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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. Adults Congenital Heart Disease. Italy: Incidence 8 per thousand - PowerPoint PPT Presentation
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Sudden Death in
Adult Congenital Heart Disease (GUCH Patients)
Sudden Death in
Adult Congenital Heart Disease (GUCH Patients)
Berardo SarubbiU.O.C. di Cardiologia
U.O. Cardiopatie Congenite dell’AdultoSeconda Università degli Studi di Napoli - A.O. Monaldi
Berardo SarubbiU.O.C. di Cardiologia
U.O. Cardiopatie Congenite dell’AdultoSeconda Università degli Studi di Napoli - A.O. Monaldi
Adults Congenital Heart Disease Adults Congenital Heart DiseaseItaly:
• Incidence 8 per thousand
• In the last 20 years 90.000 pts with CHD
Italy:
• Incidence 8 per thousand
• In the last 20 years 90.000 pts with CHD
100.000 pts with CHD aged >18 yrs70.000 pts with CHD aged <18 yrs100.000 pts with CHD aged >18 yrs70.000 pts with CHD aged <18 yrs
““Pediatric congenital cardiac becomes a Pediatric congenital cardiac becomes a postoperative adult: the changing population of postoperative adult: the changing population of
congenital heart disease”congenital heart disease”Perloff JK. Perloff JK. Circulation Circulation 1973; 47:606-6191973; 47:606-619
……it is simple a matter of time before a it is simple a matter of time before a population of adult with congenital population of adult with congenital heart disease would emerge.heart disease would emerge.
35
65
36
64
40
60
49 51
0
10
20
30
40
50
60
70
80
1985 1990 1995 2000
Adults
Children
Perc
en
to
Congenital Heart Disease in the General Population Changing Prevalence and Age Distribution. J. Marelli et al. Circulation. 2007;115:163-172.
Changes of GUCH population over the timeChanges of GUCH population over the time
ASD/VSD
TOF
Mustard/Senning
Fontan
HLHS
Truncus
20 30 40 50 60
2011
2021
20 30 40 50 60
ASD/VSD
TOF
Mustard/Senning
Fontan
HLHS
Truncus
CLINICAL EVENTSCLINICAL EVENTSAFTER SURGICAL CORRECTION:AFTER SURGICAL CORRECTION:ventricular dysfunction, arrhythmias, re-ventricular dysfunction, arrhythmias, re-
interventionintervention
Atrial septal defectAtrial septal defect Pulmonary stenosis Pulmonary stenosis Anomalous pulmonary Anomalous pulmonary drenagedrenage
5%5%
Partial AV CanalPartial AV Canal 10-15%10-15% Complete AV CanalComplete AV Canal 50%50% Aortic ValvulotomyAortic Valvulotomy MustardMustard SenningSenning FontanFontan
100%100%
Oechsling et al Am J Cardiol 2000
Causes of Death in GUCHCauses of Death in GUCH
Event GUCH Sudden Death
Event GUCH Sudden Death
Arrhythmic 33 (35.1%)Arrhythmic
33 (35.1%)
Haemorrhagic 17 (18.1%)
Haemorrhagic 17 (18.1%)
Other 7 (7.4%)
Other 7 (7.4%)
Unknown37 (39.4%)Unknown
37 (39.4%)
Sudden death is the most frequent cause of late mortality in adults with
CHD
Sudden death is the most frequent cause of late mortality in adults with
CHDSarubbi B., Somerville J.: Sudden death in grown-up congenital heart (GUCH) patients: a 26-year population-based study. JACC 1999
Sarubbi B., Somerville J.: Sudden death in grown-up congenital heart (GUCH) patients: a 26-year population-based study. JACC 1999
Gatzoulis et al Lancet 2000Gatzoulis et al Lancet 2000
793 adult pts (1985-95)793 adult pts (1985-95)33 pts died (4.2% mortality)33 pts died (4.2% mortality)
Late Death in Repaired TetralogyLate Death in Repaired Tetralogy
CAUSES OF ADMISSION FOR GUCHCAUSES OF ADMISSION FOR GUCH
Report of the British Cardiac Society - Heart 2002;88:i1-i14
02040
6080
100120
140160180200
EPS/Cat.Card. Aritmie Scomp.Card. Emorragie Endocardite
GUCH Admission - Year 2010GUCH Admission - Year 2010
A.O. Monaldi Napoli
Percentage of Fallot admitted for arrhythmiasPercentage of Fallot admitted for arrhythmias
Clinical History ECG ParametersSAECG/LPEPSRV/LV Emodinamics, Volume, FunctionTissutal characterization Autonomic Nervous System
Adult Congenital Heart Disease PtsAdult Congenital Heart Disease PtsRisk stratification for S.D.Risk stratification for S.D.
