SHA24/055005

CURRENT OUTCOMES OF THE GLENN BIDIRECTIONAL CAVOPULMONARY CONNECTION FOR SINGLE VENTRICLE PALLIATION Waheed Raedi Abdullah Al-Wadei Zohair Al-Halees Abid Awan Bahaaldin Alsoufi King Faisal Specialist Hospital & Research Center Riyadh, Saudi Arabia

Background • BCPC Established intermediary step in multi-stage palliation of SV anomalies • Improved outcomes by reaching stable in-series circulation • Stabilization of mortality risk after BCPC • Less favourable outcomes in some groups

Aims and Methods • Describe current clinical outcomes • Examine risk factors: – Survival – Failure to progress to next palliation stage – Unplanned re-operation

• Competing risks analysis: – Death – Failure to progress – Proper progression

BCPC Cohort 2002-2007 n=227 Males 61% Age Weight

7.6 M 6.2 kg

(IQR 6-10.8) (IQR 5.2-7.4)

Dominant Ventricle Morphology

Prior Palliation

Atrial septectomy (n=25)

PA reconstruction (n=14)

TAPVC (n=7)

COA (n=4)

PPI (n=3)

VSD enlargement (n=1)

SVC

Operative Details • Concomitant surgery: – – – – – – – –

PA reconstruction AVV repair DKS VSD enlargement TAPVC repair LVOTO relief Arch repair Other

• Fontan preparation

n=80 n=18 n=4 n=4 n=4 n=3 n=3 n=4

n=34

RESULTS

BCPC n=227

Death before Fontan n=38

Fontan n=172

Not Fontan candidate n=7

Awaiting Fontan n=10

Competing Risks Analysis After BCPC Alive with BCPC

Fontan

Death

Competing Risks Analysis After BCPC Alive with BCPC

Early death

Fontan

Death

Competing Risks Analysis After BCPC Alive with BCPC

Fontan Progression

Death

Competing Risks Analysis After BCPC Alive with BCPC

Fontan Continuous death

Death

Competing Risks Analysis After BCPC At 1Y: 13% dead 9% Fontan

Competing Risks Analysis After BCPC At 5Y: 17% dead 76% Fontan

SURVIVAL

Survival

Early risk Constant attrition

Survival

High early hazard

Constant low hazard

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Risk Factors For Death After BCPC

Risk Factor HLHS

HR 2.2

P 0.06

Survival

Risk Factors For Death After BCPC

Risk Factor

HR

P

HLHS

2.2

0.06

S1P or PAB

1.9

0.06

Survival

Â

Risk Factors For Death After BCPC

Risk Factor

HR

P

HLHS

2.2

0.06

S1P or PAB

1.9

0.06

Dominant RV

2.6

0.01

Survival

Â

Risk Factors For Death After BCPC

Risk Factor

HR

P

HLHS

2.2

0.06

S1P or PAB

1.9

0.06

Dominant RV

2.6

0.01

PVRI > 3 WU/ M2

4.1

0.001

Survival

Â

Risk Factors For Death After BCPC

Risk Factor

HR

P

HLHS

2.2

0.06

S1P or PAB

1.9

0.06

Dominant RV

2.6

0.01

PVRI > 3 WU/ M2

4.1

0.001

Weight < 5.2 or > 8.5

0.4

0.03*

Survival

Â

Risk Factors For Death After BCPC

Risk Factor HLHS

HR 2.2

S1P or PABMulti-variable 1.9

P 0.06

analysis: 0.06

PVRI > 3 WU/M2 Dominant RV 2.6 0.01 Dominant RV Palliation other PVRI > 3 WU/ M2 4.1 than shunt 0.001 Weight < 5.2 or > 8.5

0.4

0.03*

Â

Non significant factors

Risk Factor

HR

P

AVV repair

2.0

0.16

MPA > 15 mm Hg

1.1

0.72

Bilateral SVC

1.4

0.27

Fontan preparation

0.3

0.12

Survival

OUTCOMES FOLLOWING FONTAN

BCPC n=227

Death before Fontan n=38

Death n=17

Fontan n=172

Not Fontan candidate n=7

Alive without reoperation n=145

Awaiting Fontan n=10

Cardiac reoperation n=10

Alive n=4

Death n=6

Competing Risks Analysis After Fontan Alive without reoperation

Reoperation

Death

Competing Risks Analysis After Fontan Alive without reoperation At 3Y: 10% dead 6% reoperation

Reoperation

Death

OVERALL SURVIVAL

Survival

74%

Summary • Continuous failure and attrition following BCPC • Outcomes largely influenced by underlying deformity: – LV morphology better (TA, DILV) – RV morphology worse (HLHS, DORV)

• Elevated PVR persistent risk factor • Continuous monitoring for late failures for timely OHTX referral

Thank you