5 Χρόνια CHALLENGE: Παρόν και Μέλλον. Γεώργιος Γιαννακούλας Επίκουρος Καθηγητής Καρδιολογίας ΑΠΘ

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1 5 Χρόνια CHALLENGE: Παρόν και Μέλλον Γεώργιος Γιαννακούλας Επίκουρος Καθηγητής Καρδιολογίας ΑΠΘ

2 None Declaration of interest

3 Patients with CHD (000 s) Changing demographics of CHD Adults with CHD Children with CHD (90% of live births) 18 y.o. (80% of children with CHD) Live births with CHD (0.8% of live births) Years Kaemmerer H, et al. Dtsch Med Wochenschr 2005; 130:

4 van der Bom et al. Am Heart J 2012

5 Changing mortality in ACHD Khairy P, et al. J Am Coll Cardiol 2010

6 Marelli AJ et al. Circulation 2014

7 The number of adults with CHD exceeds the number of children by 2:1 Marelli AJ et al. Circulation 2014

8 60% of severe ACHD patients are adults! Marelli AJ et al. Circulation 2014

9 ACHD centers worldwide Webb G et al. Int J Cardiol. 2015

10 High mortality rates and a higher utilization of healthcare resources compared with younger patients. Acquired morbidities, such as coronary artery disease, seem to be key determinants of outcome in this older population in conjunction with the underlying congenital heart disease

11 Coronary heart disease in ACHD 9.2%

12 Lifelong complications Heart failure (20% of hospitalizations) Arrhythmias (atrial arrhythmias occur in 15% of ACHD patients) Pulmonary Hypertension (4% of CHD patients) Endocarditis (20-fold greater than in the general population) Thromboembolism ( fold higher than the estimated in control population) Operations (20% in adulthood) or reoperations 1. Zomer AC et al. Int J Cardiol Opotowsky AR et al. J Am Coll Cardiol Mulder BJ. Eur Respir Rev Zomer et al, Circulation Avila P et al. Can J Cardiol Hoffman A. et al. Heart 2010

13 Background Up to now there are no data on the epidemiology of CHD in Greece 13

14 CHALLENGE is funded by Hellenic Society of Cardiology

15 Purpose Estimation of the prevalence and long-term outcomes of congenital heart defects in Greece Development of an efficient organizational structure for the improvement of healthcare for patients with congenital heart disease

16 Methods and Materials Initiation of the registry in January 2012 Informed consent Patients>16 years old 18 expert ACHD centers

17 Methods and Materials

18 Results

20 ACHD centers Αχιλλοπούλειον Γ.Ν. Βόλου 1% Γ. Ν. Αθηνών Ιπποκράτειο 2% Γ.Ν. ΚΑΒΑΛΑΣ 2% Γ. Ν. 'Γεώργιος Παπανικολάου' 8% Γ. Ν. ΠΑΙΔΩΝ 'Π&Α Κυριακού' 1% Ιατρικό Διαβαλκανικό Θεσ/κης 2% ΩΝΑΣΕΙΟ Καρδιοχειρουργικό Κέντρο 48% Τζάνειο Νοσοκομείο Αθηνών 6% ΜΗΤΕΡΑ 5% Κλινική Άγιος Λουκάς 1% Νοσοκομείο Mediterraneo 2% Π.Γ.Ν. ΑΤΤΙΚΟΝ 6% Π.Γ.Ν.ΛAPIΣAΣ 1% Π Γ.Ν. Ρίου 0% Π.Γ.Ν. ΑΧΕΠΑ 15% Π.Γ.N.Ιωαννίνων 1%

21 /2012 8/ /2012 2/2013 5/2013 8/ /2013 2/2014 5/2014 8/ /2014 2/2015 5/2015 8/ /2015 2/2016 5/2016 8/ /2016 2/ /2012 8/ /2012 2/2013 5/2013 8/ /2013 2/2014 5/2014 8/ /2014 2/2015 5/2015 8/ /2015 2/2016 5/2016 8/ /2016 2/2017 Πορεία της καταγραφής N=2202