Previous Surgical InterventionPrevious Palliative InterventionAge at operationType of Surgical ApproachFollow-up duration
Previous Surgical InterventionPrevious Palliative InterventionAge at operationType of Surgical ApproachFollow-up duration
Arrhythmias in GUCHRISK STRATIFICATIONArrhythmias in GUCHRISK STRATIFICATION
Clinical HistoryClinical History
TOF: Arrhythmic TOF: Arrhythmic RisckRisck
TOF: Arrhythmic TOF: Arrhythmic RisckRisck
“Scar related”VT
“Scar related”VT
•Ventriculotomy•Interventricular Patch•RVOT Patch
•Ventriculotomy•Interventricular Patch•RVOT Patch
SD Incidence between 0.5 to 5.5%SD Incidence between 0.5 to 5.5%
Presence of symptoms of Arrhythmia or Heart Failure
History of documented AFL/AF
Presence of symptoms of Arrhythmia or Heart Failure
History of documented AFL/AF The best
predictors of SCD
SVT SVT
Increased HRIncreased HR
Reduction in C.O.Reduction in C.O.
Reduction of the ventricle filling timeReduction of the ventricle filling time
NeurohormonalActivationNeurohormonalActivation
Heart FailureHeart Failure
Sistolic-diastolic dysfunctionSistolic-diastolic dysfunction
Arrhythmias, Heart Failure and SDin GUCH
Arrhythmias, Heart Failure and SDin GUCH
Clinical History ECG ParametersSAECG/LPEPSRV/LV Emodinamics, Volume, FunctionTissutal characterization Autonomic Nervous System
Adult Congenital Heart Disease PtsAdult Congenital Heart Disease PtsRisk stratification for S.D.Risk stratification for S.D.
sVT A.Flutter SD No Arrhy.
QRS 198.9(p<.0001)
177.8(p<.0001)
193.8(p=.01)
142.5
CTR 0.67(p<.01)
0.64(p<.002)
0.63(p<.04)
0.53
Gatzoulis M.A., et al: Mechano-electrical Interaction in Tetralogy of Fallot. Circulation 1995
Gatzoulis M.A., et al: Mechano-electrical Interaction in Tetralogy of Fallot. Circulation 1995
SD not related to width of QRS
SD not related to width of QRS
O= Repaired Fallot O= Unrepaired Fallot
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.
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.
Measurement of QRS is difficult
Can be operator dependent
Can be influenced by the presence of conduction abnormalities which reduce its accuracy and reproducibility.
Measurement of QRS is difficult
Can be operator dependent
Can be influenced by the presence of conduction abnormalities which reduce its accuracy and reproducibility.
0
0.5
1
1.5
2
2.5
33.5
4
4.5
VT
SD
AF
Arrhythmia-free
QR
S a
nn
ual
ch
an
ge,
ms/y
ear
Gatzoulis et al. Lancet 2000Gatzoulis et al. Lancet 2000
Clinical History ECG ParametersSAECG/LPEPSRV/LV Emodinamics, Volume, FunctionTissutal characterization Autonomic Nervous System
Adult Congenital Heart Disease PtsAdult Congenital Heart Disease PtsRisk stratification for S.D.Risk stratification for S.D.
Signal Average ECGSignal Average ECGHigh accuracy of Signal Average ECG
to predict severe VA
CONTROLS
ALL PTS
PTS WITH
MINOR ARRYTH
MIA
PTS WITH
SEVERE ARRYTH
MIA
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
*p<0.001 vs pts with minor and severe arrhythmias.#< 0.01vs pts with severe arrhythmias
Pts operated on for TOF :
X Y Z
Time domain
Frequency domain
J. Cardiovasc. Electrophysiol. 2005
J. Cardiovasc. Electrophysiol. 2005
Clinical History ECG ParametersSAECG/LPEPSRV/LV Emodinamics, Volume, FunctionTissutal characterization Autonomic Nervous System
Adult Congenital Heart Disease PtsAdult Congenital Heart Disease PtsRisk stratification for S.D.Risk stratification for S.D.
Khairy et al, Circulation 2004Khairy et al, Circulation 2004
EPS inducible sustained VT EPS inducible sustained VT VT or SCD VT or SCD
Alexander M.E, Walsh E.P.: J.Cardiovasc. Electr.
•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
•Very low positive predictive value (20%) of VSTIM to predict SCD
•Proarrhythmia of antiarrhythmic drugs
•Management of pts with spontaneous VT and non inducible arrhythmiasAlexander M.E, Walsh E.P.:
J.Cardiovasc. Electr.
Clinical History ECG ParametersSAECG/LPEPSRV/LV Emodinamics, Volume, FunctionTissutal characterization Autonomic Nervous System
Adult Congenital Heart Disease PtsAdult Congenital Heart Disease PtsRisk stratification for S.D.Risk stratification for S.D.
