Κοιλιακές ταχυκαρδίες αιφνίδιος θάνατοςστιςσυγγενείς καρδιοπάθειες Νεώτερες εξελίξεις Ι. Παπαγιάννης Παιδοκαρδιολογική Κλινική ΜΗΤΕΡΑ
Κοιλιακή ταχυκαρδία/ Αιφνίδιος καρδιακός θάνατος Τετραλογία Fallot Αποφρακτικές παθήσεις αριστεράς κοιλίας Ανωμαλία Ebstein Συστηματική δεξιά κοιλία (D TGA s/p Mustard/Senning, L TGA) Μονήρης κοιλία
Αιφνίδιος θάνατος μετά από διόρθωση τετραλογίας Fallot Διάφορες μελέτες, συνολικά ~2.000 ασθενείς: 2% ανά 10ετία παρακολούθησης ΠΑΡΑΓΟΝΤΕΣ ΚΙΝ ΥΝΟΥ Μεγαλύτερη ηλικία εγχείρησης Μακρύτερο διάστημα παρακολούθησης Προηγούμενες παρηγορικές επεμβάσεις (shunt) Non sustained VT σε Holter Προκλητή VT σε EPS Κακή αιμοδυναμική κατάσταση Ευρύ QRS (>180 msec)
Transventricular vs transatrial repair
Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi institutional study 556 patients, 36.8+/ 12.0 yrs of age from 11 centers 43.3% had a sustained arrhythmia or arrhythmia intervention Atrial tachyarrhythmias were found in 20.1%. Ventricular arrhythmias were prevalent in 14.6% VAs were associated with number of cardiac surgeries, QRS duration, and left ventricular diastolic dysfunction Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age. Khairy P et al, Circulation. 2010;122(9):868 75
Sudden death in long term follow up of TOF Nollert et al, JACC 1997;30:1374 83
SUDDEN DEATH AND EPS Khairy et al, Circulation 2004;109:1994 2000
Κοιλιακές αρρυθμίες μετά από διόρθωση TOF: Αντιμετώπιση Χειρουργική αντιμετώπιση υπολειπόμενων αιμοδυναμικών ανωμαλιών ICD RF ablation Αντιαρρυθμικά φάρμακα
Κοιλιακή ταχυκαρδία μετά διόρθωση TOF etc: RF ablation ~10 case reports και 3 σειρές ( 50 ασθενείς) TOF, άλλες ΣΚ Άμεση επιτυχία ~90% Υποτροπή 20% Χωρίς σοβαρές επιπλοκές
Postoperative VT and RF ablation 11 patients with sustained VT after repair of congenital heart disease 4 isthmuses defined between (1) TV and RVOT patch, (2) PV and RVOT patch, (3) PV and VSD patch, and (4) VSD patch and TV Acutely successful ablation in all (11 of 15 VTs in isthmus #1) Long term freedom from VT recurrence: 91% Zeppenfeld K et al, Circulation 2007; 116(20):2241 52
Postoperative VT and RF ablation
Κοιλιακή ταχυκαρδία μετά από εγχείρηση σε τετραλογία Fallot
Κοιλιακή ταχυκαρδία και TOF Κατάλυση (RF ablation) Σε ασθενείς με άριστη αιμοδυναμική κατάσταση και καλά ανεκτή κοιλιακή ταχυκαρδία Σε ασθενείς με ICDs για να μειωθούν οι εκφορτίσεις Χρήση διαφόρων τεχνικών (entrainment mapping, electroanatomic mapping) για χαρτογράφηση και κατάλυση
SUDDEN DEATH AND TOF Effect of re operation 70 pts with severe PI, RVOTO and/or VT Pulmonary valve replacement with intraoperative cryo ablation reduced incidence of VT from 21% pre op to 9% postop (P<0.001) during a mean follow up of 4.7 yrs Therrien et al, Circulation 2001;103:2489 2494
Pulmonary valve replacement in tetralogy of Fallot: impact on survival and ventricular tachycardia. 98 patients with TOF and late PVR for RV dilation (median postop f/u 20 yrs). Matched control subjects for age, QRS duration, RV and LV size and function No significant change in QRS duration was seen for any group Overall 5 and 10 year freedom from death, VT or both was 80% and 41% No significant differences were seen in VT, death, or combined VT and/or death between groups A "window of opportunity" for intervention exists Harrild DM et al, Circulation. 2009 Jan 27;119(3):445 51
Survival and freedom from VT after TOF repair (pts with PVR vs controls)
SUDDEN DEATH AND CHD Use of ICD Use of implantable defibrillator has been demonstrated to be effective in several series of pts with CHD Indications: Resuscitated cardiac arrest Unstable VT Syncope of unexplained origin and inducible VT
ICDs in postoperative TOF 121 pts (median 33 yrs, 3,7 yrs F/U) 56% primary, 44% secondary prevention (sustained VT or resuscitated SCD) 30% at least 1 appropriate shock (actuarial annual rate of 8%) Predictors of appropriate shock in primary prevention: Higher LVEDP and non sustained VT Complications: 30% (including 5.8% per year inappropriate shocks) Mortality: 2.2% per year Khairy P et al, Circulation 2008;117:363 70
ICDs in postoperative TOF
ICDs in postoperative TOF
Postoperative specimen of TGA after the Mustard operation Warnes, C. A. Circulation 2006;114:2699-2709 Copyright 2006 American Heart Association
Transposition of the great arteries (D TGA) after Mustard or Senning baffles Sudden death is the most common cause of late mortality in this patient population. Incidence of 4.9 per 1000 patient years, second only to aortic stenosis and more than threefold greater than tetralogy of Fallot Although bradyarrhythmias were once thought to be primary triggers, pacemakers were not found to afford protection against sudden death
Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries. Retrospective, multicenter, case controlled study 47 patients after Mustard's or Senning's operation who experienced an SD event Symptoms of arrhythmia or CHF and history of documented A.Fib or A.Flutter were found to increase the risk of SD. Neither medication nor pacing was found to be protective. Most SD events (81%) occurred during exercise. VT/VF recorded during SD in 21 of 47 patients. Kammeraad et al, JACC;44(5):1095 102.