5/2012 8/2012 11/2012 2/2013 5/2013 8/2013

5/2015 8/2015 11/2015 2/2016 5/2016 8/2016

22 5/2012 8/ /2012 2/2013 5/2013 8/ /2013 2/2014 5/2014 8/ /2014 2/2015 5/2015 8/ /2015 2/2016 5/2016 8/ /2016 2/ Πορεία της καταγραφής ανά τρίμηνο

23 Severity of ACHD

24 Epidemiology of adult congenital heart disease in Greece. Results from CHALLENGE registry. Severity of ACHD in Greece 14% 47% mild moderate severe 39% Giannakoulas G, et al. Submitted

25 Epidemiology of adult congenital heart disease in Greece. Results from CHALLENGE registry. Overall Mild Moderate Severe p-value n=1813 n=848 n=702 n=263 Age 37.4± ± ± ±14.2 <0.001 Males 855 (47.2%) 360 (42.5%) 366 (52.2%) 127 (49.3%) NYHA I/II 1665 (91.8%) 781(96.1%) 653 (93.2%) 196 (74.5%) <0.001 NrSurgeries (48.8%) 625 (73.7%) 194 (27.6%) 66 (25.1%) < (31.7%) 190 (22.4%) 297 (42.3%) 87 (33.1%) (19.5%) 33 (3.9%) 211 (30.1%) 110 (41.8%) Cardiac Medication Use 726 (40.1%) 268 (31.6%) 290 (41.4%) 168 (63.9%) <0.001 Antiarrythmic drugs 282 (15.6%) 80 (9.4%) 129 (18.4%) 73 (27.8%) <0.001 Medication for HF 301 (16.6%) 93 (11.0%) 116 (16.5%) 92 (35.0%) <0.001 Anticoagulants 296 (16.3%) 112 (13.2%) 94 (13.4%) 90 (34.2%) <0.001 HF: Heart Failure; Nr: Number; NYHA: New York Heart Association

26 Gender and ACHD

27 Epidemiology of adult congenital heart disease in Greece. Results from CHALLENGE registry.

28 Arrhythmias and ACHD

29 Epidemiology of adult congenital heart disease in Greece. Results from CHALLENGE registry.

30 Elderly and ACHD

31 Congenital heart diseases in the elderly: Results from CHALLENGE registry CHALLENGE team. ESC Congress 2016

4% SV 4% AS 4% Ao Arch Abnormalities 7% PS 5%

TOF 15% ASD 33% VSD 5% AVSD 3% PDA 3% Ao arch

32 Congenital heart diseases in the elderly: Results from CHALLENGE registry PDA 3% AVSD 4% SV 4% AS 4% Ao Arch Abnormalities 7% PS 5% VSD 9% Other 9% AV/VA abnormal connections 7% TOF 15% ASD 33% VSD 5% AVSD 3% PDA 3% Ao arch abnorm 6% Other;12% TOF 7% ASD 64% The prevalence of ACHD subtypes in total population The prevalence of ACHD subtypes in elderly patients

33 Congenital heart diseases in the elderly: Results from CHALLENGE registry

34 Heart failure and ACHD

Pharmacotherapy for heart failure in adult patients with congenital heart disease: Initial results from CHALLENGE (Adult Congenital Heart Disease Registry.

35 Pharmacotherapy for heart failure in adult patients with congenital heart disease: Initial results from CHALLENGE (Adult Congenital Heart Disease Registry. A Registry from the Hellenic Cardiology Society) registry Atrial septal defect Ventricular septal defect Patent ductus arteriosus Aortic stenosis Atrioventricular septal defect Aortic arch abnormalities Tetralogy of Fallot Atrioventricular/vetriculoarterial Single ventricle Other 0% 10% 20% 30% 40% 50% 60% Giannakoulas et al. ESC Heart Failure 2016

36 Heart failure in elderly patients with ASD

37 Pharmacotherapy for heart failure in elderly patients with atrial septal defect HF pharmacotherapy 60% 40% 20% 0% 41.6% Under HF treatment 58.4% No HF treatment Ntiloudi et al., ESC Heart Failure 2017

