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Adult Congenital Heart:
Current Status &
Long Term Issues
25th Annual CV Symposium
Marc D. Knepp, MD
November 4, 2016
I HAVE NO FINANCIAL
DISCLOSURES
Objectives
1. Define the current status of Congenital Heart
Disease in the Adult
2. Identify co-morbidities of Grown Up Congenital
Heart Disease
3. Discuss the importance of long term follow up and
disease specific counseling
Congenital Heart Disease
• Malformation of the heart present at birth
• Most common birth defect
– 8 per 1000 live births
• Common Forms
– Ventricular Septal Defect (VSD)
– Atrial Septal Defect (ASD)
– Tetralogy of Fallot (TOF)
– Transposition of the Great Arteries (TGA)
• 10% diagnosed as an Adult
Treatment of CHD
• Medical therapy only temporizes
– 50% survival to adulthood
• First surgical intervention, 1938
– PDA ligation, Gross & Hubbard, Children’s
Boston
• Universally fatal until the 1940s
– Shunt surgery devised by Dr. Helen Taussig
– Performed by Dr. Arthur Blalock
• November 1944, Johns Hopkins
Early Congenital Heart Surgery
• 1954– Dr. C.W. Lillehei performed the first TOF
repair
– Cross Circulation
Cross Circulation
Cardio-Pulmonary Bypass
• Revolutionized the Care of CHD
Survival of Unrepaired
Tetralogy of Fallot
Betranou EG, Blackstone EH, Hazelrig JB, Kirklin JW. Life expectancy without
surgery in tetralogy of Fallot. American Journal of Cardiology 1978
Survival of Repaired TOF
Murphy JG, Gersh BJ, Mair DD et al. Long-term outcome in patients undergoing
surgical repair of tetralogy of Fallot. N Engl J Med 1993: 329:593-599
But some work yet to do…..
Nollert et al. Long-Term Survival in Patients With Repair of Tetralogy of Fallot: 36-Year FollowUp of 490 Survivors of the First Year After Surgical Repair. JACC 1997: 1374-1383
Survival doesn’t mean cured
• In 2015, >90% of newborns with CHD will live until
adulthood
• Even with successful neonatal or childhood
repairs, many will still need procedures and follow
up thru life
www.achaheart.org
Diller et al Circulation 2015
Diller et al Circulation 2015
Cause of Death
Adult Congenital in the US
• Over 1.3 million Adult Congenital patients
– >50% of all congenital heart patients
– 35-40,000 CHD pts/yr turn 18
• 18 different distinct Congenital Heart Lesions
• Adults with CHD have 3-4x higher rates of ER
visits, hospitalizations, and ICU admits, compared
to general population
1. www.achaheart.org
2. Facing Lifesaving Heart Surgery, Twice. Wall Street
Journal, 8/20/12
Annual ACHD
Hospitalizations
Agarwal et al. JAHA 2016
Age of Admitted ACHD Patient
Cost of Hospital ACHD Care
Long Term Follow Up Issues
• Heart Failure
• Arrhythmia
– Sudden Cardiac Death
• Re-operations/interventions
• Pregnancy
• Guidelines, whenever possible, “evidence based”
• Total of 647 references used
• Expert consensus used when lacking evidence
Seen Regularly in ACHD clinic
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Single Ventricle
Cyanotic
Transposition
Truncus Arteriosus
Conduits
Periodic ACHD follow up
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•
•
Tetralogy of Fallot
Aortic Coarctation
Complicated VSD
Complicated ASD
Anom Pulm Veins
Ebstein’s anomaly
Non-routine ACHD patients
Tetralogy of Fallot
• Repairs in 1950s
• Improved survival
• Longterm issuses
– Heart failure
– Arrhythmia
– Pulmonary Valve
replacement
– Sudden Cardiac
Death
Aortic Coarctation
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•
•
•
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Hypertension
Re-coarctation
Aneurysm
Cerebral Aneurysm
Early CAD
Premature Death
D-Transposition
Atrial Switch Repair of DTGA
•
•
•
•
Mustard/Senning
1960s
Arrhythmia
Systemic RV
failure
Arterial Switch for DTGA
•
•
•
•
•
•
Jatene
1980s
Heart Failure
PA stenosis
Aortic root dilation
Coronary ischemia
Hypoplastic Left Heart
Fontan Palliation—Single V
Fontan Issues…..
• Surgery in 1970s
– DILV
– Tricuspid Atresia
• Norwood procedure
– HLHS, 1980-90s
• 70% survival to
adulthood
•
•
•
•
•
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Arrhythmia
Systolic failure
“Fontan” failure
PLE
Liver Failure/HCC
Renal Failure
Cyanosis
PREGNANCY
Pregnancy in ACHD
• With more survivors and less morbidity more
ability to make babies
• Most woman with CHD can carry a successful
pregnancy
• Risk of CHD Recurrence
– On average 5-8%
– Up to 50%
• Cardiac disease is rapidly becoming the #1 cause
of peri-partum mortality
Normal Physiologic Changes
• Reduced Systemic Vascular Resistance
 BP falls early in gestation, 10mmHg below baseline
by 2nd trimester
• 30-50% increase in intravascular volume &
cardiac output
• Increased lower extremity venous pressure
• Fluctuation in cardiac output during labor &
delivery
• Increased risk of thromboembolism
Hemodynamic Changes
Summary
Kaleschke & Baumgartner. Heart 2011.
For Example….
With decreased SVR/BP…
Eisenmenger Syndrome
• Long standing left to right shunt
(usually thru VSD) that has become
right to left shunt due to development
of Pulmonary Hypertension.
• Contra-indication to pregnancy
– 50% mortality
Another example
“Flood behind the Dam”
Heart Failure Example
“Pump Failure”
ACHD in Peoria
• One of the 20 busiest clinics in US
– Over 1400 visits per year
• Formal transition program from Pediatric to Adult
Congenital Team
• ~40% of patient visits at Congenital Heart Center
are adults
THANK YOU!
QUESTIONS?