Sudden Death and Defibrillators in Transposition of the Great Arteries With Intra atrial Baffles 37 pts (age, 28.0±7.6 yr) from 7 centers ICDs implanted for primary prevention in 23 (62.1%) patients and secondary prevention in 14 patients (37.8%). Annual rates of appropriate shocks were 0.5% and 6.0% in primary and secondary prevention, respectively (P=0.0366). Independent predictors: secondary prevention indication (hazard ratio, 18.0; P=0.0341) and lack of β blockers (hazard ratio, 16.7; P=0.0301). Inducible VT during EP not a predictor Intracardiac electrograms documented SVT preceding or coexisting with VT in 50%. Inappropriate shocks occurred in 6.6% per year 14 patients (37.8%) experienced complications Khairy et al, Circ Arrhythmia Electrophysiol 2008; 1: 250 257
Appropriate ICD shocks (A) and survival (B) in primary and secondary prevention. Khairy P et al. Circ Arrhythm Electrophysiol 2008;1:250-257
Appropriate ICD shocks in patients with and without β-blockers Khairy P et al. Circ Arrhythm Electrophysiol 2008;1:250-257
Predictors of appropriate ICD discharge Variable Hazard Ratio 95% CI P Value Univariate analysis Secondary prevention indication 5.1 1.1, 45.5 0.0375 Ventricular septal defect At least moderate tricuspid regurgitation 4.3 0.9, 20.8 0.0742 4.1 0.8, 20.5 0.0912 QTc, ms 1.02 1.00, 1.05 0.0767 Lack of β blockers 11.3 1.3, 100.1 0.0303 Multivariate analysis Secondary prevention indication 18.0 1.2, 261.0 0.0341 Lack of β blockers 16.7 1.3, 185.2 0.0301
Sudden death after Fontan operation 261 patients, first Fontan at 7.9 years of age Over a median of 12.2 years, 76 (29.1%) died Actuarial freedom from death or transplantation was 93.7 and 82.6% at 5 and 20 years respectively with no significant difference between types of Fontan (AP, AV or TCPC) Late deaths were classified as sudden in 7 patients (9.2%), with no predictors identified Two patients with sudden death had previously documented intra atrial reentrant tachycardia Absence of aspirin or warfarin therapy shown to be a powerful independent predictor of mortality from thromboembolism Khairy et al, Circulation. 2008 Jan 1;117(1):85 92
Cumulative hazard by mode of death Khairy, P. et al. Circulation 2008;117:85-92 Copyright 2008 American Heart Association
Sudden death in other CHD Ebstein s anomaly Wolff Parkinson White syndrome Atrial tachycardia Ventricular tachycardia Aortic stenosis Related to degree of stenosis and usually of ischemic etiology Ventricular arrhythmias in neglected cases Treatment of underlying anatomic problems with tricuspid and aortic valve and ablation of accessory pathways largely prevents sudden cardiac death
Sudden unexpected death after balloon valvuloplasty for congenital aortic stenosis is very rare 528 patients who underwent BAVP for congenital AS at Children's Hospital Boston from 1984 to 2008 (6,344 pt yrs of follow up) 63 deaths, with SUD in 6 patients, 5 of which occurred at 18 months of age Exercise restricted pts (183) vs non restricted (220): 17 deaths in this cohort, with 1 SUD (the patient, who was exercise restricted, died during sleep), incidence of 0.18/1,000 patient years Brown et al, JACC 2010;56(23):1939 46
Συμπεράσματα Οι νεώτερες χειρουργικές τεχνικές έχουν μειώσει σημαντικά τον κίνδυνο ΚΤ ΑΚΘ στους ασθενείς με ΣΚ Οι μεγαλύτεροι σε ηλικία ασθενείς που χειρουργήθηκαν με παλαιότερες τεχνικές παρουσιάζουν ΚΤ και ΑΚΘ σε σημαντικά ποσοστά, ο οποίος αυξάνεται με τη διάρκεια παρακολούθησης Η σύγχρονη πρόληψη και αντιμετώπιση των αρρυθμιολογικών επιπλοκών περιλαμβάνει χειρουργική/επεμβατική βελτίωση αιμοδυναμικών προβλημάτων, κατάλυση με καθετήρα, εμφύτευση αντιαρρυθμικών συσκευών και περιορισμένη χρήση φαρμάκων