Le Cardiopatie Congenite nell’Adolescente e nell’Adulto LE ... · U.O.S. Cardiopatie Congenite dell’Adulto Seconda Università degli Studi di Napoli - A.O. Monaldi Berardo Sarubbi

Le Cardiopatie Congenite

nell’Adolescente e nell’Adulto

LE ARITMIE

Le Cardiopatie Congenite

nell’Adolescente e nell’Adulto

LE ARITMIE

Berardo SarubbiU.O.C. di Cardiologia

U.O.S. Cardiopatie Congenite dell’Adulto

Seconda Università degli Studi di Napoli - A.O. Monaldi

Berardo SarubbiU.O.C. di Cardiologia

U.O.S. Cardiopatie Congenite dell’Adulto

Seconda Università degli Studi di Napoli - A.O. Monaldi

Sudden 26%

Perioperative 18%

Non-cardiac17%

CHF21%

Other CVS18%

Oechsling et al Am J Cardiol 2000

Cause di Morte nei GUCHCause di Morte nei GUCH

Gatzoulis et al Lancet 2000Gatzoulis et al Lancet 2000

793 adult pts (1985793 adult pts (1985--95)95)

33 pts died (4.2% mortality)33 pts died (4.2% mortality)

Late Death in Repaired TetralogyLate Death in Repaired Tetralogy

CAUSE DI RICOVERO DICAUSE DI RICOVERO DI

CARDIOPATICI CONGENITI ADULTICARDIOPATICI CONGENITI ADULTI

Report of the British Cardiac Society - Heart 2002;88:i1-i14

020406080

100120140160180200

EPS/Cat.Card. Aritmie Scomp.Card. Emorragie Endocardite

Ricoveri “GUCH” Anno 2009Ricoveri “GUCH” Anno 2009

A.O. Monaldi Napoli

Percentuale di Fallot ricoverati per aritmiePercentuale di Fallot ricoverati per aritmie

EVENTI CLINICI MAGGIORIEVENTI CLINICI MAGGIORIDOPO INTERVENTO:DOPO INTERVENTO:

disfunzione ventricolare, aritmie, reinterventodisfunzione ventricolare, aritmie, reintervento

Comunicazione interatrialeComunicazione interatrialeStenosi polmonare Stenosi polmonare Drenaggio venoso polmonareDrenaggio venoso polmonare

5%5%

Canale parzialeCanale parziale 1010--15%15%

Canale CompletoCanale Completo 50%50%

Valvulotomia aorticaValvulotomia aorticaMustardMustardSenningSenningFontanFontanFallotFallot

100%100%

Modifiche della composizione della popolazione GUCHModifiche della composizione della popolazione GUCH

ASD/VSD

TOF

Mustard/Senning

Fontan

HLHS

Truncus

20 30 40 50 60

2010

2020

20 30 40 50 60

ASD/VSD

TOF

Mustard/Senning

Fontan

HLHS

Truncus

““Pediatric congenital cardiac becomes a postoperative adult: Pediatric congenital cardiac becomes a postoperative adult: the changing population of congenital heart diseasethe changing population of congenital heart disease””

Perloff JK. Perloff JK. Circulation Circulation 1973; 47:6061973; 47:606--619619

……it is simple a matter of time it is simple a matter of time before a population of adult with before a population of adult with congenital heart disease would congenital heart disease would emerge.emerge.

�Storia Clinica

�Parametri ECG Standard

�SAECG/LP

�SEE

�VD/VS Emodinamica, Volume, Funzione

�Caratterizzazione “tissutale”

�Storia Clinica


�SAECG/LP

�SEE



Cardiopatici Congeniti AdultiCardiopatici Congeniti AdultiStratificazione del Rischio AritmicoStratificazione del Rischio Aritmico

““ The essence of The essence of wisdom is the ability wisdom is the ability to make the right to make the right decision on the basis decision on the basis of inadeguate of inadeguate evidenceevidence””

Alan Gregg

�Precedenti Interventi CCH�Precedenti Interventi Palliativi�Età all’intervento�Tecnica/Approccio Chirurgico�Durata Follow-up

�Precedenti Interventi CCH�Precedenti Interventi Palliativi�Età all’intervento�Tecnica/Approccio Chirurgico�Durata Follow-up

Arrhythmias in GUCHRISK STRATIFICATION

Arrhythmias in GUCHRISK STRATIFICATION

Storia ClinicaStoria Clinica

TOF: Rischio AritmicoTOF: Rischio AritmicoTOF: Rischio Aritmico

TV da rientro“Scar related”

TV da rientro“Scar related”

•Ventricolotomia•Patch Interventricolare•Patch RVOT

•Ventricolotomia•Patch Interventricolare•Patch RVOT

Incidenza di SD da 0.5 a 5.5%Incidenza di SD da 0.5 a 5.5%

Tachicardie in CHD Tachicardie in CHD --Forme incisionaliForme incisionali

Fontan operation (cavopulmonary connection): Electroanatomic map of an IART circuit involving the anterolateral surface of the right atrium.

