1. health and fitness
2. disease
3. heart disease

4/6/2015
Adult Congenital Cardiology Care:
From Cradle to (LATE) Grave
May Ling Mah, MD MS
mayling.mah@nationwidechildrens.org
Columbus Ohio Adult Congenital Heart Program
Phone 614‐722‐5622
No Disclosures
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Objectives
y Stratify congenital heart disease patients into different risk groups which may require specialized cardiac care
y Identify high risk periods of time for adult congenital heart patients
y Recognize the anticipated complications
y Recognize reasons for and rate of reoperation for some common congenital heart diseases
Who are they, and where did they all come from?
Past Medical History is Always Important
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History of Congenital Heart Surgery (Abbreviated)
y 1938: Ligation of the Ductus arteriosus (Dr. Gross, Boston)
y 1944: Aortic coarctation repair (Dr. Crafoord, Stockholm)
y 1944: Blalock‐Taussig shunt for Tetralogy of Fallot (Baltimore)
y 1953: First “Heart‐Lung Machine” permitting intracardiac
repairs (Dr. Gibbon)
y 1954: “Complete” repair of Tetralogy of Fallot (Dr. Lillehei, Minneapolis)
It was a dark and stormy night . . .
August 31, 1954, Minneapolis
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History of Congenital Heart Surgery (Abbreviated)
y 1963: Mustard and Senning atrial switch procedures for Transposition of the Great Arteries
y 1971: Fontan palliation for single ventricles
y 1975: Jatene and Yacoub Arterial switch procedures for Transposition of the Great Arteries
Fast Forward
y Oldest congenital heart survivors are in their 6th and 7th decades
y Oldest “complex” survivors are 70 (TOF)
y The surgeries for heart defects changed over time
TOF born in 1946 versus 1996
{ D‐TGA born in 1965 versus 1985
{
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Childhood Diseases
y Dramatic advances in medical/surgical care.
y Once fatal diseases have grown into adulthood.
{ Cystic fibrosis – 40% of current pts are adult.
{ Childhood cancer – 80% survival to adulthood.
y 85+ % of CHD babies born today reach adult age.
{ 60 ‐ 90% mortality reduction from 1979 – 2005.
y Greatest advances in complex disease.
{ Patient profile is changing: older and more complex.
JACC 2001;37:1170; Am Heart J 2004;147;425; Heart 2001;85:438; Am Heart J 2009;158:874
Survival: TOF by decade of birth
(Toronto)
% Survival
1980
n= 1693 (1927-1983)
1970
1960
1950
1940
1930
Years
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Changing Age Distribution
Marelli et al., Circulation 2007; 115: 163-72.
Aggregate Survival
Changing Severity Distribution
% Survival to adulthood
Simple
100
Complex
95
90
95
90
80
80
60
Moderate
65
55
50
40
20
10
0
1940-1959
1960-1979
1980-1989
Birth Year
JACC 2001;37:1170
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ACHD Jenga
Diagnostic Issues
Congenital Heart Lesion
Decade and Country of Birth
Medical and Surgical Options
Immediate Survival
Reoperations (improved options)
Survival into Adulthood
Parents Scarred for Life
Loss of Records
Advent of a Young Field
Study of ACHA clinic directory
y United States: 72 programs
{
35 states at least 1 program
y 204 staff Cardiologists
{ 42 % Pediatrics + Pediatric Cardiology
{ 37% Internal Medicine + Adult Cardiology
{ 16% Internal Medicine/Pediatrics + Cardiology
y 70% affiliations with Ped and Adult hospital
Pediatr Cardiol 2010
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Field Development
y Number of articles in PubMed has skyrocketed on the topic
y Recommendations change often, as new data is obtained and longer‐term followup is possible
Tetralogy of Fallot
Ù Fallacies: “repair” should be durable for life
{ Cyanosis
Ù Fallacies: phlebotomy is helpful
{
Barriers to Care Delivery
y Increased disease burden – especially complex
y Lack of providers with sufficient training.
