1. Adult Congenital Heart Disease
Sudden Death in
Adult Congenital Heart Disease
(GUCH Patients)
Berardo Sarubbi
U.O.C. di Cardiologia
U.O. Cardiopatie Congenite dell’Adulto
Seconda Università degli Studi di Napoli - A.O. Monaldi

2. Incidence 8 per thousand In the last 20 years 90.000 pts with CHD
Adults Congenital Heart Disease
Italy:
Incidence 8 per thousand
In the last 20 years pts with CHD
pts with CHD aged >18 yrs
pts with CHD aged <18 yrs

3. “Pediatric congenital cardiac becomes a postoperative adult: the changing population of congenital heart disease” Perloff JK. Circulation 1973; 47:
…it is simple a matter of time before a population of adult with congenital heart disease would emerge.

4. Percento
Congenital Heart Disease in the General Population Changing Prevalence and Age Distribution. J. Marelli et al. Circulation. 2007;115:

5. Changes of GUCH population over the time
ASD/VSD
TOF
Mustard/Senning
Fontan
HLHS
Truncus
2011
ASD/VSD
TOF
Mustard/Senning
Fontan
2021
HLHS
Truncus 5

6. CLINICAL EVENTS AFTER SURGICAL CORRECTION: ventricular dysfunction, arrhythmias, re-intervention
Atrial septal defect
Pulmonary stenosis
Anomalous pulmonary drenage 5%
Partial AV Canal
10-15%
Complete AV Canal
50%
Aortic Valvulotomy
Mustard
Senning
Fontan
100%

7. Causes of Death in GUCH
Oechsling et al Am J Cardiol 2000 7

8. Event  GUCH Sudden Death
Other (7.4%)
Haemorrhagic
17 (18.1%)
Unknown
37 (39.4%)
Arrhythmic
33 (35.1%)
Sudden death is the most frequent cause of late mortality in adults with CHD
Sarubbi B., Somerville J.: Sudden death in grown-up congenital heart (GUCH) patients: a 26-year population-based study. JACC 1999

9. Late Death in Repaired Tetralogy
793 adult pts ( )
33 pts died (4.2% mortality)
Gatzoulis et al Lancet 2000

10. CAUSES OF ADMISSION FOR GUCH
Report of the British Cardiac Society - Heart 2002;88:i1-i14

11. Percentage of Fallot admitted for arrhythmias
GUCH Admission - Year 2010
Percentage of Fallot admitted for arrhythmias
A.O. Monaldi Napoli
Circa 1/3 dei pazienti ricoverati per aritmie presso la UOS di Cardiopatie Congenite dell’Adulto A.O. Monaldi nel 2005 sono Fallot.

14. Adult Congenital Heart Disease Pts Risk stratification for S.D.
Clinical History
ECG Parameters
SAECG/LP
EPS
RV/LV Emodinamics, Volume, Function
Tissutal characterization
Autonomic Nervous System

15. Clinical History Arrhythmias in GUCH RISK STRATIFICATION
Previous Surgical Intervention
Previous Palliative Intervention
Age at operation
Type of Surgical Approach
Follow-up duration

16. SD Incidence between 0.5 to 5.5%
TOF: Arrhythmic Risck
SD Incidence between 0.5 to 5.5%
“Scar related” VT
Ventriculotomy
Interventricular Patch
RVOT Patch

17. The best predictors of SCD
Presence of symptoms of Arrhythmia or Heart Failure
History of documented AFL/AF
The best predictors of SCD

18. Arrhythmias, Heart Failure and SD in GUCH
SVT
Heart Failure
Sistolic-diastolic
dysfunction
Increased HR
Neurohormonal
Activation
Reduction of the
ventricle filling time
Reduction
in C.O.

19. Adult Congenital Heart Disease Pts Risk stratification for S.D.
Clinical History
ECG Parameters
SAECG/LP
EPS
RV/LV Emodinamics, Volume, Function
Tissutal characterization
Autonomic Nervous System

21. Gatzoulis M.A., et al:
Mechano-electrical Interaction in Tetralogy of Fallot.
Circulation 1995

22. SD not related to width of QRS
Sarubbi B., Somerville J.: Sudden death in grown-up congenital heart (GUCH) patients: a 26-year population-based study. Journal American College of Cardiology 1999.
O= Repaired Fallot O= Unrepaired Fallot
SD not related to width of QRS

23. Measurement of QRS is difficult
Can be operator dependent
Can be influenced by the presence of conduction abnormalities which reduce its accuracy and reproducibility.

