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This timeline pinpoints various developments in nursing informatics. The acronyms are defined
below the diagram.
Acronym Definition
PROMIS Problem Oriented Medical Information System
UG Undergrad
UB University of Buffalo
Computers in Nursing (a journal now named CIN: Computers, Informatics,
CIN
Nursing
NIH National Institute of Health
SCAMC Symposium on Computer Applications in Medical Care
UMAB Univesity of Maryland at Baltimore
NI Nursing Informatics
NIS Nursing Information System
HIPAA Health Information Portability and Accountabilty Act
1982 The price of PCs drops as"clones" appear on the scene. The first International Medical
Although the term clone is used, these computers were Informatics Association Working
not an exact duplicate of the IBM PC. In computers, the Conference on the Impact of
term clone means that a computer allows one to run the Computers on Nursing is held in
same programs, or software. For the first 10 years after London, England. This was
the introduction of the IBM PC these clones were followed in 1985 by another
generally referred to as being "IBM compatible." Later international nursing informatics
the term heard more often was MS-DOS™ computers. conference in Calgary, Alberta,
Today the terms used is generally a PC or Windows Canada. These are now held
Computers meaning they run an operating system every three years
produced by Microsoft™.
Mosby publishes
the first edition of
the Nursing
Outcomes
Classifications
(NOC), a system
still in use.
Work begins on
the Patient Care
Data Set at the
University of
Virginia. Still in
use.
1995 Intel announces the Pentium Pro Keeping costs
microprocessor, if numbered it would be down still prevails,
a 686. The processor uses 5.5 million but focus now
transistors. moves to
improving patient
outcomes.
Capturing data at
the point of care
becomes
important (Guide
to Effective Health
Care Clinical
Systems, 1996).
To operationalize
point of care data
capture PDAs
start to appear in
hospitals.
Acccoring to Sackett and Erdley (2002), a Russian Scientist in 1966 coined the term informatika"
and defined it as "The discipline of science which investigates the structures and properties (not
specific content) of scientific information..." (Collen as cited in Sackett & Erdley).
A Frenchman, in the 1970's, Francois Gremy is credited with coining the term informatique
medical, translated to medical informatics (Hannah, Ball & Edwards, 1999). It was defined as the
informational technologies which are concerned with patient care and the medical decision
making process. Another definition stated that medical informatics is the complex data
processing by the computer to create new information.
The term "nursing informatics," was probably first used and defined by Scholes and Barber in
1980 in their address to the MEDINFO conference that year in Tokyo.
Health-care Informatics, however, is truly interdisciplinary. In its truest form it focuses on the care
of the patient, not a specific discipline (Hannah, Ball & Edwards, 1999). Thus, although there are
specific bodies of knowledge for each health care profession, nursing, dentistry, dietetics,
pharmacy, medicine, etc., they interface at the patient.
"...a specialty that integrates nursing science, computer science, and information science in
identifying, collecting, processing, and managing data and information to support nursing
practice, administration, education, and research; and to expand nursing knowledge. The
purpose of nursing informatics is to: analyze information requirements; design, implement and
evaluate information systems and data structures that support nursing; and identify and apply
computer technologies for nursing."
The final master list of valid competencies for the four levels of nursing practice is as
follows:
Uses applications for structured data entry (e.g., patient acuity or classification
applications)
Computer Skills-Monitoring
Uses computerized patient monitoring systems
Is able to navigate Windows (e.g., manipulate files using file manager, determine active
printer, access installed applications, create & delete directories)
Identifies the appropriate technology to capture the required patient data (e.g., fetal
monitoring device)
Demonstrates basic technology skills (e.g., turn computer off & on, load paper, change
toner, remove paper jams, print documents)
Truncus Arteriosus
What It Is
Truncus arteriosus is characterized by a large ventricular septal defect over which a large, single
great vessel (truncus) arises. This single great vessel carries blood both to the body and to the
lungs.
Surgical Treatment
Surgery is required to close the ventricular septal defect and separate blood flow to the body from blood flow
to the lungs. This is generally done early in infancy to prevent high blood pressure from damaging the lungs'
arteries. A patch is used to close the ventricular defect. The pulmonary arteries are then disconnected from
the single great vessel and a tube (a conduit or tunnel) is placed from the right ventricle to these pulmonary
arteries. This is sometimes called a Rastelli repair.
Ongoing Care
Medical
Patients with truncus arteriosus need regular follow-up with a pediatric or adult cardiologist with special
training in congenital heart disease. You may need to take medicine after your operation to help your heart
pump better. Your cardiologist will track you with a variety of tests. These include electrocardiograms, Holter
monitors, exercise stress tests and echocardiograms (see the Glossary) to determine when another
procedure such as cardiac catheterization may be needed.
