5 Χρόνια CHALLENGE: Παρόν και Μέλλον Γεώργιος Γιαννακούλας Επίκουρος Καθηγητής Καρδιολογίας ΑΠΘ
None Declaration of interest
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) 350 2005 2020 300 250 200 150 100 50 0 Years Kaemmerer H, et al. Dtsch Med Wochenschr 2005; 130:97-101.
van der Bom et al. Am Heart J 2012
Changing mortality in ACHD Khairy P, et al. J Am Coll Cardiol 2010
Marelli AJ et al. Circulation 2014
The number of adults with CHD exceeds the number of children by 2:1 Marelli AJ et al. Circulation 2014
60% of severe ACHD patients are adults! Marelli AJ et al. Circulation 2014
ACHD centers worldwide Webb G et al. Int J Cardiol. 2015
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
Coronary heart disease in ACHD 9.2%
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 (10-100-fold higher than the estimated in control population) Operations (20% in adulthood) or reoperations 1. Zomer AC et al. Int J Cardiol 2013 2. Opotowsky AR et al. J Am Coll Cardiol 2009 3. Mulder BJ. Eur Respir Rev 2010 4. Zomer et al, Circulation 2011 5. Avila P et al. Can J Cardiol. 2014 6. Hoffman A. et al. Heart 2010
Background Up to now there are no data on the epidemiology of CHD in Greece 13
CHALLENGE is funded by Hellenic Society of Cardiology
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
Methods and Materials Initiation of the registry in January 2012 Informed consent Patients>16 years old 18 expert ACHD centers
http://www.hcs.crf.gr/ Methods and Materials
Results
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%
0 500 1000 1500 2000 2500 5/2012 8/2012 11/2012 2/2013 5/2013 8/2013 11/2013 2/2014 5/2014 8/2014 11/2014 2/2015 5/2015 8/2015 11/2015 2/2016 5/2016 8/2016 11/2016 2/2017 0 500 1000 1500 2000 2500 5/2012 8/2012 11/2012 2/2013 5/2013 8/2013 11/2013 2/2014 5/2014 8/2014 11/2014 2/2015 5/2015 8/2015 11/2015 2/2016 5/2016 8/2016 11/2016 2/2017 Πορεία της καταγραφής N=2202
5/2012 8/2012 11/2012 2/2013 5/2013 8/2013 11/2013 2/2014 5/2014 8/2014 11/2014 2/2015 5/2015 8/2015 11/2015 2/2016 5/2016 8/2016 11/2016 2/2017 250 Πορεία της καταγραφής ανά τρίμηνο 200 150 100 50 0
Severity of ACHD
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
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±16.1 38.9±17.4 36.7±14.9 34.3±14.2 <0.001 Males 855 (47.2%) 360 (42.5%) 366 (52.2%) 127 (49.3%) 0.001 NYHA I/II 1665 (91.8%) 781(96.1%) 653 (93.2%) 196 (74.5%) <0.001 NrSurgeries 0 885 (48.8%) 625 (73.7%) 194 (27.6%) 66 (25.1%) <0.001 1 574 (31.7%) 190 (22.4%) 297 (42.3%) 87 (33.1%) 2 354 (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
Gender and ACHD
Epidemiology of adult congenital heart disease in Greece. Results from CHALLENGE registry.
Arrhythmias and ACHD
Epidemiology of adult congenital heart disease in Greece. Results from CHALLENGE registry.
Elderly and ACHD
Congenital heart diseases in the elderly: Results from CHALLENGE registry CHALLENGE team. ESC Congress 2016
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
Congenital heart diseases in the elderly: Results from CHALLENGE registry
Heart failure and ACHD
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
Heart failure in elderly patients with ASD
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
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 (1.04-1.16) <0.001 NS NS p Male gender 1.72 (0.85-3.48) 0.13 NS NS NYHA ΙΙΙ/ΙV 6.67 (2.47-18.01) <0.001 5.32 (1.86-15.23) 0.002 Antiarrhythmic drugs 3.84 (1.93-8.01) <0.001 3.36 (1.45-7.36) 0.004 ASD closure Open defect/surgical closure Percutaneous/ surgical closure 0.001 0.98 (0.34-2.45) 0.27 (0.08-0.90) 0.047 0.97 0.03
Anticoagulation and ACHD
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
Anticoagulation therapy in adult patients with congenital heart disease in Greece: Results from CHALLENGE registry 100 90 P<0.001 100 90 P<0.001 80 80 70 70 60 60 50 40 30 20 10 0 without anticoagulants on anticoagulants NYHA I/II NYHA III/IV 50 40 30 20 10 0 on anticoagulants without anticoagulants >60 ετών <60 ετών
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 1.034 (1.026-1.042) <0.001 1.036 (1.026-1.046) <0.001 Male gender 1.51 (1.17-1.95) 0.002 1.5 (1.11-2.01) 0.008 NYHA 4.97 (3.47-7.13) <0.001 2.06 (1.35-3,13) 0.001 Bethesda 1.77 (1.48-2.11) <0.001 NS NS Antiarrhythmic drugs 8.08 (6.03-10.83) <0.001 4.19 (3,04-5,79) <0.001 Surgeries (0,1, 2) 2.1 (1.79-2.48) <0.001 2.08 (1.68-2.58) <0.001
PAH and ACHD
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 8.57 1.11-66.42 0.04 Female gender 3.25 0.70-15.01 0.13 Eisenmenger 0.63 0.17-2.40 0.50 Age 1.03 0.99-1.07 0.21 Arrhythmia 1.84 0.56-6.05 0.31 HF hospitalization 5.04 1.53-16.59 0.008 CHALLENGE team. Submitted ESC Congress 2017
Conclusions (I) Severe ACHD patients: were younger more symptomatic underwent multiple surgeries were more frequently under medical therapy for heart failure and arrhythmias.
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
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.
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.
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.
Limitations Misclassification bias The entire data of the patients were not accessible The high proportion of expert centers might induce bias
Future perspectives->follow up
28000 ACHD patients wanted!!
More to come. More registered patients More participating centers More investigators More results to publish
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