{ ACHD minority of Adult HD: 1 million vs 22+ million.
{ Expected > 9,000 new patients per year.
y Inconsistent transition and follow‐up for appropriate specialty care.
y Knowledge gap {
Ownership of their own health information
y Sense that they have been “cured”
y Insurability
JACC 2001;37:1170; Am Heart J 2004;147;425; Heart 2001;85:438
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Cured
Why can they not be trusted?
By
Surgeon
Common Problems
y Hospitalizations y Arrhythmia
y Heart failure
y Reoperation
y Pregnancy
y Neurocognitive Delay
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CHD Patient Timeline
Wellness
Goals of advanced congenital heart care – minimize decline and maximize wellness throughout life
Vulnerable period
Reasons for lost to follow-up
•
Generally well – gradual decline
•
Lack of education about disease
•
Lack of knowledge ACHD resources
•
Loss of coverage
•
Gaining independence – mobile
•
Child-bearing
Hospitalizations
y Increasing emergent admissions with age
{ CHD – 12‐18 yo: 20% vs 19‐44 yo: 40‐50% emergent
{ Often first visit to center
y 83% of the time cardiac in nature
{ Arrhythmia; Heart failure; Sudden arrest; Procedures
y Increasing presentation with pregnancy
y Complications during non‐cardiac procedures
y Complex disease account for the most
{
65% of all ACHD admissions
J Thorac Cardiovasc Surg 2003:126;1048; JACC 2007:49;875; JACC 2001:37;1161
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Arrhythmia
y Certain arrhythmias anticipated in post‐op ACHD
y Tetralogy of Fallot
{ 30% SVT; 10% Ventricular Tachycardia
{ 5% AV block/Sinus node dysfunction
{ Increasing need for ICDs and pacemakers
y TGA s/p atrial switch; Single ventricle s/p Fontan
{ 50% SVT; Increased sinus node dysfunction with age
{ Increased risk after 2nd decade from surgery
Circ 2009:120;1679; Circ 2007:115;534
Arrhythmia
Complex intra‐cardiac and venous anatomy
Transposition
s/p atrial switch
Hemodynamic intolerance of traditional medical management
Tricuspid Atresia
s/p atriopulmonary
Fontan
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Arrhythmia
Management:
• Onset usually heralds alteration in physiology
y Tendency to NOT tolerate loss of AV synchrony
{
Atrial arrhythmias, SA node dysfunction, and AV block
y Hasten deterioration in ventricular function { Favor early cardioversion (pacing, medical, DCCV) to restore sinus rhythm over rate control
y Thrombus remains a concern
{ Systemic vs Pulmonary; consider residual shunting
Heart Failure
Am J Card 2006:97;1238
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Heart Failure
Compared to non‐CHD HF
y Different anatomy: single ventricle; systemic RV
y Multi‐factorial cause adds complexity
y Average age: non‐CHD much older
y Mortality rates: non‐CHD higher mortality
y Reports of higher NYHA scores
y Small numbers with lack of RCTs
y Standard therapy utilized, but consider residual defects and rhythm status.
J Cardiac Failure 2005;11:306; Int J Cardiol 2004;97:25
Reoperation
y Operations during life common
{ 50% > 2 operations; 23% > 3 operations
y Operative mortality: 2 ‐ 5%
y Highest risk: cyanotic, increasing age, heart failure, co‐morbid conditions, inexperienced center
JACC 2007:49;875; JACC 2001:37;1161; Ann Thorac Surg 2009:88;1284
50% of US patients who have undergone surgery require surgery at an older age (Warnes et al, 2001)
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The Cardiac Picture of Chronic Disease
Starts in Childhood
Number of Prior Sternotomies
0
33%
1
2
67%
3
4
5
>6
Jacobs et al. 2014 Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann
Reoperation Mortality
Dearani J, et al. Cardio Young 2007
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Factors for Success in Adults having Reoperation
y Mortality (Nationwide Inpatient Sample).
Hospital: Adult vs Pediatric Ù Similar Mortality (4.3% vs 5.1%).
