Download Congenital Heart Disease

Congenital Heart Disease
Persisting in Adults
Karen Kuehl MD MPH
WASHINGTON ADULT CONGENITAL HEART
Overview: Congenital
Heart Disease (CHD)
1. Diverse group of diagnoses
2. Operated/repaired versus unrepaired
3. All operated defects have their own residua of
the defect and the repair
4. Treatment changes over time; so the residua of
CHD repaired in 2005 will be different than
same defect repaired in 1970.
5. Of the more severe defects, it is not a given that
the heart has four chambers, two great arteries,
is located in the left chest, etc.
WASHINGTON ADULT CONGENITAL HEART
How many people with ACHD are
there?
• 1,000,000 Adults with Congenital Heart
Disease in US
– 50% have “simple” disease: e.g. ASD repair
– 30% have moderate disease
– 20% have severe disease
• 800,000 children in US have congenital
heart disease: spectrum is more severe
• Annual contribution to adult population
WASHINGTON ADULT CONGENITAL HEART
Specialized Centers
• Bethesda 32 mandated care in Specialized Centers for
Adult Congenital Heart Disease—in 2001
• Care of the Adult With Congenital HeartDisease.
Presented at the 32nd Bethesda Conference, Bethesda,
Maryland, October 2–3, 2000. J Am Coll Cardiol
2001; 37: 1161–98.
• Adult Congenital Heart Association, patient advocacy
group, lists/monitors clinics self reporting as SCACHD.
• www.achaheart.org
WASHINGTON ADULT CONGENITAL HEART
ACHD centers
• Adults with Congenital Heart Disease
should have disability determination made
in specialized centers for the care of adults
with CHD—Bethesda 32 guidelines in
2001 called for care of patients with
moderate or severe illness in such centers
• Win/Win: in such centers it may be
identified that intervention will ameliorate
disability
WASHINGTON ADULT CONGENITAL HEART
Examples
• Mild disease: about 500,000 people in US
– Repaired VSD, small VSD
– Ligated or closed PDA
• Moderate disease: about 300,000 people in US
– Tetralogy of Fallot
– Coarctation of the aorta
• Complex: about 200,000 people in US
–
–
–
–
Single Ventricle
Eisenmenger syndrome
Transposition (dextro- or levo-)
Pulmonary atresia
WASHINGTON ADULT CONGENITAL HEART
NORMAL HEART
Blood from
Body
Blood from
Lungs
Right Atrium
Right
Ventricle
To Lungs
Left Atrium
Left
Ventricle
To Body
WASHINGTON ADULT CONGENITAL HEART
Mild: Ventricular Septal Defect
Blood from
Body
Blood from
Lungs
Right Atrium
Right
Ventricle
To Lungs
Left Atrium
Left
Ventricle
To Body
WASHINGTON ADULT CONGENITAL HEART
Moderate: Tetralogy of Fallot
Blood from
Body
Blood from
Lungs
Right Atrium
Right
Ventricle
To Lungs
Left Atrium
Left Ventricle
To Body
WASHINGTON ADULT CONGENITAL HEART
Complex: Single Ventricle
Blood from
Body
Blood from Lungs
Right Atrium
Left Atrium
Left Ventricle
To Lungs
To Body
WASHINGTON ADULT CONGENITAL HEART
Symptomatic Progression
• Many adults with significant limitation will
be unaware of symptoms
– Onset of symptom may be abrupt e.g. atrial
fibrillation
– Patients limit selves to level of comfort and
have no previous memory of doing more
– 35% of admissions in European database are
via ER in patients followed in SCACHD
WASHINGTON ADULT CONGENITAL HEART
Perception of Disability
• 50% are not in care at all according to
discussion groups on advocacy website
• As patients are older, care is more likely to
occur in community, surgeries in
community hospitals without specialized
care (Gurvitz)
• Perception that patient has been “cured”
WASHINGTON ADULT CONGENITAL HEART
Current Specific ACHD Criteria
Listing 4.06 Symptomatic congenital heart
disease…with one of the following:
A. Cyanosis at rest, and:
1. Hematocrit of 55 percent or greater; or
2. Arterial pO2 saturation of less than 90 percent in
room air, or resting arterial pO2 of 60 Torr or
less.
Many people meeting these criteria have fair to
good exercise capacity
WASHINGTON ADULT CONGENITAL HEART
Current Specific ACHD Criteria
Listing 4.06 Symptomatic congenital heart
disease…with one of the following:
B. Intermittent right-to-left shunting resulting in
cyanosis on exertion (e.g., Eisenmenger's
physiology) and with arterial pO2 of 60 Torr or
less at a workload equivalent to 5 METs or less.
Suggest using fall in pulse oximetry with exercise
rather than requiring Arterial blood gases.
WASHINGTON ADULT CONGENITAL HEART
Current Specific ACHD Criteria
Listing 4.06 Symptomatic congenital heart
disease…with one of the following:
• C. Secondary pulmonary vascular obstructive
disease with pulmonary arterial systolic pressure
elevated to at least 70 percent of the systemic
arterial systolic pressure.
