1. Marc G Cribbs, MD Director, Alabama Adult Congenital Heart Program University of Alabama at Birmingham Children’s of Alabama Alabama Adult Congenital Heart Program

2.  0.4—1.2% of all live births  ~6,000,000 recognized pregnancies/year  600,000 neonates with CHD  CHD:  #1 congenital malformation  Blamed in up to 20% of miscarriages Epidemiology of Congenital Heart Disease (CHD)

3. 1980 – 1970 – 1960 – 1940 – 0 10 20 30 40 Percent Survival to 18 Years Old Decade Born with CHD 50 60 70 80 90 100 20 Warnes CA, et al. J Am Coll Cardiol. 2001;37(5):1170-1175. 1990 Moons P et al. Circulation 2010.122:2264-2272 Survival to 18 years of age with CHD % Survival

4. Incidence of CHD Expanding Population of Adolescents & Adults with CHD Early Complete Repair Improved Surgical Techniques Advances in NICU Care Increased Early Survival Lower Perioperative Mortality Increased Mid Term Survival Fetal Diagnosis

5. 1980 – 1970 – 1960 – 1940 – 0 10 20 30 40 Percent Survival to 18 Years Old Decade Born with CHD 50 60 70 80 90 100 20 Warnes CA, et al. J Am Coll Cardiol. 2001;37(5):1170-1175. 1990 Moons P et al. Circulation 2010.122:2264-2272 40 80 75 90 Survival to 18 years of age with CHD % Survival

6. Survival is expected – from the crib to old age

7. William, RG, et al. JACC. 2006; 47(4): 701-707 PEDIATRIC ADULT 70% 30% 50% 40% 60% 1965 2000 2010 20,000 NEW ACHD pts/year Percentage of Adults with Congenital Heart Disease

8. Distribution of ACHD disease Van der Velde et al. Euro. J. Epid. 2005 The Netherlands

9. ACHD: patient clinic visits 1700 1600 1500 1400 – – 1300 – – 1200 – – 1100 – – 1000 – – 900 – – 800 – – 700 – – 600 – – 500 – – 400 – – 300 – – 200 – – 100 – – 0 – 20022000200120032004200520062007 2008 Number of patients 1800 1900 2000 2100 2200 20092010 2300 2400 2500 346 128 210 460 630 910 1518 1780 2114 2310 2525 COACH Program 2012: ~3,000 patients

10. …60% of patients are Lost to follow-up by age 18. Mackie, et al. Circulation. 2009;120:302-309.

11. Lost to follow-up why is this so important?

12. Verheugt CL et al.EHJ 2010.31:1220-29. Non Cardiac (23%) Multiple (3.6%) Arrhythmia (21.9%) Heart Failure (24.5%) Cardiac, Other (5.1%) Peri-Operative (7.1%) Vascular (14.3%) Many of these can be prevented if the patient maintains UNinterrupted care

13.  Organization of care into regional ACHD centers of excellence

14. http://www.achaheart.org(2010) ACHD “Centers”

15. Alabama Adult Congenital Heart Patients Estimated 9,000 adults with congenital heart disease

16. Alabama ACHD pts: where are they now? 60% of patients are Lost to follow-up…

17.  Team:  ACHD Physicians  ACHD RNs  ACHD Imaging  Interventional Cardiology  Electrophysiology  CV Surgery  Maternal Fetal Medicine Alabama Adult Congenital Heart Program

18. Alabama ACHD clinic locations Birmingham Huntsville Montgomery Mobile The Kirklin Clinic

19. Marc G Cribbs, MD Director, Alabama Adult Congenital Heart Program University of Alabama at Birmingham Children’s of Alabama Alabama Adult Congenital Heart Program