1. 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

2. Part 1
Introduction

3. 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

4. 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

5. Part 2
METHODOLOGY

6. 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

7. 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)

8. Part 3
RESULTS

9. 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)

10. Era of repair

11. Temporal trends in median age at repair*
*Excluded 1950s and 2000s as too few patients in each category

12. 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 1
>1
65.4
29.0
5.6
Repair
Infundibular resection
Transannular patch
VSD closure and pulmonary homograft
19.1
30.1
5.9
45.0

13. Overall survival lower than general population
Log rank test p <0.001
Tetralogy cohort
Control group

14. 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

15. 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
*Univariate mode only

16. Quality of life NYHA class Median peak VO2 69.5% predicted
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)

17. MRI n=181
RV volumes
RV ejection fraction

18. CPET n=169
% predicted VO2max
VE/VCO2 slope

19. RVEF versus peak VO2
Spearman’s rho 0.226
p = 0.013

20. Reintervention Procedure N PVR 166 Surgical 147 Percutaneous 192 3
110 14
Percutaneous 19 17
Early revision 24
AVR/root replacement
Tricuspid valve 8
Balloon pulmonary angioplasties

21. Historical trends in repeat intervention

22. Freedom from repeat intervention
Total reintervention
PVR

23. Multivariate analysis for reintervention
Variable HR
95% CI
P-value
Any reintervention
Transannular patch
1.72
1.23 – 2.39
0.001
Nonclassical ToF
2.95
1.57 – 5.54
Older age at repair
0.96
0.92 – 1.00
0.008
PVR only
1.79
1.26 – 2.56
4.22
2.16 – 8.25
<0.001
0.92
0.87 – 0.96

24. PVR and survival
Log rank test p = 0.539
PVR group
Severe PR group

25. The effect of PVR on RV size and function
Indexed RVEDV ml/m2
RV ejection fraction %
P < 0.001
P = 0.154
N=17

26. The effect of PVR on exercise performance
Peak VO2 as % predicted
VE/VCO2 slope
P = 0.623
P = 0.050
N=16

27. 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

28. Freedom from arrhythmia

29. 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

30. 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%

31. Freedom from device insertion

32. Part 4
SUMMARY

33. 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

34. 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

35. 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

36. Part 4
QUESTIONS