Level 2 Experienced Nurse
Computer Skills-Monitoring
Applies monitoring system appropriately according to the data needed
Computer Skills-Research
Uses computer applications for statistical analysis and nursing research
Informatics Knowledge-Data
Informatics Knowledge-Education
Implements and evaluates application/system training programs for users and clients
Plans and develops application/system training programs for users, clients
Constructs guidelines for the purchase of software and hardware
Participates with practicing nurses, nurse administrators, and nurse researchers to define
and develop new computer competencies
Teaches users/clients about effective and ethical uses of applications and systems
Informatics Knowledge-Impact
Assesses current capabilities and limitations of technology (e.g., data transfer rates, chip
capacity)
Determines projected impacts to users and organizations when changing to
computerized information management
Determines the reasons for slow response time (e.g., heavy demands on computer
system at time of shift change
Discusses new careers available to informaticists
Determines the social, legal, and ethical impacts of changing to computerized information
management
Discusses the interdependencies computerized information management creates (e.g.,
changes when MD enters own orders)
Determines the limitations, reliability of computerized patient monitoring systems
Level 3 Informatics Specialist, cont.
Applies strategies for change management to produce satisfied and productive users
Informatics Knowledge-Usability
Analyzes the health and safety aspects of the work station and its location
Applies human factors and ergonomics to the design of the computer screen, location
and design of devices, and design of software
Informatics Skills-Analysis
Develops and implements work plans during application development and
implementation
Analyzes business practices to determine need for reengineering the information flow
Applies principles of computing (e.g., reading an algorithm)
Analyzes user areas to determine procedural errors versus hardware and software
problems
Interprets information flow within the organization
Develops procedures to establish and maintain the validity and integrity of data and
databases
Develops screen layouts, report formats and custom views of clinical data working
directly with clinical departments and individual users
Maintains database (e.g., adding, deleting fields, structuring input for others, relational
database)
Incorporates established data and database management standards into database
design Participates in the development of new tools for management purposes
Develops methods of data communication, hardware and software integration, and data
transformation
Applies skills in the systems life cycle to support all computer-enabled patient care
activities.
Determines project scope, objectives, and resources for each proposed application,
system or enhancement
Develops policies related to privacy, confidentiality and security of patient and client data
Recommends procedures for achieving data integrity and security
Analyzes the capability of information technology to support programs of data integrity
and security
Demonstrates skills in the systems life cycle to support policies, procedures and
knowledge bases in organizations
Informatics Skills-Role
Influences change to improve the impact of informatics on the system of care
Integrates knowledge from other informatics disciplines with nursing to improve patient
care, administration, education and/or research
Participates in top level decisions and policy design which impact clinical information
management
Informatics Skills-Testing
Develops procedures and scenarios for acceptance testing, conversions, and interface
testing
Informatics Skills-Training
Computer Skills-Simulation
Evaluates factors related to safety, effectiveness, cost and social impact when
developing and implementing information management technologies
Based upon information management technologies evaluation data, recommends and/or
modifies clinical practice enhancements
Evaluates the performance and impact of information management technologies on
clinical practice, education, administration &/or research
Develops a framework(s) for evaluating applications and system performance in clinical
care, education, research, and/or administration
Research
Develops innovative and analytic techniques for scientific inquiry in nursing informatics
Practice
Applies advanced analysis and design concepts to the system life cycle process
Integrates domain knowledge within computerized decision support systems
Analyzes complex issues (e.g., confidentiality, privacy, and data security)
Recommends policies based upon analytical findings
Designs and/or evaluates enterprise-wide strategies for managing the impact of
information systems implementation
Designs the structure for complex data sets
Develops new methods of organizing data to enhance research capabilities
Develops innovative methods of data communication, hardware and software integration,
and data transformation
Designs unique system alternatives for clinical care, education, administration or
research
Exerts leadership of interdisciplinary teams to provide strategic IS direction
Influences top-level decisions and policy design which impact clinical information
management
Education
Applies sophisticated educational design and research evaluation concepts to the use of
innovative computer-based education techniques (e.g., distance education)
Develops theoretically-based curricular models for nursing informatics
Other Results
The competency “writes an original computer program and modifies it” was rejected early
during round 1. The other 4 competencies did not reach consensus (an 80% agreement) to
Six competencies were valid competencies, but no agreement on a practice level was reached. One
of these 6 competencies achieved the 80% threshold of agreement that it was at the wrong level, but
still did not reach agreement on a correct level. This competency was “uses authoring tools to develop
CAI for students, nurses and/or patients.” It was originally at the experienced nurse level; 77% of the
respondents placed this competency at the informatics specialist level. The other five valid
Recognizes computerized diagnosis equipment (e.g., CAT scan, MRI, digital imaging)
(originally at the informatics specialist level)
Conducts research to examine impacts of computer technology in nursing (originally at
the informatics specialist level)
Conducts research in informatics (originally at the informatics specialist level)
Teaches informatics competencies required for specific role functions for the practicing
nurse, the nurse administrator (originally at the nurse innovator level)
There were no competencies that changed levels of practice as a result of the Delphi
study.
