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ADULT CONGENITAL HEART DISEASE: THE ELEPHANT IN THE ROOM Alison Knauth Meadows, MD, PhD Director, Adult Congenital Hear t Program Nor thern California Kaiser Permanente OUTLINE Definition of adult congenital heart disease A historical perspective Challenges faced by patients and providers International efforts to improve the delivery of care Our approach to care - Northern California Kaiser Permanente DEFINITION ADULTS WITH CONGENITAL HEART DISEASE: SURGICAL HISTORY 1938: First PDA ligation 1944: First aortic coarctation repair 1945: First Blalock-Taussig shunt 1950’s: Introduction of cardiopulmonary bypass - facilitated repair of TOF, CAVC, D-TGA, VSDs 1970’s: Fontan procedure introduced 1980’s: Neonatal surgery began - allowed repair of D-TGA with arterial switch procedure and palliation for HLHS ADULTS WITH CONGENITAL HEART DISEASE: OTHER ADVANCES Advances in catheter based interventions Advances in imaging 2D and 3D echocardiography with tissue Doppler Cardiac MRI Cardiac CT Advances in intensive care management Balloon pump ECMO Ventricular assist devices Advances in medical management ADULTS WITH CONGENITAL HEART DISEASE: THE EXPLOSION! Incidence of CHD = 1% 85% will survive to adulthood > 1,000,000 adults with CHD in the US Adults with CHD > children with CHD > 1/3 have moderate to severe disease Fewer than 30% are getting specialty care ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES Few are trained both in congenital heart disease AND the care of adults The pediatric cardiologists don’t want to do it! ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES Few are trained both in congenital heart disease AND the care of adults The adult cardiologists don’t want to do it! ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES The anatomy and physiology are complex No hearts look alike before intervention No hearts look alike after intervention ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES There are insufficient data to guide care ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES Complications and comorbidities are often different from those in the typical adult with acquired heart disease. ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES Complications and comorbidities are often different from those in the typical adult with acquired heart disease. Syncope in 25 year old patient with tetralogy of Fallot may be ventricular tachycardia ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES Complications and comorbidities are often different from those in the typical adult with acquired heart disease Chronic headaches in patient with D-TGA status post Mustard may be intracardiac baffle obstruction ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES Complications and comorbidities are often different from those in the typical adult with acquired heart disease Nausea and vomiting in patient with a single ventricle status post Fontan may be systemic venous congestion ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES Complications and comorbidities are often different from those in the typical adult with acquired heart disease SHTN in patient with repaired coarctation may have residual obstruction ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES If a patient presents with symptoms, we have often waited too long! 27 year old with TOF who presents with right heart failure during second pregnancy ADULTS WITH CONGENITAL HEART DISEASE: THE CHALLENGES Young adults with CHD are Frequently lost to follow up Often ill prepared to take responsibility for their health and lives Often demonstrate high risk behaviors PROGRESS IN THE DELIVERY OF CARE AHA/ACC guidelines Training requirements Subspecialty certification T YPES OF ADULT CONGENITAL HEART DISEASE Simple Disease At least one consultation with ACHD specialist Moderate Disease Yearly follow up with ACHD specialist Complex Disease At least yearly, often more frequent visits with ACHD specialist DO WE HAVE ENOUGH PROVIDERS? ADULT CONGENITAL HEART DISEASE FELLOWSHIP PROGRAMS 19 fellowship programs world wide 11 USA (58 graduates) 3 Canada (31 graduates) 6 international (49 graduates) Curriculum for fellowship programs Pediatric pathway Internal medicine pathway Curriculum based on 32 nd Bethesda Conference Application for subspecialty certification (ABIM, August 2011) ACHD PROGRAM NORTHERN CALIFORNIA KAISER To provide comprehensive medical and interventional care for adults with CHD Medical management EP management Medications EPS interventions PM/AICD management Cardiac catheterization Hemodynamics Congenital interventions Congential CV surgery Cardiac MRI/CT Echocardiography Exercise testing with metabolic cart ACHD PROGRAM NORTHERN CALIFORNIA KAISER What are we doing for these patients ? Our estimates – 10,000 adults with CHD in the Northern California Kaiser Permanente health care system Our program - Established October 2010 Our team – ACHD ACHD ACHD ACHD cardiologist – Alison Meadows nurse practitioner – Jennifer Gilmartin program coordinator – Marlene Enderlein echocardiogram technician – Doug Huffines NORTHERN CALIFORNIA KAISER PERMANENTE HUB AND SPOKE MODEL South Bay Capital Central East Bay ACHD Program SF Kaiser North East Bay Golden Gate ACHD PROGRAM, NORTHERN CA KAISER To serve as resource for health care providers to meet the needs of adults with CHD Initial consultation Continued co-follow for complex patients Pediatric cardiologists (<18-21 yrs) Adult cardiologists (>18-21 yrs) Regional case conferences Educational webinars 1 on 1 telephone communication ACHD PROGRAM, NORTHERN CA KAISER Outpatient clinic 350 patients, the majority we continue to co-follow 20-30 new consults per month Transition: With regional Kaiser pediatric cardiology, we are developing a transition program for young adults with complex CHD to help them take responsibility for their hearts, health, and lives. High risk OB/Gyn: With high risk Ob/Gyn, we are co-managing high risk ACHD patients through pregnancy, L&D, and post partum. Electrophysiology: We help manage PM/AICDs to give recommendations about optimal pacing strategies. ACHD PROGRAM, NORTHERN CA KAISER Inpatient care Medical admissions Congestive heart failure Arrythmias Other comorbidities Transfers from other facilities Surgical admissions > 50 congenital CV surgical cases at Kaiser SFO Average 1 case per week ACHD PROGRAM, NORTHERN CA KAISER Noninvasive imaging/testing Echocardiography Cardiac MRI – Nearly 300 cardiac MRIs, 60% congenital (pediatric and adult) ETT with metabolic cart – In process ACHD PROGRAM, NORTHERN CA KAISER Cardiac catheterization Pediatric cardiac cath lab (UCSF) - interventional (or complex hemodynamic) caths (> 50 cases) Adult cardiac cath lab (Kaiser SFO) - hemodynamic cardiac caths and coronary interventions EPS and EP interventions: Petiatric EP lab (UCSF) - patients with complex anatomy and complex rhythm issues EP lab (Kaiser SCL) - many EP studies and ablations ACHD PROGRAM NORTHERN CALIFORNIAL KAISER CONCLUSIONS Adults with Congenital Hear t Disease represent a large and growing population of medically and psychosocially complex patients There are many challenges we face in providing optimal care for these patients Few are trained both in CHD and the care of adults Their anatomy and physiology are complex There are insufficient data to guide care Complications and comorbidities are often different from those in the typical adult with acquired heart disease and therapeutic options are often not standard If a patient presents with symptoms, we have often waited too long! Patients are often lost to follow up, often representing with symptoms. CONCLUSIONS Internationally, we have made progress in improving the delivery of care by establishing Guidelines Training requirements Subspecialty certification In the Northern California Kaiser Permanente system, we have developed a program to provide comprehensive medical and interventional care for adults with CHD