Download ADULT CONGENITAL HEART DISEASE: THE ELEPHANT IN THE

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