• The level of PA pressures that are relevant need
to be modified in the context of single ventricle
physiology
WASHINGTON ADULT CONGENITAL HEART
Case Example
Middle aged patient with dextrocardia,
double outlet right ventricle, hypoplastic
left ventricle, sub pulmonary stenosis,
ventricular inversion (systemic ventricle is
right ventricle), bilateral superior vena
cava, fistulae from coronary arteries to
right and left ventricle, atrial flutter. See
diagram
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Case Example
Blood from Body
Blood from Lungs
Right
Atrium
LV
Left
Atrium
Right
Ventricle
To Lungs
To Body
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Case Example
• Three episodes of atrial fibrillation and
congestive failure in 2006, 2007, 2008
• No follow up between visits: uninsured
• Works intermittently part time without
insurance available
• Dyspneic on 1-2 flights stairs
• Pulse oximetry 73% in room air
WASHINGTON ADULT CONGENITAL HEART
Case Example
•
•
•
•
Arterial pO2 48 mm Hg
At 2006 cardiac catheterization
Hct 65%
2008 pulse oximetry 73%, no clinical
indication for arterial blood gas
• Application for disability denied
WASHINGTON ADULT CONGENITAL HEART
How Does She Fit Adult Criteria?
• ECG: Consistent with dextrocardia, right bundle
branch block. ST-T changes do not assist in
defining ischemia
• Stress test not done. Short of breath on two
flights steps
• Cardiac catheterization: 2 years ago showed
End diastolic pressures in RV and LV are
normal. Cardiac output is normal. No coronary
obstruction. Coronary fistulae are present
WASHINGTON ADULT CONGENITAL HEART
How Does She Fit Adult Criteria?
• By echo or catheterization or MRI: LV
dimensions are not relevant.
• RV (systemic ventricle) ejection fraction is
normal, as is LV ejection fraction by echo
• Diastolic failure is not related to LV
thickness in this anatomy
WASHINGTON ADULT CONGENITAL HEART
How Does She Fit Adult Criteria?
• Congenital heart disease with resting
cyanosis with saturation < 90% or pO2 <
60 torr—should have qualified this patient
but blood gas data was from cardiac
catheterization in 2006.
WASHINGTON ADULT CONGENITAL HEART
How Does She Fit Adult Criteria
Coronary fistula contribute to myocardial
ischemia, with run off into low pressure
ventricle
Ischemia (lack of adequate oxygen delivery
to heart muscle) in AHCD may be global,
not identifiable by perfusion studies
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Sharing the problem of failure
• Criteria should be easily accessible
on line—not to help MD’s “game the
system” but to help MD’s figure out
what is being sought in determination
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Disability Assessment
• Limitations/Barriers
Assessment in community by providers without
specific skills in ACHD
Lack of recognition of symptoms by patient
Testing to assess disability is multi-disciplinary;
required specialized knowledge: A rose is not a
rose by any other name when the rose is an MRI
or echo
WASHINGTON ADULT CONGENITAL HEART
Appropriate medically
acceptable imaging
• Imaging should be obtained from a center
specializing in the care of congenital heart
disease and preferably from e.g. an
echocardiography laboratory that is
certified for quality. Images obtained from
an inexpert site may be misleading in their
interpretations
WASHINGTON ADULT CONGENITAL HEART
Congestive Heart Failure
• Diastolic failure is an increasingly frequent
finding in adults with structural CHD
• Fontan Failure is a form of congestive heart
failure characterized by low cardiac output.
Pulmonary artery and LV end diastolic
pressures are often normal. These people
have hearts with a single ventricle and no
pumping chamber between atria and lungs.
WASHINGTON ADULT CONGENITAL HEART
Fontan Palliation
Blood from
Body
Blood from Lungs
Right Atrium
Left Atrium
Left Ventricle
To Lungs
To Body
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Fontan Failure
BODY
LUNGS
Heart = Pump
Fragile circulation
Requires very, very low
resistance in lung to allow
passive filling
Requires excellent function
of ventricle to allow filling
Requires different,
functional, criteria for
disability
WASHINGTON ADULT CONGENITAL HEART
Assessment of Disability
• Adults with CHD should have disability
assessment made in specialized centers for
adults with congenital heart disease.
• Bethesda 32 already recommends that
significant testing, e.g. catheterization, MRI, be
done in such centers
• Possible cost benefit to such recommendation
– Finding individuals whose disability may be improved
by appropriate diagnosis and treatment
WASHINGTON ADULT CONGENITAL HEART
Assessment 2
• Functional measures
• Exercise testing with maximal oxygen
consumption
• Remove AICD as contraindication to
paying for exercise testing
• Cardiac output indexed to size from
cardiac catheterization
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Assessment 3
•
•
•
•
Medical regimen for CHF and its durability
Brain natrurietic peptide levels
Recognition of Fontan failure as entity
Pulse oximetry rather than arterial blood
gas
• Fall in pulse oximetry with exercise testing
WASHINGTON ADULT CONGENITAL HEART
Assessment 4
• Z scores ( number of standard deviations
above the mean) for cardiac dimensions
rather than diameters or volumes
• Emphasize MRI dimensions and function
for the right ventricle where possible rather
than echocardiography (my bias)
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Assessment 5
• Cardiomyopathy:
– Non compaction of right or left ventricle is an
associate of congenital heart disease: may
not meet current diagnostic criteria
• Ischemia exists in ACHD in patchy form
without coronary artery disease
WASHINGTON ADULT CONGENITAL HEART
THANKS
Thank you for the opportunity to present
these thoughts and to help people with
congenital heart disease obtain
appropriate recognition of their disability
when it exists.
WASHINGTON ADULT CONGENITAL HEART