36ο Πανελλήνιο Καρδιολογικό Συνέδριο

Λήδα Πιερέττα Παπαβασιλείου M.D., Ph.DΔ .Θ.Κ.Α ΥΓΕΙΑ

Ηλεκτροφυσιολογία. Διαστρωμάτωση κινδύνου γιααιφνίδιο καρδιακό θάνατο σε ασθενείς που δενσυμπεριλαμβάνονται σε μεγάλες κλινικές μελέτες

Σε ασθενείς με συγγενείς καρδιοπάθειες

Congenital heart disease and risk stratification

2 populations

Children Adults

4 clinical status

Children Adults

Corrected

Non corrected

Corrected

Non corrected

Unlimited number of anatomical and physiopathological variants

PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease.

CHD incidence and SCD

• CHD:8/1000 live births• 800.000 adults with CHD in US in 2001 (45% mild, 40%

moderate, 15% severe), over 1.000.000 in 2014• SCD 15-26%• Heart failure 13-27%• Annual incidence 0.1%/y much higher than age

matched controls (100-fold higher than age-matchedcontrols)

• Most infants with CHD will survive adulthood rate willincrease

PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease.

Michael J. Silka • Yaniv Bar-Cohen. Pediatr Cardiol (2012) 33:452–460

Figure 3.2

PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease.

Intrinsic anatomy, Consequences of surgery

Canadian Journal of Cardiology 2014 30, e1-e63DOI: (10.1016/j.cjca.2014.09.002)PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease.

Factors leading to arrhythmia in CHD

Abnormal hemodynamicsRepeat surgeryDistorsion of cardiac anatomySinus node dysfunctionCellular injury (hypoxia, ECC, fibrosis, trauma)

Causes leading to SCD in CHD

Ventricular tachycardia IART with rapid AV conductionand hemodynamic failureor degeneration throughaccessory pathwaysVentriculotomy

VSD

Myopathy failing ventricle

Failing RV Failing LV Failing single ventricle

Specific pathologies at higher risk of SCDToFPost Mustard or Senning (TGA, systemic RV)ccTGA (systemic RV)LV obstruction (coartation, Ao stenosis,bicuspid Ao)Fontan (univentricular circulation)VentriculotomyVSDEpstein’anomaly

Koyak et al SCD in Adults With CHD. Circulation. 2012;126:1944-1954

Michael J. Silka • Yaniv Bar-Cohen. Pediatr Cardiol (2012) 33:452–460

Implications of implantable cardioverter defibrillator therapy in congenital heart disease and pediatrics.Alexander M. J. Cardiovasc. Eletrophysiol, Vol15, pp72-76, January 2004

ICD in children with CHD

Implications of implantable cardioverter defibrillator therapy in congenital heart disease and pediatrics.Alexander M. J. Cardiovasc. Eletrophysiol, Vol15, pp72-76, January 2004

ICD implantation in patients with CHDTechnical issues regarding access, venous occlusion, embolic events, residual defects,baffles, site of placement of leads and many many more…..

Tetralogy of Fallot .The never ending story

Most studied

15-30% Monomorphic VT 2-4% SCD

20-30% IART

Zeppenfeld et al VT Ablation in Congenital Heart Disease. Circulation. 2007;116:2241-2252

3 corridors of macroreentry

Conal septumVSD as obstacle

Rim between outflow track incisionPV (non transannular patch)

Right lateral patch marginTV annulus

Tetralogy of Fallot .The never ending story

Most studied

Risk Factor for SCD in ToFGarson 1991 RV systolic >60 mmHg RV diastolic pressure >8 mmHg

Gatzoulis 2000 QRS>180 ms QRS ratechange

Pulmonaryregurgitation

Age atrepair

Ghai 2002 LV dysfunction

Indicatorstudy

2013 RV mass/volume >0.3g/ml LVEF <55% MLVEF <54% F

Atrialtachycardia

Diller 2012 LV longitudinal strain RVEDA MAPSE

Bonello 2013 (CMR) RV outflow trackakinetic >30 mm

Reduced RV EF Age Age atrepair

Russo 2005 (SAECG) Longer filtered QRS

Chiu 2012 Increase in Microvolt T wave alternance (not superior to SAECG)