{ Surgeon: Adult vs Pediatric
Ù Differing Mortality (4.84% vs 1.87%)
{
y Survival advantage with high pediatric volume
• Co‐morbid conditions required specialty consultation in > 30% of cases
J Thorac Cardiovasc Surg 2008:136;307; Circ 2008:118;2345
Pregnancy: Re‐entry to Care
Maternal Cardiology Clinic Referral Source
10% increase in TOTAL ACHD clinical volumes
OB (61%)
Peds (24%)
Medicine (8.5%)
Self/Echo (6.8%)
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Why does this require a Congenital Cardiologist?
Maternal Cardiology Clinic Disease Mix
Congenital (71%)
Arrhythmia (6.8%)
Cardiomyopathy (6.8%)
Marfan/CTD (6.8%)
Other (8.5%)
General Maternal Cardiology Considerations
y Interplay of two complex systems
y What does the patient know about her disease?
y What is “heart failure” in pregnancy?
y Who can/should get pregnant?
y What can be done to make it successful?
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Interplay of Disease
Benign in Pregnancy
Serious in Pregnancy
ASD, repaired
Mild AS
VSD, repaired
Benign
Cardiac
TOF with PS
Mod AS
TOF with PI
Bicuspid AV
PPM CM
Marfans
Serious
Cardiac
Mod Aortic Regurg
Mustard/Senning
Mod Mitral Regurg
Fontan
Severe AS
Severe Aortic Regurg
Severe Mitral Regurg
Severe MS
Eisenmengers
Cyanotic
Traditional Heart Failure Scoring
y New York Heart Association Classifications
Pregnancy Week
=1-10
=11-16
=17-28
=29-40
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Hemodynamic changes in pregnancy
Plasma Volume
Increase up to 40%
Systemic vascular resistance
Decrease 25‐30%
Cardiac output
Increases 40%
Heart Rate
Increases 10‐20bpm
Blood pressure
Decreases 1st and 2nd trimester
Back to normal in 3rd trimester
Central Venous Pressure
Unchanged
Nelson-Piercey, Handbook of Obstetric Medicine 2002
Intrapartum Hemodynamics
y Labor – further increase in cardiac output { Uterine contractions Æ
auto‐transfusion of 300‐500ml of blood back into circulation
y Pain Æ sympathetic response Æ increase in HR and BP
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Postpartum Hemodynamic Changes
y Immediate rise in cardiac output
(Relief of IVC obstruction and uterine contraction)
y Cardiac output increases 60‐80% followed by rapid decline to pre‐labor values within 1 hour of delivery
y Transfer of fluid from extravascular space increases venous return and stroke volume
Nelson-Piercey, Handbook of Obstetric Medicine 2002
What does this mean for us?
y Minimal data on more complex lesions
{ Must extrapolate
Art, not science
Ù Understand “how the blood goes round”
Ù
{
Understanding of individual shunts and valve lesions often must be considered in combination
y Of assistance . . .
{ General hemodynamic principles
{ General risk stratification models
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General hemodynamic principles
y Right heart valve disease
Obstruction well tolerated
{ Regurgitation not as well tolerated
{ Case example: Tetralogy of Fallot
{
y Left heart valve disease
Obstruction (even mild) not as tolerable
{ Regurgitation well tolerated
Ù drop in SVR and placenta are better afterload reducers than ACE‐Inhibitors
{ Case example: Bicuspid aortic valve disease
{
General hemodynamic principles
y Anemia
{ Obstructive Diseases, echo gradients worsen
{ May or may not matter functionally
{ WILL matter for anesthesia
y Vascular abnormalities
{ Marfans
{ Bicuspid Aortic Valves
y Cyanosis
{ Outcomes worsen in relation to lower sats
{ Often due to intracardiac shunts
{ Increased risk of thromboembolism
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Neurocognitive Outcomes
y Risk for long‐term neuropsychological consequences
y Association with genetic syndromes
Prevalence of neurodevelopmental impairment in the population with congenital heart
disease (CHD).
