About Congenital Heart Defects
It can be frightening to learn that your child has a congenital
heart defect (one that's present at birth). But congenital
heart disease is a relatively common condition that affects almost
1 in every 100 newborns in the United States. Medical knowledge,
technology, and experience can offer accurate diagnosis and
treatments that allow nearly every form of congenital heart disease
to be treated.
Most defects today are treated with surgery, catheter
procedures, and sometimes medication. Thanks to advances in
techniques,
surgical
and heart catheterization procedures can treat congenital heart
defects that once could not be treated effectively.
Cardiac surgery and cardiac catheterizations are now being
performed on younger children - in fact, it's common for them
to be done during infancy or even the newborn period. This has
resulted in many long-term advantages for these children.
Although nothing can be guaranteed with 100% certainty, most
kids with heart problems can enjoy happy and healthy
futures.
Why Do Some Babies Have Congenital Heart Defects?
The human
heart
begins to form as a single tubular structure at about the fourth
week of
pregnancy
. By the eighth week, this tube will gradually increase in length,
eventually twisting upon itself. A wall, or septum, grows to divide
the upper (atrial) and lower (ventricular) chambers into left and
right sides. Four valves made of tissue develop, which will keep
blood moving forward through the cardiac chambers, lungs, and body
as the heart pumps.
Because the placenta (and not the fetus' lungs) does the
work of exchanging oxygen and carbon dioxide, it's possible for
even severe developmental abnormalities of the heart to exist
without causing difficulties for the fetus. Such abnormalities may
become important only after the fetus' circulation transitions
to the newborn state after birth (when the umbilical cord is
clamped at the time of delivery, the placenta is no longer involved
in the baby's circulation).
The newborn becomes dependent upon the lungs and circulatory
system for the oxygen and blood flow needed to survive outside of
the womb. The right side of the heart receives oxygen-poor blood
flowing back from the body and pumps it to the lungs, where the
circulating blood picks up more oxygen. The left side of the heart
receives oxygen-rich blood from the lungs and pumps it out to the
body.
Multiple
genetic
and environmental factors interact to alter the development of the
heart during the early stages of a fetus' development (the
first 8 to 9 weeks during pregnancy). Sometimes, the cause of a
congenital heart defect is known. Certain environmental exposures
during the first trimester of pregnancy may cause structural
abnormalities (including anticonvulsant medications such as
phenytoin, the dermatologic medication isotretinoin, or lithium
salts for manic-depressive illness). Uncontrolled diabetes, alcohol
or drug abuse, or exposure to industrial chemicals during pregnancy
can also increase the risk of congenital heart malformations. But
most of the time, the specific cause of congenital heart disease is
not known.
Over the past 25 years, advances in ultrasound imaging
techniques have led to the availability of sophisticated tools such
as fetal echocardiography, making it possible for many congenital
heart malformations to be diagnosed as early as the 12th to 20th
week of pregnancy. The use of such imaging has reassured many
parents-to-be that their baby's heart is normal. For others, it
has offered an opportunity to know long before the birth that
there's a malformation. This gives the family and doctors
the ability to make well-informed decisions about the best
treatment options.
Signs and Symptoms of Congenital Heart Disease
After birth, the first sign of congenital heart disease is often
the presence of a
heart murmur
. A murmur in itself is not a disease, but simply a sound. As the
heart pumps blood, it sometimes creates vibrations that are heard
through the doctor's stethoscope as a noise, or murmur.
Not all heart murmurs are signs of abnormalities - in fact,
heart murmurs usually don't indicate the presence of any
heart problem. Sometimes, a doctor can determine with the
stethoscope alone whether a particular murmur is a sign of heart
disease. In other cases, additional tests - such as chest X-rays,
electrocardiograms (EKGs)
, or echocardiograms - are performed to help determine the exact
nature of a murmur.
Although many children with minor forms of congenital heart
disease may not require any treatment, some can have serious
symptoms early on that will require medical or surgical treatment
within the first year of life. One such symptom can be breathing
difficulties from
lung
congestion. This is usually the result of excessive blood flow from
the left side to the right side of the heart through abnormal
connections between the two sides of the circulation, such as holes
in the heart (as in
ventricular septal defect
,
atrial septal defect
, atrioventricular canal, and patent ductus arteriosus).
Or the congestion could be the result of obstructions to blood
flow on the left side of the heart, resulting in a backup of blood
in the blood vessels returning blood from the lungs (such as in
aortic stenosis,
coarctation of the aorta
, and hypoplastic left heart syndrome). The shortness of breath in
these babies may interfere with their ability to feed and may
result in an inability to gain weight adequately. Such babies may
require medical treatment or a procedure such as surgery or cardiac
catheterization within the first weeks of life.
Other symptoms of congenital heart disease relate to an
inadequate amount of oxygen carried within the blood. These infants
usually appear to have blue skin, a condition called cyanosis. This
can be due to an obstruction of blood flow to the lungs (such as in
tricuspid atresia or pulmonary atresia) or due to a hole within the
heart that allows oxygen-poor blood to flow from the right to the
left side of the heart and out to the body (such as in total
anomalous pulmonary venous return or Ebstein's anomaly). It can
also be related to an abnormal positioning (transposition) of the
arteries leaving the heart. In any of these cases, less red
oxygenated blood comes from the lungs and more blue unoxygenated
blood is carried to the body, causing the blue skin color.
