SHA24/064003

MANAGEMENT OF THE SINGLE VENTRICLE AND POTENTIALLY OBSTRUCTIVE SYSTEMIC VENTRICULAR OUTFLOW TRACT Joined 24th SHA & 10th GHA Conference 13-16 February 2013, Riyadh, Saudi Arabia Bahaaldin Alsoufi, MD King Faisal Specialist Hospital and Research Center Riyadh, Saudi Arabia

BACKGROUND • Multi-stage palliation: current strategy for SV anomalies • Success depends on favourable hemodynamic conditions • SVOTO: hypertrophy and unfavourable hemodynamics • Inability to progress or late failure

PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA RV BVF

Freedom et al, Circulation 86

PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA – DILV with TGA

RA

LA

RV BVF

LV

LV

AO

BVF

RV

PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA – DILV with TGA – Unbalanced AVSD

PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA – DILV with TGA – Unbalanced AVSD – DORV with uncommitted VSD – D- or L-TGA with VSD and small RV

PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA – DILV with TGA – Unbalanced AVSD – DORV with uncommitted VSD – D- or L-TGA with VSD and small RV

• Hypertrophy of sub-aortic conus: – DORV +/- small LV +/- MS/MA

PATIENTS AT RISK

MANAGEMENT • Determinant factors: – Semilunar valves – BVF / VSD – Arch obstruction

Neonate

Neonatal SVOT relief DKS

+/- Arch

SVOT relief with BCPC Neonatal PAB +/- COA

VSD enlargement

BCPC DKS

Palliative switch

VSD enlargement

Neonate

Neonatal SVOT relief DKS

+/- Arch

X

VSD enlargement

Palliative switch

SVOT relief with BCPC Neonatal PAB +/- COA

BCPC DKS VSD enlargement

• Address SVOTO at neonatal age: – DKS + shunt (+ arch = Norwood)

• Address SVOTO at BCPC: – 1st stage: PAB +/- COA – 2nd stage: • BCPC + DKS • BCPC + VSD enlargement

DKS + SHUNT • Advantages: – Address SVOTO early – Arch augmentation

• Disadvantages: – CPB and DHCA in neonates – Complex post-operative course – Early and interim mortality

Modified Norwood Operation for Single Left Ventricle and Ventriculoarterial Discordance: An Improved Surgical Technique Ralph S. Mosca, MD, Hani A. Hennein, MD, Thomas J. Kulik, MD, Dennis C. Crowley, MD, Erik C. Michelfelder, MD, Achi Ludomirsky, MD, Edward L. Bove, MD

N=20 Absolute BVF size: increase with time in 50% of patients Not predictable Indexed BVF size: 20% overall decrease

Ann Thorac Surg 1997

Modified Norwood Operation for Single Left Ventricle and Ventriculoarterial Discordance: An Improved Surgical Technique Ralph S. Mosca, MD, Hani A. Hennein, MD, Thomas J. Kulik, MD, Dennis C. Crowley, MD, Erik C. Michelfelder, MD, Achi Ludomirsky, MD, Edward L. Bove, MD

Ann Thorac Surg 1997

Ann Thorac Surg 1997

Norwood / ASO

COA + PAB

Ann Thorac Surg 1995

PAB + COA • Advantages: – No CPB or DHCA – Can be done via thoracotomy

• Disadvantages: – Residual arch gradient (HAA) – Distortion of PAs – Distortion of PV? – Development of SVOTO

Sub-As in 31/43 (72%) of PAB SV Pts Mean PAB age 0.21 Y Mean Sub-AS diagnosis age 2.52 Y

PAB produces myocardial hypertrophy and accelerates potential stenosis

Circulation 1986

BVFAI (cm2/m2)

Smaller BVF = higher chance of sub AS BVFAI < 2 cm2/m2

DKS

Early Obst.

Late Obst.

JACC 1992

BVFAI (cm2/m2)

COA = higher chance of sub AS

COA

No COA

JACC 1992

Effect of volume unloading with CPC

JTCVS 2012

Decrease in VSD size with CPC and PAB More difference after CPC

JACC 1995

BCPC + DKS • Advantages: – Stable modified in series physiology – Address SVOTO – Concomitant arch – No heart block

• Disadvantages: – Seminlunar valve function – Lt main bronchus and LPA

DKS

Originally described for D-TGA

Deleon et al, Ann Thorac Surg 1994

DOUBLE BARREL DKS

Carter et al, Ann Thorac Surg 1994

Preserved semilunar valve function with double barrel technique

JTCVS 2011

KFSHRC EXPERIENCE 1997-2012 n=36 Males 69% Age Weight

8.9 M 6.7 kg

(3.6 M – 9.1 Y) (5 – 27 Kg)

• All had prior palliation (n=36): – PAB – COA/Arch repair – Atrial septectomy – PPI

n=35 n=11 n=8 n=1

Survival

83%

All deaths: first 6 months None SVOT / DKS related Years after DKS

86%

TCPC + DKS

69%

Survival

BCPC + DKS

P=0.30

Years after DKS

SVOT gradient (mm Hg)

P<0.001

P=1.0

23.4 Âą 18.7

0

0

Semilunar Valves

VSD ENLARGEMENT • Advantages: • Independent of semilunar valves • Can manage late SVOTO • Disadvantages: • Heart block • Inadequate SVOTO relief • Recurrent SVOTO • Ventriculotomy

VSD ENLARGEMENT

1/9 operative death 3/9: recurrent SVOTO 1/9 PPI

Ann Thorac Surg 2001

VSD ENLARGEMENT

Ann Thorac Surg 2008

L AVV BVD to AO Sub PS Dominant LV

R AVV

Neonate

Obstructive VSD

Non-Obstructive VSD

DKS + shunt Norwood

COA + HAA

No HAA

Norwood

COA PAB + COA Norwood

No COA PAB

PAB

BCPC + DKS

BCPC + VSD enlargement PS PI DORV remote VSD

Late presentation

PV sutured

PV not sutured

VSD enlargement

? DKS VSD enlargement

3 patients with previously transected MPA No SVOTO No Semilunar valve insufficiency

Ann Thorac Surg 1999

PALLIATIVE SWITCH • Advantages: • Can be done in neonates alternative to Norwood • No LPA entrapment • Unobstructed SVOT

• Disadvantages: • Coronaries • PAs

• Root • Concomitant PAB or shunt ?

0/14 early or late death 14/14 BCPC 11/14 TCPC + 3/14 awaiting TCPC 64% PA augmentation at TCPC

Ann Thorac Surg 2013

VSD ENLARGEMENT

Kirkland/BarrattBoyes Cardiac Surgery 3rd Edition

DKS

PALLIATIVE SWITCH