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
Â
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
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