BEX)

From: Bautista-Hernandez, V, et al. JACC 2007;49:485-90

TGA AND LVOTO

RASTELLI REV OR NIKAIDOH?RASTELLI, REV OR NIKAIDOH?

From: Hu, S, et al. JTCVS 2008;135:331-8

TGA AND LVOTO

AORTIC TRANSLOCATIONThe image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.

AORTIC TRANSLOCATION

From: Hu, S, et al. JTCVS 2008;135:331-8

TGA AND LVOTO

MODIFIED REV PROCEDUREMODIFIED REV PROCEDURE

From: Hu, S, et al. JTCVS 2008;135:331-8

TGA AND LVOTO

PREOPERATIVE CHARACTERISTICSPREOPERATIVE CHARACTERISTICS

From: Emani, SM, et al. Circulation 2009;120:S53-58

TGA AND LVOTO

LATE COMPLICATIONSLATE COMPLICATIONS

From: Emani, SM, et al. Circulation 2009;120:S53-58

TGA AND LVOTO

FREEDOM FROM LVOT REINTERVENTIONFREEDOM FROM LVOT REINTERVENTION

From: Emani, SM, et al. Circulation 2009;120:S53-58

SUMMARYTGA AND LVOTO

SUMMARY• Surgical Results Good – Low Mortality, Similar To

TGA/VSD• TGA/VSD Or TGA/IVS With Isolated PV

Abnormality – ASOTGA/VSD/IVS With S b al ar Obstr ction Choice Of• TGA/VSD/IVS With Subvalvar Obstruction – Choice Of

Operation Varies Based On Resectability Of LVOT, PV Annulus Size

• Nikaidoh-Bex Useful For VSD/PS With Moderate PV Hypoplasia Or Septal Malalignment

• Rastelli/REV For Pulmonary Atresia, Significant Valvar y , gPS And Hypoplasia –REV May Have Less Reop

• Results With Rastelli Suboptimal – LVOTO & Conduit Changes CommonChanges Common

• Late Function Of AV And Root Still Unknown

TGA AND LVOTO

QUESTIONSQUESTIONS• When is PV too small for ASO?• What is long-term function of bicuspid PV?• Is the fate of the AV and root same as in Ross

operation?• Is the aortic valve at more risk for late AR after aorticIs the aortic valve at more risk for late AR after aortic

translocation?• Is coronary transfer with aortic translocation

i t d ith l t l i / t i ?associated with late occlusion/stenosis?• Is the REV operation associated with less LV

dysfunction and recurrent LVOTO than the Rastelli?

• Is VSD enlargement technique at Rastelli or REV associated with long-term survival?associated with long term survival?

• Will REV patients all require PV implantation late?