term treatment is HEART TRANSPLANTATION. The most
frequently occurring of these grave malformations
are
- Tetralogy of Fallot, which is a complex of four
structural anomalies: VSD, pulmonary artery
and valve malformation, aortic displacement
(the aorta arises between the ventricles rather
than solely from the left ventricle), and hyper-
trophic left ventricle (thickening of the left ven-
tricle’s wall). - Transposition of the great arteries (TGA), in
which the aorta and the pulmonary artery are
switched. The aorta arises from the right ventri-
cle instead of the left, carrying the deoxy-
genated blood from the right ventricle out to
the body. The pulmonary artery arises from the
left ventricle instead of the right, taking oxy-
genated blood back to the lungs from the left
ventricle. - Hypoplastic (or hypotrophic) left heart syn-
drome (HLHS), in which the left ventricle or
the entire left heart fails to develop, resulting in
essentially a two-chamber heart. The aorta is
usually small or deformed. Blood in the heart is
a mix of oxygenated and deoxygenated, and
the right ventricle pumps to both the lungs and
the body. - Persistent truncus arteriosus, which is a combi-
nation of VSD and deformities of the PULMONARY
ARTERIESand aorta that disrupts the heart’s abil-
ity to pump oxygenated blood to the body. - Anomalous pulmonary venous return, in
which the PULMONARY VEINSattach to the right
atrium instead of the left atrium, returning
oxygenated blood to the same chamber that
pumps deoxygenated blood to the lungs. This
malformation typically occurs in combination
with ASD, so the flow of blood between the
atria moves some oxygenated blood into the
left atrium and subsequently the left ventricle.
Often, ULTRASOUND during PREGNANCY reveals
these significant heart deformities, allowing the
neonatal team to be prepared for them at the
infant’s birth. In many situations initial treatment
includes administering PROSTAGLANDINSto maintain
a patent ductus arteriosus, which allows some
oxygenated blood into the body’s circulation.
Congenital heart disease in adults Some forms
of congenital heart disease first manifest in adult-
hood, such as hypertrophic cardiomyopathy and
LQTS. Other forms of heart disease in adults may
have congenital origins, such as the ARRHYTHMIA
disorder WOLFF-PARKINSON-WHITE SYNDROME and
some VALVULAR HEART DISEASE. Cardiologists believe
that most situations of SUDDEN CARDIAC DEATH
reflect undetected congenital heart anomalies,
either structural or functional (arrhythmias). With
congenital heart disease, whether undetected or
previously treated, comes increased risk for ENDO-
CARDITIS (especially with valve malformations),
arrhythmias, and clot formation leading to HEART
ATTACK, STROKE, or PULMONARY EMBOLISM.
A growing number of adults had corrective sur-
gery for congenital heart disease as infants or chil-
dren. Cardiologists do not yet know the long-term
effects of these operations or what precautions are
necessary to protect cardiovascular health later in
life. The generation born in the 1970s was the first
to have these options available. As this generation
comes into middle age, cardiologists will learn
much about how repaired hearts accommodate the
routine cardiovascular stresses of life and whether
they are more susceptible to acquired forms of
heart disease such as CORONARY ARTERY DISEASE(CAD)
andHEART FAILURE. At present, the longest survival
of infant heart transplantation is 15 years and of
adolescent heart transplantation is 16 years.
Rejection of the donor heart remains a significant
concern, and most cardiologists expect retransplan-
tation will become necessary for most people who
receive heart transplants in infancy or childhood.Symptoms and Diagnostic Path
The most common symptoms of congenital heart
disease, notably malformations of the heart, in
newborns is cyanosis and difficulty BREATHING.
Congenital heart disease not immediately appar-
ent at birth may manifest later in childhood with
symptoms such as fainting with physical exertion,
shortness of breath with mild activity, slowed
growth, rapid heartbeat and respirations, and fre-
quent upper respiratory infections. Young children
experiencing shortness of breath often squat,
which makes it easier for them to breathe.congenital heart disease 39