ΣΥΝΔΡΟΜΟ ΜΑΚΡΟΥ QT Eπεµβατική και µη επεµβατική διαστρωµάτωση κινδύνου

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1 ΣΥΝΔΡΟΜΟ ΜΑΚΡΟΥ QT Eπεµβατική και µη επεµβατική διαστρωµάτωση κινδύνου Ελένη Χατζηνικολάου-Κοτσάκου Επεµβατική Καρδιολόγος-Ηλεκτροφυσιολόγος ΚΛΙΝΙΚΗ ΑΓΙΟΣ ΛΟΥΚΑ Σ

2 Σύνδροµο Long QT µια κληρονοµούµενη διαταραχή (καναλοπάθεια) που χαρακτηρίζεται από παράταση της κοιλιακής επαναπόλωσης στο ΗΚΓ φηµα και προδιαθέτει στην εµφάνιση συγκοπής και αιφνιδίου θανάτου µέσω πρόκλησης πολύµορφης κοιλιακής ταχυκαρδίας TdP

3 Modified Swartz criteria ( 1985) Σύνδροµο Long QT

4 Σύνδροµο Long QT

5 Σύνδροµο Long QT ΜΕΤΡΗΣΗ QT HKΓ-φηµα 12 απαγωγών, µε ταχύτητα 25 mm/s και 10 mm/mv amplitude στις απαγωγές ΙΙ, V5, V6 Μέση τιµή 3-5 συστολών

6 Σύνδροµο Long QT

7 βάσει αυτού η αποτελεσµατική θεραπευτική αντιµετώπιση και πρόληψη.. 1/

8 A patient symptomatic or asymptomatic, with or without family history with this ECG Is he or she in high risk for SCD????? II V2 V5

9 Σύνδροµο Long QT

10 Risk stratification by clinical parameters Ø Length of the QT/QTc interval on serial resting ECG Ø QTc Variability Ø Gender

11 Risk stratification by clinical parameters

12 Risk stratification by clinical parameters

13 Risk stratification by clinical parameters

14 Risk stratification by clinical parameters

15 Risk stratification by clinical parameters q The presence of overt T wave alternans, especially when evident despite proper therapy q Patients with syncope or cardiac arrest before age 7 q Patients who have syncope or cardiac arrest in the first year of life, may not fully protected by traditional therapies q Patients who suffer arrhythmic events despite being on full medical therapy

16 Risk stratification by genotype

17 Risk stratification by genotype Risk Stratification

18 Risk stratification by genotype

19 Risk stratification by genotype

20 Σύνδροµο Long QT ΗΚΓ-φικά Χαρακτηριστικά Συνδρόμου LQT

21 Which is the genotype of the patient with this ECG??? Ασυµπτωµατικός άρρεν 25 ετών µε ιστορικό οικογενούς ΑΘ Ποιός είναι ο γονότυπος του συνδρόµου; QTc 560 msec

22 Risk stratification by genotype

23 Risk stratification by genotype

24 Risk stratification by combined parameters

25 3 Risk stratification by genotype

26 Risk stratification by genotype

27 Identification and Genotype Prediction in Long-QT Syndrome 1}Postural change in QTc was obtained by taking the QTc difference between rest and standing ECGs. 2}Absolute QT shortening refers to the difference between the rest and peak exercise QT intervals (QTpeak QTrest). 3 }The QTc prolongation was calculated by taking the difference between the peak exercise and rest QTc intervals (QTcpeak QTcrest). 4}QT hysteresis was determined by calculating the QT interval difference between exercise and 2-minute into the recovery phase at similar heart rates (within 10 bpm). Patients with LQTS exhibited a greater prolongation in QTc on standing than LQTS-negative, P=0.029 During exercise, patients with LQT1 mutations had an attenuated QT shortening compared with LQTS-negative and LQT2 patients B, P< In addition, LQT1 patients had a marked QTc prolongation during exercise, whereas only a modest change in the QTc interval was observed in LQT2 and LQT-negative patients, P< Burst bike and gradual bike testing showed similar results, with LQT1 patients demonstrating significantly impaired shortening of their QT interval and pronounced QTc lengthening with exercise). In contrast, LQT2 patients had greater QT hysteresis than LQT1 and LQTS-negative patients, P< The area under the ROC curve for identifying LQT2 by hysteresis was (95% CI, to 0.930; P<0.0001), for LQT1 by peak exercise prolongation was (95% CI, to 0.896; P=0.0002), and for LQTS from postural QTc change was (95% CI, to 0.789; P=0.0095; In the patients with concealed LQTS (resting QTc 460 ms in men, 480 ms in women), postural QTc increase was >30 ms in 68%. Exercise QTc prolongation was >60 ms in 94% of concealed LQT1 patients. Hysteresis was >25 ms in 67% of concealed LQT2 patients. But the value of exercise test or adrenaline stimulation is still under control studies.. Jorg A Wong et al, Circulation:Arrhythmia and Electrophysiology, 2010;3:

28 Risk stratification by genotype

30 Risk stratification by genotype

31 Risk stratification by genotype

32 GENETIC SCREENING GENETIC SCREENING

33 RISK STRATIFICATION BY INVASIVE TESTS RISK STRATIFICATION THE ROLE OF INVASIVE TEST ELECTROPHYSIOLOGY STUDY

34 ELECTROPHYSIOLOGY STUDY v There is little role for diagnostic EP testing in LQTS. v Studies have shown v that programmed ventricular stimulation v QT interval response to pacing v and infusion of β-blocking medication in LQTS are of limited value v Monophasic action potential studies demonstrated afterdepolarizations but were not prognostic v and proarrhythmia can occur with ventricular stimulation.

35 RISK STRATIFICATION BY ALL PARAMETERS

36 Avoidance of strenous exercise especially swimming without supervison in LQT1 patients Reduction in exposure to abrupt loud noises in LQT2patients

37 Acquired LQT usually results from drug therapy. hypokalemia or hypomagnesemia ischemia and bradycardia can increase the risk of druginduced LQTS. Polymorphism of gene MiRP-1 (T8A),is present in 1.6% of general population and is associated with antibiotic bactrimel proarrhytmic effect

39 Messages for Home

40 THANK YOU FOR YOUR ATTENTION

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