Sudden Death in Adult Congenital Heart Disease (GUCH Patients) Sudden Death in Adult Congenital Heart Disease (GUCH Patients) Berardo Sarubbi U.O.C. di

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



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



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



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.



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)



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



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…

Documents

Sudden Death in Adult Congenital Heart Disease (GUCH Patients) Sudden Death in Adult Congenital Heart Disease (GUCH Patients) Berardo Sarubbi U.O.C. di