Changing Profile of Adult Congenital Heart Disease

Transcription

1 Congenital Heart Disease New Developments for the General Cardiologist Changing Profile of Adult Congenital Heart Disease European Society of Cardiology August 27, 2012 Ariane Marelli MD, FRCP, FACC, MPH Associate Professor of Medicine, McGill University Director, McGill Adult Unit for Congenital Heart Disease

2 Disclosures I received peer reviewed funding from the Canadian Institute of Health Research, the Heart and Stroke Foundation of Canada and the Fonds de Recherches en Sante du Quebec

3 Outline 1. Introduction: definitions; classification; recommendations for care 2. Special needs: arrhythmia; risk factors CAD; co-morbidity; changing interventional profile; prevention of complications

4 Spectrum of Disease GUCH Euro Heart Survey 4,110 patients Engelfriet P et al, Euro Heart Journal 2005;26:2325

5 Classification of GUCH disease Simple Moderate Complexity Great Complexity Isolated valve Tetralogy of Fallot Cyanotic CHD Small shunts Coarctation Single ventricle (all variants) Repaired shunts (no complications) RVOT osbtruction or regurgitation (significant) Atrioventricular Septal Defects Ebstein s Non-isolated VSD Open shunts Simplified Bethesda Classification Warnes C et al, 2008: 118; e721 Valve atresia (all variants) Transposition Conduit repair Pulmonary Arterial HTN

6 Increase in Severe CHD Adults and Children Marelli AJ et al, Circulation 2007; 115: 163

7 Changing landscape Patients with mild or moderate disease constitute the majority of patients followed even in specialized centers But the patient population which is growing at the fastest rate are the ones with severe disease which is going to alter what we see

8 ESC Guidelines Who should look after GUCH patients? Severe Disease GUCH care Moderate Disease Shared care Mild Disease Non GUCH centre care

9 Recommendations for Care General Cardiology General Cardiology GUCH Center ESC/AHA/ACC/CCS Guidelines

10 Increasing Numbers Equalization of Adults and Children Severe CHD 1985 to 2000 Marelli AJ et al, Circulation 2007; 115: 163

11 Health Services Utilization Hospitalization rates in Adults 22,096 adults compared to the general population Mackie A.S et al, AJC 2007; 99:839

12 Changing landscape As a community, our goal is to keep up with the changing profile of the CHD population, to synchronize the care we deliver in order to ultimately improve its quality and make it more cost-effective

13 Complications 4,110 patients Engelfriet P et al, Euro Heart Journal 2005;26:2325

14 Atrial Arrhythmia Incidence of Atrial Arrhythmia in 44, 248 adult pts Bouchardy J. et al Circulation 2009;120:1679

15 Aging Hearts Incidence of Atrial Arrhythmia in 44, 248 adult pts Bouchardy J. et al Circulation 2009;120:1679

16 Right-sided lesions Bernier et al Am J Cardiol 2010;106:547

17 Atrial Fibrillation in Atrial Septal Defect Bouchardy et al, Int J Cardiol Feb 26 th 2012

18 Pulmonary Arterial Hypertension At risk 5970 CHD patients PH % PH 2389 CHD patients at risk 248 PH patients 10% of pts at risk or 4.2% of total Prevalence of PAH Dutch Registry Duffels et al, Int J Cardiol. 2007;120:198

19 Pulmonary Arterial Hypertension Prevalence of PH in ACHD CONCOR Vader Velde ET et al, European Journal Epidemiology 2005;20:549

20 All forms of Pulmonary Hypertension Kaplan-Meier Survival 5.8% of 38,430 ACHD patients Lowe B et al, JACC 2011; 58:538

21 All forms of Pulmonary Hypertension Lowe B et al, JACC 2011; 58:538

22 Co-Morbidity 9,952 CHD patients primary care database in UK Billett J et al, Heart 2008;94:1194

23 CAD and Coarctation Roifman et al, Circulation. 2012;126:16

24 CAD and Coarctation 756 patients with CoA and 6481 patients with VSD Roifman et al, Circulation. 2012;126:16

25 Mortality 8,123 deaths over 1,008, 835 patient-years Khairy P et al, J ACC 2010; 56:1149

26 Comorbidity Geriatric ACHD cohort of 3,239 patients Afilalo J et al, JACC 2011; 58:1509

27 Comorbidity Afilalo J et al, JACC 2011; 58:1509

28 Changing Interventional Profile 474 operations in GUCH patients Srinathan, S K et al. Heart 2005;91:

29 Valvular interventions 17,444 cardiac operations in 71,979 patients for 1,009,430 person-years Ionescu-Ittu R et al Ann Thorac Surg 2010; 90:1563 9

30 Complication Prevention 4,110 patients Median age 27 years Engelfriet P et al, Euro Heart Journal 2005;26:2325

31 Complication Prevention Overall cumulative incidence of IE 5.8/1,000 children 37,598 children with CHD were followed from birth to age 18 Rushani D et al JACC 2012, 59(Suppl A): A199

32 Complication Prevention Rushani D et al JACC 2012, 59(Suppl A): A199

33 Summary Most of what we see is moderate or mild disease Most cases should be referred to a GUCH centre at least once Atrial arrhythmias are common and have a different natural history in CHD compared to non CHD patients Pulmonary HTN is becoming increasingly interesting as a marker of poor outcome not only in those with PAH Risk factor control for CAD is very important especially in the young as shown in patients with CoA There are growing interventions especially for moderate disease Prevention of IE remains cornerstone and unsettled