Download P - AATS

Ultra long term outcomes in adult
survivors of tetralogy of Fallot and the
effect of pulmonary valve replacement
Dobson R1,2, Danton M2, Walker N2, Tzemos, N1,Walker H2
1Institute
of Cardiovascular and Medical Sciences, University of Glasgow
2Scottish Adult Congenital Cardiac Service, Golden Jubilee National Hospital
Part 1
INTRODUCTION
The problem
• Survival to adulthood of infants with tetralogy of Fallot
(ToF) now exceeds 90% in modern cohorts1,2
• Form a significant proportion of the workload of adult CHD
services
• The ability of post-ToF repair interventions to modify the
long term prognosis for these patients has not been fully
defined
1Ide
et al 2009, 2Park et al 2010
Objective
• To define the long term outcomes of adult survivors of
ToF with respect to
– Survival, functional capacity and adverse events
– The effect of pulmonary valve replacement on clinical and
functional outcome
Part 2
METHODOLOGY
Data collection
• National centralized model for ACHD care in Scotland
• Computerised database
• Electronic records were where possible corroborated with
op notes and medical certificate of cause of death
Methods
• Overall survival analysis; KM curve compared to age and
gender matched controls
• Morbidity outcomes
–
–
–
–
Ventricular arrhythmia
Atrial arrhythmia
Device (pacemaker or ICD)
Reintervention and PVR
• Current functional status (clinical / CPET / CMR data)
Part 3
RESULTS
Baseline characteristics
• 376 patients (male:female 59:41) post ToF repair who
survived to at least age 16
• Mean age at repair 5.2 years (SD 7.3); median 3 years
• Mean follow-up from repair 28.3 years (SD 9.4)
Era of repair
Temporal trends in median age at repair*
*Excluded 1950s and 2000s as too few patients in each category
ToF subtypes and repair details
Variable
%
Subtype
Classical
ToF-PA, ToF absent pulmonary valve, ToF-AVSD
Unknown
93.6
4.1
1.3
Palliative shunt
0
1
>1
65.4
29.0
5.6
Repair
Infundibular resection
Transannular patch
VSD closure and pulmonary homograft
Unknown
19.1
30.1
5.9
45.0
Overall survival lower than general population
Log rank test p <0.001
Tetralogy cohort
Control group
Deaths
• 15 patients died at a mean age of 49 +/- SD 13.7 years
• Cause of death
–
–
–
–
–
–
Heart failure (5)
Postoperative – PVR (3)*
Sepsis (3)
Sudden (2)
Stroke (1)
Malignancy (1)
*From 166 PVR procedures
Multivariate analysis for death
Variable
HR
95% CI
P-value
Older age at repair
1.11
1.04 – 1.19
0.003
Male gender
5.64
1.08 – 29.49
0.041
Nonclassical ToF
13.43
2.51 – 71.92
0.002
QRS duration*
1.07
1.02 – 1.11
0.003
*Univariate mode only
Quality of life
• NYHA class
– I: 87.5%,
– II: 5.3%
– III: 0.5%
• Median peak VO2 69.5% predicted
• Social deprivation score = 4.1
– Scottish population mean 4; p=0.51
• Total Fertility Rate 0.18 (1.61 for national data 2013)
MRI n=181
RV volumes
RV ejection fraction
CPET n=169
% predicted VO2max
VE/VCO2 slope
RVEF versus peak VO2
Spearman’s rho 0.226
p = 0.013
Reintervention
Procedure
N
PVR
166
Surgical
147
1
2
3
110
14
3
Percutaneous
19
1
2
17
1
Early revision
24
AVR/root replacement
2
Tricuspid valve
8
Balloon pulmonary angioplasties
24
Historical trends in repeat intervention
Freedom from repeat intervention
Total reintervention
PVR
Multivariate analysis for reintervention
Variable
HR
95% CI
P-value
Transannular patch
1.72
1.23 – 2.39
0.001
Nonclassical ToF
2.95
1.57 – 5.54
0.001
Older age at repair
0.96
0.92 – 1.00
0.008
Transannular patch
1.79
1.26 – 2.56
0.001
Nonclassical ToF
4.22
2.16 – 8.25
<0.001
Older age at repair
0.92
0.87 – 0.96
<0.001
Any reintervention
PVR only
PVR and survival
Log rank test p = 0.539
PVR group
Severe PR group
The effect of PVR on RV size and function
Indexed RVEDV ml/m2
P < 0.001
N=17
RV ejection fraction %
P = 0.154
The effect of PVR on exercise performance
Peak VO2 as % predicted
P = 0.623
N=16
VE/VCO2 slope
P = 0.050
Prevalence of arrhythmia
• Atrial arrhythmia in 13.3%, ventricular arrhythmia in 3.4%
• Therapy
– 12.8% on regular antiarrhythmics
– 5.1% ICD
– 2.1% had radiofrequency ablation
Freedom from arrhythmia
Multivariate analysis for arrhythmia
• Atrial arrhythmia
– Older age at repair conferred hazard ratio of 1.10 (95% CI 1.07 –
1.13) p = <0.001
• No significant variables identified for ventricular
arrhythmia
Device insertion (pacemaker or ICD)
• 9% of patients overall
• Device type
– Pacemaker in 4% (VVI 0.8% and DDD in 3.2%)
– ICD in 4.8%
– CRT-D in 0.3%
Freedom from device insertion
Part 4
SUMMARY
Mortality
• Long term (>30 years) survival remains excellent
– For patients who survive to age >16
• Heart failure is the main cause of death
• Older age at repair, non-classical forms of ToF, and male
gender confer increased risk
Morbidity
• Arrhythmia is common
• High rates of repeat intervention, mainly PVR, performed
with low mortality
• PVR reduces RV volumes but does not improve exercise
capacity
Limitations and future directions
• Single center retrospective cohort
• Functional data is cross-sectional for a heterogenous
group
• Historical loss of follow-up – patients geographically
remote from surgical center may still be under the radar
• Creation of an international registry will enable far more
powerful and robust analysis of prognosis and
intervention
Part 4
QUESTIONS