10%
30%
60%
Bradley S. Marino et al. Circulation. 2012;126:1143-1172
Copyright © American Heart Association, Inc. All rights reserved.
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Lessons from Other Disease
y Sickle Cell (King, 2014)
{
Multivariable linear regression of full‐scale IQ (FSIQ)
Absence of college education head of household Presence of silent cerebral infarct Each $1000 of family income per capita Each increase in year of age Each 1% decrease in HgB oxygen sat {
{
‐6.2
‐5.2
+0.33 ‐0.96
‐0.75 (p 0.005)
(p=0.017)
(p=0.023)
(p=0.023)
(p=0.03)
Why wouldn’t these factors may affect our patients as well?
Isn’t sickle cell a chronic progressive disease? This isn’t like our patients . . . Or is it?
What Does This Mean?
y Presumption of cognitive “normality” is wrong
{ Even if there is no syndrome
{ Even in our TOF and d‐TGA patients (“moderate” disease)
{ Outside of socioeconomic and nurture characteristics
Ù Effect sizes were GREATER when comparison was to siblings
y Neurocognitive defects are salient in older CHD survivors (not ameliorated over time)
y Larger effect sizes in older patients
{
Is this like sickle cell?
y Defects most pronounced in full‐scale IQ and processing speed. Verbal IQ was preserved
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Clinical Impact
y Functioning at a level below the family norm or expectation is very relevant
y This self‐perceived relative “incompetence” was expressed by many cases
y Self‐impression impacts psychosocial adjustment and behavior
Self‐perceived impaired psychosocial functioning in 18.6% of adolescents 13‐18 with CHD (Uzark et al 2008)
{ Severity of disease does not predictably correlate with psychosocial outcomes (Uzark et al 2008)
{
What do I do?
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Who Wouldn’t Want to Help?
y Identification of patients lost to congenital cardiology
{ Montreal data: 50% attrition even in single‐payer system!
y Recognition of changes in patients you know best
y Often lots of medications and lots of monitoring
y Recognition that ACHD is a chronic, systemic condition
{ “Common” or “Typical” issues and procedures can tip the balance in patients
Ù Gall bladder surgery
Ù Pregnancy
Ù Any procedure requiring anesthesia
{ Conservative measures when possible
y Communication is KEY in team approach
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Disease Severity Classifications
y Simple
{ Isolated native disease (BAV; Mitral valve; small VSD).
{ Intervened with low future risk: ASD; VSD; PDA.
y Moderate
{ Atrioventricular septal defects; Significant ASDs; Coarctation
of the aorta; Ebstein’s anomaly; Tetralogy of Fallot; Anomalous pulmonary venous drainage.
y Complex
{ Residual cyanosis (Eisenmenger); Single ventricle: s/p Fontan procedure; Transposition s/p atrial/arterial switch.
JACC 2001;37:1161
Circ 2008;118(23):e714-833
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ACC/AHA Guidelines Highlights
1. ACHD Care should be coordinated at ACHD centers
a.
New diagnosis; Ongoing care of medium/complex CHD; Evaluation before non‐cardiac surgery; Interventions
b.
Pre‐pregnancy evaluation and counseling
2. ACHD Pacemakers and Devices by staff familiar with anatomy and prior procedures 3. SBE prophylaxis: Lack of evidence demonstrating proven benefit of infective endocarditis prophylaxis
a.
Reasonable in highest risk patients: Prosthetic valves, previous endocarditis, unrepaired/palliated CHD, repaired CHD within 6 months of procedure, and repaired CHD with residual defects. Circ 2008;118(23):e714-833
Take Home Message . . .
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Our Villagers
Conclusions
y Due to the success of pediatric medicine, the number of patients with congenital heart disease reaching adulthood will continue to increase.
y No longer are patients considered cured – lifelong potential for issues – many which can be anticipated.
y Care of congenital heart disease in adulthood requires an experienced team approach, especially for moderate‐severe disease (this includes you).
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