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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 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

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Sudden Death in Adult Congenital Heart Disease (GUCH Patients)

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  1. Sudden Death in Adult Congenital Heart Disease (GUCH Patients) Berardo Sarubbi U.O.C. diCardiologia U.O. CardiopatieCongenitedell’Adulto SecondaUniversitàdegliStudidi Napoli - A.O. Monaldi

  2. Adults Congenital Heart Disease • 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

  3. “Pediatriccongenitalcardiacbecomes a postoperativeadult: the changingpopulationofcongenitalheartdisease”Perloff JK. Circulation1973; 47:606-619 …itissimple a matteroftimebefore a populationofadultwithcongenitalheartdiseasewould 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 2011 20 30 40 50 60 ASD/VSD TOF Mustard/Senning Fontan 2021 HLHS Truncus 20 30 40 50 60

  6. CLINICAL EVENTSAFTER SURGICAL CORRECTION:ventriculardysfunction, arrhythmias, re-intervention

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

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

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

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

  12. AdultCongenitalHeartDiseasePts Riskstratificationfor S.D. • Clinical History • ECG Parameters • SAECG/LP • EPS • RV/LV Emodinamics, Volume, Function • Tissutal characterization • Autonomic Nervous System

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

  14. TOF: Arrhythmic Risck SD Incidencebetween 0.5 to 5.5% “Scarrelated” VT • Ventriculotomy • Interventricular Patch • RVOT Patch

  15. Presence of symptoms of Arrhythmia or Heart Failure • History of documented AFL/AF The best predictors of SCD

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

  17. AdultCongenitalHeartDiseasePts Riskstratificationfor S.D. • Clinical History • ECG Parameters • SAECG/LP • EPS • RV/LV Emodinamics, Volume, Function • Tissutal characterization • Autonomic Nervous System

  18. GatzoulisM.A., et al: Mechano-electricalInteraction in TetralogyofFallot. Circulation 1995

  19. 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. O=Repaired FallotO= Unrepaired Fallot SD not related to width of QRS

  20. Measurement of QRS is difficult • Can be operator dependent • Can be influenced by the presence of conduction abnormalities which reduce its accuracy and reproducibility.

  21. Gatzoulis et al. Lancet 2000

  22. AdultCongenitalHeartDiseasePts Riskstratificationfor S.D. • Clinical History • ECG Parameters • SAECG/LP • EPS • RV/LV Emodinamics, Volume, Function • Tissutal characterization • Autonomic Nervous System

  23. SignalAverage ECG Time domain High accuracy of Signal Average ECG to predict severe VA Pts operated on for TOF : Frequency domain Y Z X *p<0.001 vs pts with minor and severe arrhythmias. #< 0.01vs pts with severe arrhythmias

  24. J. Cardiovasc. Electrophysiol. 2005

  25. AdultCongenitalHeartDiseasePts Riskstratificationfor S.D. • Clinical History • ECG Parameters • SAECG/LP • EPS • RV/LV Emodinamics, Volume, Function • Tissutal characterization • Autonomic Nervous System

  26. EPS inducible sustained VT  VT or SCD Khairy et al, Circulation 2004

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

  28. 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.

  29. AdultCongenitalHeartDiseasePts Riskstratificationfor S.D. • Clinical History • ECG Parameters • SAECG/LP • EPS • RV/LV Emodinamics, Volume, Function • Tissutal characterization • Autonomic Nervous System

  30. ATRIAL FLUTTER and RV FUNCTION after MUSTARD 1 normal; 2 milddepression; 3 moderate depression; 4 severe depression. Gelatt M J et al. JACC, Jen1997: 29 (1); 194-201

  31. Gatzoulis et al. Lancet 2000

  32. 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% Mod-Severe LV systolic dysf.

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

  34. AdultCongenitalHeartDiseasePts Riskstratificationfor S.D. • Clinical History • ECG Parameters • SAECG/LP • EPS • RV/LV Emodinamics, Volume, Function • Tissutal characterization • Autonomic Nervous System

  35. MYOCARDAL FIBROSIS AND LIFE THREATENING VENTRICULAR ARRHYTHMIAS

  36. 3D Late Gad CMR 3D CMR EP Merge VT ablated at site RVOT scar RVOT scar

  37. AdultCongenitalHeartDiseasePts Riskstratificationfor S.D. • Clinical History • ECG Parameters • SAECG/LP • EPS • RV/LV Emodinamics, Volume, Function • Tissutal characterization • Autonomic Nervous System

  38. Circulation 2002

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

  40. strategies to prevent SD in GUCH

  41. Issues for the use of AICD in ACHD • Indications • Inappropriate shocks and lead failure • Unique anatomical situations in CHD • Technical difficulties

  42. 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

  43. 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

  44. European Heart Journal 2006

  45. 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

  46. PACE 2004; 27:924-932

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