•• Fontan: 60%Fontan: 60%

•• Mustard/Senning: 25%Mustard/Senning: 25%

•• TOF: 25%TOF: 25%

•• CAV: 15%CAV: 15%

•• DIA: 10%DIA: 10%

Walsh EP et al. Circulation. 2007;115:534Walsh EP et al. Circulation. 2007;115:534--545545

�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

�Storia Clinica


�SAECG/LP

�SEE



�Storia Clinica


�SAECG/LP

�SEE




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 QRSSD 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.

00.5

11.5

22.5

33.5

44.5

VTSDAFArrhythmia-free

QR

S a

nnua

l cha

nge,

ms/

year

Gatzoulis et al. Lancet 2000Gatzoulis et al. Lancet 2000

�Storia Clinica


�SAECG/LP

�SEE



�Storia Clinica


�SAECG/LP

�SEE




Signal Average ECGSignal Average ECG

High accuracy of Signal Average ECGto predict severe VA

CONTROLS

ALL PTSPTS

WITH MINOR

ARRYTHMIA

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 (µµµµV)

26 ± 8 41 ± 32 45.3 ± 34.6 26 ± 16

*p<0.001 vs pts with minor and severearrhythmias.

#< 0.01vs pts with severe arrhythmias

Pts operated on for TOF :

X Y Z

Time domain

Frequency domain

J. Cardiovasc. Electrophysiol. 2005J. Cardiovasc. Electrophysiol. 2005

�Storia Clinica


�SAECG/LP

�SEE



�Storia Clinica


�SAECG/LP

�SEE




Khairy et al, Circulation 2Khairy et al, Circulation 2004004

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 arrhythmias

Alexander M.E, Walsh E.P.: J.Cardiovasc. Electr.

�Storia Clinica


�SAECG/LP

�SEE



�Storia Clinica


�SAECG/LP

�SEE




ATRIAL FLUTTER and RV FUNCTION after MUSTARDATRIAL FLUTTER and RV FUNCTION after MUSTARD

Gelatt M J et al. JACC, Jen1997: 29 (1); 194-201

1 normal; 2 mild depression; 3 moderate depression; 4 severe dep1 normal; 2 mild depression; 3 moderate depression; 4 severe depression.ression.

0

20

40

60

80

100

VTSDAFArrhythmia-free

RVSP TR PR (>60mmHg) (> moderate) (> moderate)

% p

atie

nts

Gatzoulis et al. Lancet 2000Gatzoulis et al. Lancet 2000

Tachicardie in CHD Tachicardie in CHD --Stretch elettromeccanico: TOFStretch elettromeccanico: TOF

Gatzoulis MA et al. Circulation 1995;92:231Gatzoulis MA et al. Circulation 1995;92:231--77

RV overloadRV overloadRV overload

RV systolic wall stressRV systolic wall stressRV systolic wall stress

Prolonged QRSProlonged QRSProlonged QRS

Ventricular arrhythmiasVentricular arrhythmiasVentricular arrhythmias

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%

�Storia Clinica


�SAECG/LP

�SEE



�Storia Clinica


�SAECG/LP

�SEE




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 RV OT scar

Come prevenire e/o trattare le aritmie nei GUCH ?

Come prevenire e/o trattare le aritmie nei GUCH ?

Abbiamo veramente

necessità di tanti

fattori di rischio ?

Abbiamo veramente

necessità di tanti

fattori di rischio ?

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 dysfunction

The 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

European Heart Journal 2006 European Heart Journal 2006

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…)

“ …Se si guarda troppo fisso una stella , si

perde di vista il firmamento… “

““ ……Se si guarda troppo Se si guarda troppo fisso una stella , si fisso una stella , si

perde di vista il perde di vista il firmamentofirmamento…… ““

E.A. PoeE.A. Poe

Issues for the use

of AICD in ACHD

Issues 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

�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

�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; 27:924-932PACE 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

�Subcutaneous array placed around the thorax

�Transvenous design ICD lead placed on the epicardium

�Transvenous design ICD lead placed in a subcutaneous position

�Subcutaneous array placed around the thorax

�Transvenous design ICD lead placed on the epicardium

�Transvenous design ICD lead placed in a subcutaneous position

J. Cardiovasc. Electrophysiol.17:41-46; 2006

J. Cardiovasc. Electrophysiol.17:41-46; 2006

PACE 2004; 27:924-932PACE 2004; 27:924-932

Take-home messageTakeTake--home messagehome message

•• Le tachicardie sono la Le tachicardie sono la causa principale di morbilitcausa principale di morbilitàà e mortalite mortalitàànella popolazione GUCHnella popolazione GUCH