Treatment for Congenital Heart Defects
Many heart abnormalities (including patent ductus arteriosus,
ventricular septal defect, truncus arteriosus, atrioventricular
septal defect, tetralogy of Fallot, and transposition of the great
arteries) can be corrected with a single operation in early
infancy. More complex abnormalities (including hypoplastic left
heart syndrome and tricuspid atresia) may require a series of two
or three operations beginning in the newborn period and completed
at approximately 3 years of age. With most complex abnormalities,
the children spend the majority of their time in the care of their
parents at home, with occasional visits to the pediatric
cardiologist (a heart specialist) as well as to the child's
primary care doctor.
Less invasive procedures done in the cardiac catheterization
laboratory, rather than the operating room, may be used to treat
some conditions. Such treatments may include balloon angioplasty or
valvuloplasty to relieve an obstruction of a blood vessel (such as
in coarctation of the aorta) or a valve obstruction (such as in
pulmonary or aortic stenosis). In these procedures, a pediatric
cardiologist inserts a catheter, a thin plastic tube with a special
balloon attached, into a blood vessel. The balloon is then inflated
to stretch open the narrow area of the blood vessel or heart valve.
Another procedure called transcatheter device occlusion may be used
to close abnormal openings or holes within the heart or blood
vessels (such as in patent ductus arteriosus, atrial septal
defects, and ventricular septal defects) without requiring
surgery.
Some abnormalities, such as small- or moderate-sized ventricular
septal defects, may close or decrease in relative size as your
child
grows
. While waiting for the hole to close, the doctor may prescribe
medicines for your child, which some kids also need to take after
surgery.
Whether treated surgically or medically, your child will need to
regularly visit a pediatric cardiologist. At first, these
appointments may be fairly frequent (perhaps every month or two),
but after treatment, they may be cut back, sometimes to just once a
year. Your child's cardiologist may use tools like X-rays,
electrocardiograms, or echocardiograms to monitor the defect and
the effects of treatment.
Preventing Infection
Kids with congenital heart disease are at risk for the
development of bacterial endocarditis, an infection of the tissue
that lines the heart and blood vessels. This serious illness
requires prolonged treatment with intravenous antibiotics in a
hospital setting.
Any time a child has a surgical procedure, the surgical incision
can introduce
bacteria
into the bloodstream. Although the white blood cells of the body
usually destroy these germs before an infection can occur, the
rough surfaces that may be present within a congenitally malformed
heart may allow some germs to survive and reproduce, resulting in
an infection of the heart lining.
Fortunately, the risks of bacterial endocarditis can be greatly
reduced by taking a dose of specific antibiotics before any
scheduled medical procedures that have a risk for introducing germs
into the bloodstream. This includes dental work and certain types
of surgery.
However, some parents misinterpret this to mean that
dental visits
and cleanings are potentially dangerous and that they can avoid
risk by avoiding the dentist. This is incorrect! In fact, the
riskiest thing to do is to ignore dental health, which may allow
teeth to develop cavities and gums to become infected. Along with
taking antibiotics correctly, it's important for children with
heart defects to take good care of their teeth by brushing and
flossing properly. Your child should begin visiting a dentist as
early as possible, and those visits should be as frequent as the
dentist recommends.
Taking measures to prevent bacterial endocarditis is recommended
for kids with almost all congenital cardiac malformations (except
in the case of isolated ostium secundum and atrial septal defect).
Discuss these preventive measures with your child's doctor,
pediatric cardiologist, and dentist. Local chapters of the American
Heart Association (AHA) or your pediatric cardiologist can give you
free wallet cards detailing the recommended antibiotics and their
appropriate doses.
If You Suspect a Problem
Although sudden serious downturns during or after cardiac
treatment aren't common, you should watch for certain signs
that could signal a need for medical attention. If your child
appears to be working harder than normal to breathe, call your
child's doctor right away.
Other signs that warrant immediate medical attention
include:
- a bluish tinge or color (cyanosis) to the skin around the
mouth or on the lips and tongue
- an increased rate of breathing or difficulty breathing
- poor appetite or difficulty feeding (which may be associated
with color change)
- sweating while feeding
-
failure to thrive
(failure to gain weight or weight loss)
- decreased energy or activity level
- prolonged or unexplained fever
Call the doctor immediately if your child has any of these
symptoms.
Caring for Your Child
Parenting kids with heart defects includes learning about basics
like feeding, giving medicines, and watching for signs of trouble,
but it also involves encouraging kids to become involved in their
own care.
Because most congenital heart defects are now treated during
infancy, it's often necessary to explain to an older child what
happened in the past. When your child is old enough to understand,
explain why he or she has a surgical scar, needs to take
medication, or needs to visit the pediatric cardiologist. Describe
the treatment in a way your child can understand and don't try
to hide the details.
If kids believe they have a role in their care, they're
likely to be more confident and positive. Your doctor may be able
to suggest ways to discuss these issues.
Participation in some physical activities may be limited, but
kids can still play and explore with friends. Always check with
your child's cardiologist about which activities your child
should or should not be doing. Certain competitive sports may be
restricted, for example.
Although it's tempting for parents to be overly protective,
sheltering kids can make them feel isolated and stigmatized - which
may do more harm than a heart defect in the long run. So do
everything you can to make sure your child leads as normal a life
as possible.
Reviewed by:
Steven Dowshen, MD
Date reviewed: November 2007
Originally reviewed by:
Terrence U. H. Chun, MD
Note: All information is for educational purposes only. For specific medical advice,
diagnoses, and treatment, consult your doctor.
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