15-30% Monomorphic VT 2-4% SCD

20-30% IART

PES in ToF

Khairy 2004Sustained Monomorphic VT 30%

Sustained Polymorphic VT 4%

Khairy 2008: factors related to appropriate ICD discharge1. LV dysfunction2. Inducible or clinical sustained VT3. QRS >180 ms

Event free 89% in –PES in 15 yrs

Walsh. SCD in Adult CHD. HeartRhythm 2014;11:1735–1742

Risk factors after repair of tetralogy of Fallot

Particular cases

Eisenmenger

Polymorphic VT

Pulmonary regurgitation

ControindicatedPES andprobably ICD

Index of severe RV dysfunction

Even if corrected arrhythmic risk persists

Volume overload, hypertrophy, dyssynchrony, cyanosis, hypoxia, ischemia, scars

TGA

Higher risk of SCD compared with ToF 5-10%SCD is the leading cause of death after atrial baffle surgeryWilson 1998:SCD in Mustard- Senning 10%Koyak 2012: 10,5% in D-TGA (84% in Mustard-Senning 7% in ccTGA3-5% CAV at birth and 20% for ccTGA

S/P Mustard o SenningTill early 80’s

S/P arterial switchSince 1980

ccTGA

S/P Rastelli

Atrial bafflesextensive atrial scarSystemic RV

Systemic LV

Systemic RV

TGANo particular risk stratification available

Major risk factor1. IART with rapid AV conduction degenerating in VT2. Clinical HF3. Myopathic systemic RV4. QRS> 140 ms??

PES: not usefulAblation of IART in case of syncope or recurrent arrhythmia

Polymorphic VT due to myopathic RV

Pressure –volume overload Hypertrophy Hypoxia Ischemia Fibrosis

RV dilatation

Khairy P et al. Circulation EP 2008;1:250-257

Univentricular heart (systemic ventrical RV or LV)

Koyak 2012 : SCD 4%

Boston children Hospital: SCD 9% first 5 yrs then 10% HF at 10

yrs

IART 60% with hemodynamic deterioration

Late sinus node dysfunction leading to protein losing enteropathy

and plastic bronchitis

Fontan patients

LV outflow obstructionAo stenosisBicuspid ValveCoartation

SCD :5%

1993:SCD 15%

Koyak 2012 : SCD 12%

High degree of ventricular ectopy up to 34%

Reduction of events due to earlier and more effective intervention

(percutaneous treatments)

Ebstein’s anomaly

Koyak 2012 : SCD 3.5%

RBBB pattern after surgery

Accessory pathways

The atrialized portion of the ventricle is morphologically

and electrically RV but functionally RA. Mechanical

stimulation may induce ventricular arrhythmias

VT rare if not associated with LV dysfunction or other

CHD.

Septal defectsKoyak 2012 :

ASD: 4.6%

VSD: 4.6%

Endocardial cushions: 5.2%

Cardiac resynchronization therapy in congenital heartdisease

3 types of patients

Systemic LVCardiomyopathiesCongenital AV blockToF

Systemic RVMustard-SenningccTGA

Single ventricleFontan

CRT benefit inEF

QRS

NYHA

Exercise performance

Response rate 32-76%

Best results in single ventricle with multisite pacing

Best results in systemic left ventricles ( similar with CRT in adults)

Systemic RV best results with atrial synchronous single site RV pacing withoptimal manipulation of AV delay

Recommendations for ICD

Recommendations for CRT

Conclusions

Low incidence of SCD but 100-fold higher than age-matched population

SCD in CHD large psychological impact in a population that suffersfrom years of repeated surgery and hospitalization

Adult risk stratification guidelines does not suit this population

Most studied pathology Tetralogy of Fallot

Cardiac resyncronizzation therapy best results in systemic LV

Tailormade indications for prevention of sudden cardiac death

More at risk: ToF, Mustard, Senning, ccTGA, Fontan, Epstein,ventriculotomy

EPS most indicated in ToF with over risk factors, not concluding inTGA, contraindicated in Eisenmenger syndrome

Technical issues during implant due to complex anatomy, surgicalrepair, baffles or tunnels, vein obstruction ect

Recommendations PES

PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease.

PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease.