|
Amanda Jekowsky , American College of
Cardiology, 202-375-6645, ajekowsk@acc.org
November
7, 2008
Guidelines Break New Ground in Adult
Congenital Heart Disease
First-ever guidelines offer cardiologists
comprehensive tools for managing lifetime care
These days most children born with congenital
heart disease live well into adulthood, thanks to innovative
surgical, interventional and medical treatments. That means
that not only are cardiologists caring for a growing number
of adults with repaired heart defects, but the resulting cardiac
anatomy and physiology are often much more complex than in
the past.
To assist cardiologists in making everyday clinical decisions
for this challenging group of patients—and in knowing
when to refer patients to specialists with expertise in congenital
heart disease—the American College of Cardiology and
the American Heart Association have jointly released a comprehensive
set of practice guidelines on the management of adults with
congenital heart disease (CHD).
The guidelines—the first of their kind in the United
States—appear in the December 2, 2008, issue of the
Journal of the American College of Cardiology (JACC) and
the December 2, 2008, issue of Circulation. They
will be published online on November 7, 2008.
“Adult cardiologists have never before been presented
with patients who have such complex congenital heart disease,
because in the past, most of these patients did not survive
to adulthood,” said Roberta G. Williams, M.D., co-chair
of the guidelines writing committee. “These guidelines
are an important component of a multi-pronged strategy to
provide appropriate care for adults with congenital heart
disease.” Dr. Williams is chair of pediatrics at the
Keck School of Medicine of the University of Southern California
and vice president for pediatric and academic affairs for
Childrens Hospital Los Angeles.
Congenital heart defects can be relatively simple—a
small, repairable hole between the left and right sides of
the heart, for example. But many forms of CHD are much more
complex. A baby may be born with only one ventricle (the lower
chambers of the heart; normally there are two). Or the two
large arteries that carry blood away from the baby’s
heart may be switched, so that blood does not flow through
the lungs and pick up oxygen before being pumped to the rest
of the body. Heart valves may be misshapen or narrowed. There
are many other types of congenital heart defects, and many
variations of each, making congenital heart disease one of
the most challenging areas of cardiology.
Over the years, cardiac surgeons and interventional cardiologists
have become highly skilled at repairing heart defects. That
doesn’t mean that all children who have undergone surgical
procedures are completely cured, however. “That’s
a common misconception,” said Carole A. Warnes, M.D.,
co-chair of the guidelines writing committee, a professor
of medicine at the Mayo Clinic in Rochester, MN, and director
of Mayo’s adult congenital heart disease clinic. “Some
patients leave their pediatric cardiologist and think they
don’t need to see a physician anymore, and others are
seen by a cardiologist with no training in congenital heart
disease. We really need to focus on how to better care for
this population.”
In reality, nearly all patients need follow-up throughout
adult life. Some will eventually need additional surgery to
correct complications that arise over time. Most will need
guidance on how their heart condition affects the important
milestones of adulthood, such as employment, pregnancy and
physical activity.
Without ongoing care and follow-up by a physician with training
in congenital heart disease, the results can be tragic. Worsening
of a leaky heart valve may be overlooked until the patient
is in heart failure, making surgery highly risky or even impossible.
A young woman may be told she cannot have children, when in
fact, pregnancy might be possible if managed at a center with
expertise in congenital heart disease. A seemingly routine
appendectomy may turn into a life-threatening crisis.
To help avoid such problems, the guidelines provide comprehensive
information on the clinical features, diagnosis, and medical,
surgical and interventional therapy of a range of congenital
heart defects. They also point out common problems and pitfalls,
highlight key issues to look for during follow-up, and make
recommendations on genetic testing, pregnancy, contraception
and physical activity.
In addition, the guidelines call for:
- Coordination of the ongoing care of patients through
regional centers of excellence with expertise in adult congenital
heart disease;
- Individual and family counseling, including the early
education of children on their heart condition and what
to do to stay healthy;
- A formal transition process to help teenagers and young
adults cross the bridge from their pediatric cardiologist
to an adult cardiologist;
- Outreach and education programs to bring patients back
into the healthcare system if they are no longer receiving
appropriate care and follow-up;
- Education about the risks of infection of the inner lining
of the heart or the heart valves posed by dental procedures,
tattoos, body piercings, and other procedures that may introduce
bacteria into the bloodstream;
- Thorough clinical evaluation of patients before noncardiac
surgery or any procedure requiring anesthesia or sedation,
coordinated through a regional center of excellence;
- Counseling about safe contraception;
- Consultation before pregnancy, including genetic counseling,
so that patients understand the risks to both mother and
baby.
The guidelines were developed in collaboration with the American
Society of Echocardiography, Canadian Cardiovascular Society,
Heart Rhythm Society, International Society for Adult Congenital
Cardiac Disease, Society for Cardiovascular Angiography and
Interventions, and Society of Thoracic Surgeons.
“We benefited from the diverse experience of adult
cardiologists, pediatric cardiologists, cardiac surgeons,
advanced practice nurses, and multiple institutions across
the country,” Dr. Williams said. “It represents
a wonderful collaboration.”
###
The American College of Cardiology is leading the way to optimal
cardiovascular care and disease prevention. The College is
a 36,000-member nonprofit medical society and bestows the
credential Fellow of the American College of Cardiology upon
physicians who meet its stringent qualifications. The College
is a leader in the formulation of health policy, standards
and guidelines, and is a staunch supporter of cardiovascular
research. The ACC provides professional education and operates
national registries for the measurement and improvement of
quality care. More information about the association is available
online at www.acc.org .
The American College of Cardiology (ACC) provides these news
reports of clinical studies published in the Journal of
the American College of Cardiology as a service to physicians,
the media, the public and other interested parties. However,
statements or opinions expressed in these reports reflect
the view of the author(s) and do not represent official policy
of the ACC unless stated so.
|
