Adult Congenital Heart Disease 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

Incidence 8 per thousand In the last 20 years 90.000 pts with CHD 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

“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.

Percento 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 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 5

CLINICAL EVENTS AFTER SURGICAL CORRECTION: ventricular dysfunction, arrhythmias, re-intervention Atrial septal defect Pulmonary stenosis Anomalous pulmonary drenage 5% Partial AV Canal 10-15% Complete AV Canal 50% Aortic Valvulotomy Mustard Senning Fontan 100%

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

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

Late Death in Repaired Tetralogy 793 adult pts (1985-95) 33 pts died (4.2% mortality) Gatzoulis et al Lancet 2000

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

Percentage of Fallot admitted for arrhythmias GUCH Admission - Year 2010 Percentage of Fallot admitted for arrhythmias A.O. Monaldi Napoli Circa 1/3 dei pazienti ricoverati per aritmie presso la UOS di Cardiopatie Congenite dell’Adulto A.O. Monaldi nel 2005 sono Fallot.

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

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

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

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

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.

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

Gatzoulis M.A., et al: Mechano-electrical Interaction in Tetralogy of Fallot. Circulation 1995

SD not related to width of QRS 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 Fallot O= Unrepaired Fallot SD not related to width of QRS

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

Gatzoulis et al. Lancet 2000

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

Signal Average ECG High accuracy of Signal Average ECG Time domain High accuracy of Signal Average ECG to predict severe VA Pts operated on for TOF : CONTROLS ALL PTS PTS WITH MINOR ARRYTHMIA PTS WITH SEVERE ARRYTHMIA 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 Frequency domain X Y Z *p<0.001 vs pts with minor and severe arrhythmias. #< 0.01vs pts with severe arrhythmias

J. Cardiovasc. Electrophysiol. 2005

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

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

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.

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.

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

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

Gatzoulis et al. Lancet 2000

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.

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

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

MYOCARDAL FIBROSIS AND LIFE THREATENING VENTRICULAR ARRHYTHMIAS

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

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

Circulation 2002

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

strategies to prevent SD in GUCH

Issues for the use of AICD in ACHD 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

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

European Heart Journal 2006

6 Epicardial; 14 transvenous Therapy-rate 2.8 per patient-years of F-U 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

PACE 2004; 27:924-932

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

Do we really need so many risk factors ?

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 PACE 2004; 27:47-51

...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…) Yap S. et al.: Eur. Heart J. 2006

“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…