ATRIAL FLUTTER and RV FUNCTION after ATRIAL FLUTTER and RV FUNCTION after MUSTARDMUSTARD
Gelatt M J et al. JACC, Jen1997: 29 (1); 194-201
1 normal; 2 mild depression; 3 moderate depression; 4 severe depression.1 normal; 2 mild depression; 3 moderate depression; 4 severe depression.
0
20
40
60
80
100
VT
SD
AF
Arrhythmia-free
RVSP TR PR (>60mmHg) (> moderate) (> moderate)
% p
atie
nts
Gatzoulis et al. Lancet 2000Gatzoulis et al. Lancet 2000
Mod-SevereLV systolic
dysf.
Normal-MildLV 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%
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%
Davlouros et al JACC 2002Davlouros et al JACC 2002
Right and Left ventricular interactionRight and Left ventricular interactionAt rest (MRI)At rest (MRI)
Clinical History ECG ParametersSAECG/LPEPSRV/LV Emodinamics, Volume, FunctionTissutal characterization Autonomic Nervous System
Adult Congenital Heart Disease PtsAdult Congenital Heart Disease PtsRisk stratification for S.D.Risk stratification for S.D.
MYOCARDAL FIBROSIS AND LIFE MYOCARDAL FIBROSIS AND LIFE THREATENING VENTRICULAR THREATENING VENTRICULAR
ARRHYTHMIASARRHYTHMIAS
RVOT scar
3D Late Gad CMR 3D CMR EP Merge VT ablated at site RVOT scar
Clinical History ECG ParametersSAECG/LPEPSRV/LV Emodinamics, Volume, FunctionTissutal characterization Autonomic Nervous System
Adult Congenital Heart Disease PtsAdult Congenital Heart Disease PtsRisk stratification for S.D.Risk stratification for S.D.
Circulation 2002
Circulation 2002
ToF patients with VT have significant impairment of sympatho-vagal balance, characterized by a reduction of vagal drive
ToF patients with VT have significant impairment of sympatho-vagal balance, characterized by a reduction of vagal drive
Issues for the use of AICD in ACHDIssues for the use of AICD in ACHD
Indications
Inappropriate shocks and lead failure
Unique anatomical situations in CHD
Technical difficulties
Indications
Inappropriate shocks and lead failure
Unique anatomical situations in CHD
Technical difficulties
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
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
No data in the literature comparing medical therapy with AID implantation in either paediatric or adult CHD populationAttempt to ablate the VT focus either in the EP lab or in the operating room in ACHD before considering AID implantationLong 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 populationAttempt to ablate the VT focus either in the EP lab or in the operating room in ACHD before considering AID implantationLong term efficacy and safety of this approach in ACHD in unknown
International J. of Cardiology 2008International J. of Cardiology 2008
European Heart Journal 2006 European Heart Journal 2006
20 pts aged 16±6yrs 11 CHD6 Epicardial; 14 transvenousTherapy-rate 2.8 per patient-years of F-U53% appropriate; 47% inappropriate1.5 appropriate per patient-year of FU1.3 inappropriate per patient-year of FU
20 pts aged 16±6yrs 11 CHD6 Epicardial; 14 transvenousTherapy-rate 2.8 per patient-years of F-U53% appropriate; 47% inappropriate1.5 appropriate per patient-year of FU1.3 inappropriate per patient-year of FU
PACE 2004; 27:924-932PACE 2004; 27:924-932
PACE 2004; 27:924-932PACE 2004; 27:924-932
J. Cardiovasc. Electrophysiol.
15:72-76; 2004
J. Cardiovasc. Electrophysiol.
15:72-76; 2004
Epicardial lead malfunction is common
on long -term follow-up.
Some leads have a failure of 28% at 4yrs
Epicardial lead malfunction is common
on long -term follow-up.
Some leads have a failure of 28% at 4yrs
Do we really need so many risk
factors ?
Do we really need so many risk
factors ?
PACE 2004; 27:47-51PACE 2004; 27:47-51
Malignant arrhythmias occur even in patients with:
no residual lesion
no QRS prolongation
no ventricular dysfunction
Malignant arrhythmias occur even in patients with:
no residual lesion
no QRS prolongation
no ventricular dysfunctionThe recognition of those who would
benefit from an ICD remains a clinical challenge
The recognition of those who would benefit from an ICD remains a clinical
challenge
Yap S. et al.: Eur. Heart J. 2006 Yap S. et al.: Eur. Heart J. 2006
...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…)
...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…)
““Pediatric congenital cardiac becomes a Pediatric congenital cardiac becomes a
postoperative adult: the changing population of postoperative adult: the changing population of
congenital heart disease”congenital heart disease”Perloff JK. Circulation 1973; 47:606-619Perloff JK. Circulation 1973; 47:606-619
… we are obliged to look beyond the present and define our ultimate goal: the quality of long-term survival…
… we are obliged to look beyond the present and define our ultimate goal: the quality of long-term survival…