Download Adults with congenital heart disease: Patched but not cured

Adults with congenital heart disease: Patched but not cured
by Michael Earing, MD
Michael Earing, MD, is a cardiologist and program director of Adult Congenital Heart Disease at
Children’s Hospital of Wisconsin. He also is an associate professor of Adult Cardiovascular Medicine
and Pediatric Cardiology at The Medical College of Wisconsin and a member of Children’s Specialty
Group.
Introduction
Congenital heart defects are the most common group of birth defects, occurring in approximately
9 in 1,000 live births1. Without treatment, the majority of patients would die in infancy or
childhood, with only 5-15 percent surviving into adulthood2. The advent of surgical procedures
such as ligation of patent ductus arteriosus, resection of coarctation of aorta and the legendary
Blalock-Taussig shunt, as well as advances in diagnostic, interventional and critical care skills,
have resulted in survival of approximately 90 percent of these children to adulthood. Now, for
the first time in history, it is estimated that more adults than children are living with congenital
heart disease in the U.S., with a 5 percent increase every year. This growth rate predicts that in
the next decade, the number of adults with congenital heart disease living in the U.S. will reach
1 million3-4. Unfortunately, the number of physicians formally trained to care for this complex
group of patients is small. In the most recent study, published in 2006, it is estimated that only
31 physicians have received formalized training to care for adults with congenital heart disease in
the U.S. in the last 10 years5. As a result, many adults with congenital heart disease do not have
access to appropriately trained physicians or required medical and surgical services1, 3-5.
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Long-term medical considerations
About 25 percent of adults with congenital heart disease have a mild form that has allowed
them to survive into adulthood without surgical or interventional cardiac catheterization. The
most common lesions in this category include mild aortic valve stenosis (usually in setting of
bicuspid aortic valve), small restrictive ventricular septal defects, mild pulmonary valve stenosis
and mitral valve prolapse. (See Table 1.) These patients need less frequent followup to assess
for progression of disease and to identify associated complications. The majority of adults with
congenital heart disease now
living in the U.S., however, are
Table 1
patients who have had previous
Congenital heart defects associated with survival into adulthood
intervention. (See Table 2.)
without surgery or interventional cardiac catheterization.
Although the majority of
Mild pulmonary valve stenosis
children who undergo surgical
Bicuspid aortic valve
intervention will survive to
Small to moderate size atrial septal defect
adulthood, with few exceptions,
Small ventricular septal defect
“total correction” is not the rule.
Small patent ductus arteriosus
The few exceptions include
Mitral valve prolapse
patent ductus arteriosus,
Partial atrioventricular canal (ostium primum atrial septal defect and
ventricular septal defects and
cleft mitral valve)
atrial septal defects, and only
Marfan syndrome
if they are closed early before
Ebstein’s anomaly
the development of irreversible
Congenitally corrected transposition (atrio-ventricular and ventriculocardiac or lung damage and no
arterial discordance)
residual lesions exist.
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Physician Referral Center (800) 266-0366
Michael Earing, MD, conducts a follow-up appointment with an adult patient at Children’s Hospital of
Wisconsin. Dr. Earing is one of the few physicians in the U.S. who has received formalized training to care for
adults with congenital heart disease.
Table 2
Most common congenital heart defects surviving to adulthood
after surgery or interventional catheterization.
Aortic valve disease following balloon valvuloplasty or surgical
valvotomy
Pulmonary valve stenosis following balloon valvuloplasty or surgical
valvotomy
The Herma Heart Center
Adult Congenital Heart
Disease Program,
established in 2004, is
dedicated to advancing
Tetralogy of Fallot
the care of adults with
Ventricular septal defect
congenital heart disease.
Complete atrioventricular canal
It offers all aspects of care
Transposition of the great arteries
for adults with congenital
Coarctation of the aorta
heart disease. In addition,
Complex single ventricles after the modified Fontan procedure
the program is dedicated,
through research and
chw.org
education, to improve
the lives of all current
and future patients with
congenital heart disease.
Adult c ongenital
hea rt dis ease
Because adult patients with congenital heart disease
now are surviving longer than ever before, it is becoming
increasingly apparent that even the most simple lesions can
be associated with long-term complications4, 6. These longterm complications include both cardiac and noncardiac
problems. Cardiac complications include arrhythmias and
conduction defects, ventricular dysfunction, residual shunts,
valvular lesions (regurgitation and stenosis), hypertension
and aneurysms. Noncardiac sequelae include developmental
abnormalities such as developmental delay, somatic
abnormalities such as facial dysmorphism (cleft palate/lip),
central nervous abnormalities such as seizure disorders from
A collaboration between
Children’s Hospital of
Wisconsin and Froedtert
Hospital, the program
now serves more than
1,700 patients annually.
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previous thromboembolic events or cerebrovascular accidents, disturbances of the senses such
as hearing or vision loss and, finally, pulmonary sequelae such as restrictive and obstructive lung
disease. In addition, psychosocial problems involving employment, life and health insurance,
participation in sports, sexual activity and contraception are common. As a result of these longterm complications, the majority of adults with congenital heart disease need lifelong followup4.
References
1. Perloff JK, Warnes CA. Challenges Posed by Adults with Repaired Congenital Heart Disease. Circulation. 2001; 103(21): 2637.
2. Mitchell SC, Korones SB, Berendes HW. Congenital Heart Disease in 56,109 Births: Incidence and Natural History. Circulation. 1971;
43: 323-332.
3. Congenital Heart Disease After Childhood: An Expanding Patient Population: 22nd Bethesda Conference, Maryland, October 18-19,
1990. J Am Coll Cardiol. 1991; 18: 311-342.
4. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease: A
Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to
Develop Guidelines on Management of Adults with Congenital Heart Disease): Developed in Collaboration with American Society of
Echocardiography, Heart Rhythm Society, International Society of Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Intervention, and the Society of Thoracic Surgeons. Circulation. 2008; 118: e714-e833.
5. Gurvitz MZ, Chang RK, Ramos FJ, et al. Variation in Adult Congenital Heart Disease Training in Adult and Pediatric Cardiology Fellowship Programs. J Am Coll Cardiol 2005. Sep; 46(5): 893-8.
6. Earing MG, Connolly HM, Dearani JA, et al. Long-term Follow-up of Patients After Surgical Treatment of Isolated Pulmonary Valve
Stenosis. Mayo Clin Proc 2005. Jul; 80(7): 871-6.
Adult congenital heart disease CME webcast
Practical Pediatrics Grand Rounds CME webcasts provide lectures on recent advances and
current knowledge in pediatrics. Each lecture is available on demand, allowing you to earn
CME credit when it is convenient for you.
“Congenital Heart Disease in Adults” by Michael Earing, MD
Objectives
• Discuss common medical issues that affect adults with congenital heart disease.
• Prevent current psychosocial challenges faced by adults with congenital heart disease.
• Discuss current research projects in progress to address medical issues and psychosocial
issues.
This lecture is available online at chw.org/practicalpediatrics.
See Page 19 for CME information.
For more information, visit chw.org/hermaheartcenter.
For outcomes from our Herma Heart Center, visit
chw.org/quality.
To refer a patient, visit chw.org/refer or call toll-free
(800) 266-0366.
Adult c ongenita l
hea rt dis eas e
Heart Matters
Children’s Hospital of Wisconsin’s Herma Heart Center is one
of the nation’s top programs for medical and surgical treatment
of congenital heart defects and heart disease in children. As
one of the largest pediatric cardiac programs in the United
States, we have set national benchmarks for surgical outcomes.
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Physician Referral Center (800) 266-0366