•• Sono la principale causa di Sono la principale causa di ospedalizzaioneospedalizzaione

•• La La terapia farmacologicaterapia farmacologica spesso spesso èè poco efficacepoco efficace

•• Esistono Esistono difficoltdifficoltàà anatomicheanatomiche alla alla terapia nonterapia non--farmacologicafarmacologica

•• Le aritmie possono essere correlate alle Le aritmie possono essere correlate alle specifiche CHDspecifiche CHD, al , al peggioramento emodinamicopeggioramento emodinamico, alla , alla correzione chirurgicacorrezione chirurgica

•• EE’’ necessario che i cardiologi che lavorano nelle GUCH Unit necessario che i cardiologi che lavorano nelle GUCH Unit abbiano unabbiano un’’adeguata esperienza aritmologica (diagnosi, terapia adeguata esperienza aritmologica (diagnosi, terapia farmacologica, terpia nonfarmacologica, terpia non--farmacologica, problematiche correlate farmacologica, problematiche correlate alle cardiopatie congenite)alle cardiopatie congenite)

Aritmie e CHD:Aritmie e CHD:quali aritmie in quali CHDquali aritmie in quali CHD

Walsh EP et al. Circulation. 2007;115:534Walsh EP et al. Circulation. 2007;115:534--545545

Bradicardie in CHD: Fontan Bradicardie in CHD: Fontan

•• BAV III: 10%BAV III: 10%

•• Disfunzione SA: 50%Disfunzione SA: 50%

Meccanismi causa di aritmia in CHDMeccanismi causa di aritmia in CHD

•• tipici di alcune cardiopatie congenite tipici di alcune cardiopatie congenite •• conseguenza dello stress emodinamico o conseguenza dello stress emodinamico o ipossico ipossico •• secondari a sequele chirurgiche secondari a sequele chirurgiche

Bradicardie in CHD: DIABradicardie in CHD: DIA

Disfunzione del nodo Disfunzione del nodo del seno per del seno per incannulazione vena incannulazione vena cava superiore.cava superiore.

Bradicardie in CHD: DIABradicardie in CHD: DIA

TRNSTRNS

4700 msec4700 msec

P.M. anni 19.P.M. anni 19.

Difetto interatriale tipo Difetto interatriale tipo

seno venoso s/p seno venoso s/p

correzione radicalecorrezione radicale. .

Studio elettrofisiologico Studio elettrofisiologico

endocavitario. endocavitario. Disfunzione Disfunzione

del nodo del seno.del nodo del seno. TRNS: TRNS:

tempo di recupero del tempo di recupero del

nodo del senonodo del seno

Istologia: frammentazione del Istologia: frammentazione del tessuto di conduzione in pz con tessuto di conduzione in pz con

BAV post operatorio (chiusura BAV post operatorio (chiusura DIV con patch).DIV con patch).

NAV e fascio di His in rapporto NAV e fascio di His in rapporto

a patch posto a chiusura di a patch posto a chiusura di

DIV perimembranoso DIV perimembranoso sottoaortico.sottoaortico.

Bradicardie in CHD: DIVBradicardie in CHD: DIV

Bradicardie in CHDBradicardie in CHD

V.A. anni 16. V.A. anni 16.

Difetto interventricolare Difetto interventricolare

s/p correzione radicales/p correzione radicale. .

Studio elettrofisiologico Studio elettrofisiologico

endocavitario: endocavitario: Blocco Blocco

AtrioAtrio--ventricolare ventricolare

sottohissianosottohissiano. .

Bradicardie in CHD: MustardBradicardie in CHD: Mustard

••Disfunzione del nodo senoDisfunzione del nodo seno--atrialeatriale

••Distruzione chirurgica di tessuto atrialeDistruzione chirurgica di tessuto atriale

••Aree di ritardata attivazione atrialeAree di ritardata attivazione atriale

••Dispersione della refrattarietDispersione della refrattarietàà atrialeatriale

TGA s/p Mustard TGA s/p Mustard

operationoperation

Bradicardie in CHD: MustardBradicardie in CHD: Mustard

•• BAV IIIBAV III

•• Blocco SABlocco SA

Documents

Le Cardiopatie Congenite nell’Adolescente e nell’Adulto LE ... · U.O.S. Cardiopatie Congenite dell’Adulto Seconda Università degli Studi di Napoli - A.O. Monaldi Berardo Sarubbi