24. Gatzoulis et al. Lancet 2000

26. Adult Congenital Heart Disease Pts Risk stratification for S.D.
Clinical History
ECG Parameters
SAECG/LP
EPS
RV/LV Emodinamics, Volume, Function
Tissutal characterization
Autonomic Nervous System

27. Signal Average ECG High accuracy of Signal Average ECG
Time domain
High accuracy of Signal Average ECG
to predict severe VA
Pts operated on for TOF :
CONTROLS
ALL PTS
PTS WITH MINOR ARRYTHMIA
PTS WITH SEVERE ARRYTHMIA
QRS 40 (ms)
125  4 *
162  29
156  29 #
181.5  19.6
LAS 40 (ms)
33.6  13.4
32  22
28.5  19.8 §
45.1  26.7
RMS 40 (V)
26  8
41  32
45.3  34.6
26  16
Frequency domain X Y Z
*p<0.001 vs pts with minor and severe
arrhythmias.
#< 0.01vs pts with severe arrhythmias

28. J. Cardiovasc. Electrophysiol. 2005

29. Adult Congenital Heart Disease Pts Risk stratification for S.D.
Clinical History
ECG Parameters
SAECG/LP
EPS
RV/LV Emodinamics, Volume, Function
Tissutal characterization
Autonomic Nervous System

30. EPS inducible sustained VT  VT or SCD Khairy et al, Circulation 2004

31. 7% of pts with neg. VSTIM studies died during follow-up
37% of pts with documented sustained VT/VF had no inducible ventricular arrhythmia with VSTIM
Alexander M.E, Walsh E.P.:
J.Cardiovasc. Electr.

32. Very low positive predictive value (20%) of VSTIM to predict SCD
Proarrhythmia of antiarrhythmic drugs
Management of pts with spontaneous VT and non inducible arrhythmias
Alexander M.E, Walsh E.P.:
J.Cardiovasc. Electr.

33. Adult Congenital Heart Disease Pts Risk stratification for S.D.
Clinical History
ECG Parameters
SAECG/LP
EPS
RV/LV Emodinamics, Volume, Function
Tissutal characterization
Autonomic Nervous System

34. ATRIAL FLUTTER and RV FUNCTION after MUSTARD
1 normal; 2 mild depression; 3 moderate depression; 4 severe depression.
Gelatt M J et al. JACC, Jen1997: 29 (1);

35. Gatzoulis et al. Lancet 2000

36. Normal-Mild
LV systolic dysf.
The combination of QRS ≥180ms and significant LV syst. dysfunction has a positive predictive value for SCD of 66% and negative predictive value of 93%
Mod-Severe
LV systolic dysf.

37. Right and Left ventricular interaction
At rest (MRI)
Davlouros et al JACC 2002

38. Adult Congenital Heart Disease Pts Risk stratification for S.D.
Clinical History
ECG Parameters
SAECG/LP
EPS
RV/LV Emodinamics, Volume, Function
Tissutal characterization
Autonomic Nervous System

39. MYOCARDAL FIBROSIS AND LIFE THREATENING VENTRICULAR ARRHYTHMIAS

40. VT ablated at site RVOT scar
3D Late Gad CMR
3D CMR EP Merge
VT ablated at site RVOT scar
RVOT scar

41. Adult Congenital Heart Disease Pts Risk stratification for S.D.
Clinical History
ECG Parameters
SAECG/LP
EPS
RV/LV Emodinamics, Volume, Function
Tissutal characterization
Autonomic Nervous System

42. Circulation 2002

43. ToF patients with VT have significant impairment of sympatho-vagal balance, characterized by a reduction of vagal drive

44. strategies to prevent
SD in GUCH

45. Issues for the use of AICD in ACHD Indications
Inappropriate shocks and lead failure
Unique anatomical situations in CHD
Technical difficulties

46. CHD patients are not mentioned as a different group and it is assumed that general guidelines are applicable to these patients as there are not yet clear indications for AID therapy in this group

47. Long term efficacy and safety of this approach in ACHD in unknown
No data in the literature comparing medical therapy with AID implantation in either paediatric or adult CHD population
Attempt to ablate the VT focus either in the EP lab or in the operating room in ACHD before considering AID implantation
Long term efficacy and safety of this approach in ACHD in unknown
International J. of Cardiology 2008

48. European Heart Journal 2006

49. 6 Epicardial; 14 transvenous Therapy-rate 2.8 per patient-years of F-U
20 pts aged 16±6yrs
11 CHD
6 Epicardial; 14 transvenous
Therapy-rate 2.8 per patient-years of F-U
53% appropriate; 47% inappropriate
1.5 appropriate per patient-year of FU
1.3 inappropriate per patient-year of FU
PACE 2004; 27:

50. PACE 2004; 27:

51. J. Cardiovasc. Electrophysiol.
15:72-76; 2004

52. Epicardial lead malfunction is common
on long -term follow-up.
Some leads have a failure of 28% at 4yrs

53. Do we really need so many risk factors ?

54. Malignant arrhythmias occur even in patients with:
no residual lesion
no QRS prolongation
no ventricular dysfunction
The recognition of those who would benefit from an ICD remains a clinical challenge
PACE 2004; 27:47-51

56. ...the finding that the diagnosis of TOF was associated with less appropriate shocks might imply that the abundance of risk factors described for this subgroup has decreased the threshold to consider ICD therapy in this group (more TOF patients had an ICD as primary prevention…)
Yap S. et al.: Eur. Heart J. 2006

57. “Pediatric congenital cardiac becomes a postoperative adult: the changing population of congenital heart disease” Perloff JK. Circulation 1973; 47:
… we are obliged to look beyond the present and define our ultimate goal: the quality of long-term survival…