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

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Presentation on theme: "Sudden Death in Adult Congenital Heart Disease (GUCH Patients) Sudden Death in Adult Congenital Heart Disease (GUCH Patients) Berardo Sarubbi U.O.C. di."— Presentation transcript:

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

2 Adults Congenital Heart Disease Italy: 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 yrs 70.000 pts with CHD aged <18 yrs 100.000 pts with CHD aged >18 yrs 70.000 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; 47:606-619 …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;115:163-172.

5 Changes of GUCH population over the time ASD/VSD TOF Mustard/Senning Fontan HLHS Truncus 2030405060 2011 2021 2030405060 ASD/VSD TOF Mustard/Senning Fontan HLHS Truncus

6 CLINICAL EVENTS AFTER SURGICAL CORRECTION: ventricular dysfunction, arrhythmias, re-intervention Atrial septal defect Atrial septal defect Pulmonary stenosis Pulmonary stenosis Anomalous pulmonary drenage Anomalous pulmonary drenage5% Partial AV Canal Partial AV Canal10-15% Complete AV Canal Complete AV Canal50% Aortic Valvulotomy Aortic Valvulotomy Mustard Mustard Senning Senning Fontan Fontan100%

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

8 Event GUCH Sudden Death Arrhythmic 33 (35.1%) Arrhythmic 33 (35.1%) Haemorrhagic 17 (18.1%) Haemorrhagic 17 (18.1%) Other 7 (7.4%) Unknown 37 (39.4%) Unknown 37 (39.4%) 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 Gatzoulis et al Lancet 2000 793 adult pts (1985-95) 33 pts died (4.2% mortality) Late Death in Repaired Tetralogy

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

11 GUCH Admission - Year 2010 A.O. Monaldi Napoli Percentage of Fallot admitted for arrhythmias

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

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

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

17 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

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

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

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21 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

22 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.

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. 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

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

27 Signal Average ECG Signal Average ECG High accuracy of Signal Average ECG to predict severe VA CONTR OLS ALL PTS PTS WITH MINOR ARRYTH MIA PTS WITH SEVERE ARRYTH MIA QRS 40 (ms) 125 4 *162 29156 29 #181.5 19.6 LAS 40 (ms) 33.6 13.4 32 2228.5 19.8 § 45.1 26.7 RMS 40 ( V) 26 841 3245.3 34.626 16 *p<0.001 vs pts with minor and severe arrhythmias. #< 0.01vs pts with severe arrhythmias Pts operated on for TOF : X YZ Time domain Frequency domain

28 J. Cardiovasc. Electrophysiol. 2005

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

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

31 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

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

34 ATRIAL 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 depression.

35 Gatzoulis et al. Lancet 2000

36 Mod-Severe LV systolic dysf. 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%

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

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

39 MYOCARDAL FIBROSIS AND LIFE THREATENING VENTRICULAR ARRHYTHMIAS

40 RVOT scar 3D Late Gad CMR3D CMR EP MergeVT ablated at site RVOT scar

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

42 Circulation 2002

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

44

45 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

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 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 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 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; 27:924-932

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

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56 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…)

57 Pediatric congenital cardiac becomes a postoperative adult: the changing population of congenital heart disease Perloff 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…


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