38 Pharmacotherapy for heart failure in elderly patients with atrial septal defect: Results from CHALLENGE registry Univariate Analysis Multivariate Analysis Odds Ratio (95% confidence interval) P Odds Ratio (95% confidence interval) Age 1.10 ( ) <0.001 NS NS p Male gender 1.72 ( ) 0.13 NS NS NYHA ΙΙΙ/ΙV 6.67 ( ) < ( ) Antiarrhythmic drugs 3.84 ( ) < ( ) ASD closure Open defect/surgical closure Percutaneous/ surgical closure ( ) 0.27 ( )

39 Anticoagulation and ACHD

40 Anticoagulation therapy in adult patients with congenital heart disease in Greece: Results from CHALLENGE registry Anticoagulation therapy Gender differences in anticoagulation 16.4% 83.6% without anticoagulants on anticoagulants 44% 56% males females Giannakoulas et al., ALPIC 2016

41 Anticoagulation therapy in adult patients with congenital heart disease in Greece: Results from CHALLENGE registry P< P< without anticoagulants on anticoagulants NYHA I/II NYHA III/IV on anticoagulants without anticoagulants >60 ετών <60 ετών

42 Anticoagulation therapy in adult patients with congenital heart disease in Greece: Results from CHALLENGE registry Univariate Analysis Multivariate Analysis Odds Ratio (95% confidence interval) P Odds Ratio (95% confidence interval) p Age ( ) < ( ) <0.001 Male gender 1.51 ( ) ( ) NYHA 4.97 ( ) < (1.35-3,13) Bethesda 1.77 ( ) <0.001 NS NS Antiarrhythmic drugs 8.08 ( ) < (3,04-5,79) <0.001 Surgeries (0,1, 2) 2.1 ( ) < ( ) <0.001

43 PAH and ACHD

44 Risk stratification in PAH associated with congenital heart disease. Results from CHALLENGE registry Univariable predictors for cumulative mortality risk HR CI (95%) P value NYHA class III/IV Female gender Eisenmenger Age Arrhythmia HF hospitalization CHALLENGE team. Submitted ESC Congress 2017

45 Conclusions (I) Severe ACHD patients: were younger more symptomatic underwent multiple surgeries were more frequently under medical therapy for heart failure and arrhythmias.

46 Conclusions (II) CHD patients older than 60 years old : were mostly females had mild CHD were more symptomatic underwent fewer surgeries were more frequently under cardiac medical therapy

47 Conclusions (IIΙ) Almost 40% of elderly patients with ASD were under HF medical treatment. Predictors of administration of HF pharmacotherapy Advanced functional class History of arrhythmias were Percutaneous ASD closure was a preventive factor.

48 Conclusions (IV) The most common form of ACHD that required HF treatment was SV. Patients with SV, AV/ VA abnormal connections, TOF and AVSD were more frequently under treatment for arrhythmias.

49 Conclusions (V) One sixth of ACHD patients were under anticoagulation therapy. The administration of anticoagulants was determined by: history of arrhythmias Age male gender NYHA class and number of surgeries Whereas ACHD severity was not an independent predictor.

50 Limitations Misclassification bias The entire data of the patients were not accessible The high proportion of expert centers might induce bias

51 Future perspectives->follow up

52 28000 ACHD patients wanted!!

53 More to come. More registered patients More participating centers More investigators More results to publish

55 THE CHALLENGE TEAM Κέντρα ΣΚ: Σ. Ράμμος Γ. Γιαννακούλας Κ. Βασιλειάδης Α. Φρογουδάκη Α. Τζίφα Δ. Ντέλλος Σ. Μπρίλη Δ. Παρχαρίδου Α. Μαγγίνας Μ. Παπαφυλακτού Ν. Καμπουρίδης Α. Χαϊμαδή Α. Δούρας Γ. Παπαδόπουλος Π. Νταβλούρος Π. Λαθρίδου Α. Πίτσης Δ. Ντιλούδη Χ. Καρβούνης Συμβάλλοντες στην καταγραφή Σ. Φούσας Κ. Τσιούφης Γ. Παρχαρίδης Κ. Βανταλής Ι. Βλασερός Νέα κέντρα Α. Πατριανάκος Ι. Κανακάκης

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