The results of this Delphi study created a master list of informatics competencies for
nurses at four levels of practice. Please see the full length article in Nursing Research for the
Truncus Arteriosus
What It Is
Truncus arteriosus is characterized by a large ventricular septal defect over which a large, single great vessel (truncus)
arises. This single great vessel carries blood both to the body and to the lungs.
Surgical Treatment
Surgery is required to close the ventricular septal defect and separate blood flow to the body from blood flow to the lungs. This is
generally done early in infancy to prevent high blood pressure from damaging the lungs' arteries. A patch is used to close the
ventricular defect. The pulmonary arteries are then disconnected from the single great vessel and a tube (a conduit or tunnel) is
placed from the right ventricle to these pulmonary arteries. This is sometimes called a Rastelli repair.
Ongoing Care
Medical
Patients with truncus arteriosus need regular follow-up with a pediatric or adult cardiologist with special training in congenital heart
disease. You may need to take medicine after your operation to help your heart pump better. Your cardiologist will track you with a
variety of tests. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms (see the Glossary)
to determine when another procedure such as cardiac catheterization may be needed.
Tetralogy of Fallot
(Note: It will be helpful to review normal heart function before reading this.)
The tetralogy of Fallot has four components. The first major one is a ventricular septal defect. This
is a large hole between the two ventricles that lets venous (bluish) blood pass from the right
ventricle to the left one. From there it goes to the aorta and on to the body without passing through
the lungs to be refreshed with oxygen.
The second major component of tetralogy of Fallot is a stenosis (narrowing) at, or just beneath, the
pulmonary valve. The narrowing partly blocks the flow of venous blood into the lungs. This varies in severity
from child to child.
The last two components of tetralogy of Fallot are: (1) the right ventricle is more muscular than normal; (2)
and the aorta lies directly over the ventricular septal defect. This results in blueness (cyanosis), which may
appear soon after birth, in infancy or later in childhood. These "blue babies" may have sudden episodes of
severe cyanosis with rapid breathing. They may even become unconscious. During exercise, older children
may become short of breath and have fainting spells. These symptoms happen because not enough blood
flows to the lungs to supply the child's body with oxygen. Some infants with severe tetralogy of Fallot may
need an operation that will give temporary relief by increasing blood flow to the lungs with a shunt. This
procedure is done by making a connection between the aorta and the pulmonary artery. Thus, some blood
from the aorta flows into the lungs to get more oxygen. This reduces the cyanosis and allows the child to
grow and develop until the repair can be done when the child is older.
Most children with tetralogy of Fallot have open-heart surgery before school age. The operation involves
closing the ventricular septal defect and removing the obstructing muscle. If the pulmonary valve is narrow,
it's opened; and if it's small, a graft or patch may be needed to finish the repair. After surgery, the long-term
outlook varies a great deal. Usually it's quite good, but it depends largely on how severe the defects were
before surgery -- especially the amount of pulmonary narrowing.
Lifelong follow-up is needed to be sure that any remaining defects or problems are treated properly. Children
with tetralogy of Fallot risk an infection of the heart's walls or valves (endocarditis) before and after surgery.
Please see our section on “Endocarditis” to determine whether your child will need to take antibiotics before
certain dental procedures. Good dental hygiene also lowers the risk of endocarditis. For more information
about dental hygiene and preventing endocarditis, ask your pediatric cardiologist.
Congenital means inborn or existing at birth. Among the terms you may hear are congenital heart defect, congenital heart
disease and congenital cardiovascular disease. The word "defect" is more accurate than "disease." A congenital cardiovascular
defect occurs when the heart or blood vessels near the heart don't develop normally before birth.
Congenital cardiovascular defects are present in about 1 percent of live births. They're the most common congenital
malformations in newborns. In most cases scientists don't know why they occur. Sometimes a viral infection causes serious
problems. German measles (rubella) is an example. If a woman contracts German measles while pregnant, it can interfere with
how her baby's heart develops or produce other malformations. Other viral diseases also may cause congenital defects.
Heredity sometimes plays a role in congenital cardiovascular defects. More than one child in a family may have a congenital
cardiovascular defect, but this rarely occurs. Certain conditions affecting multiple organs, such as Down's syndrome, can involve
the heart, too. Some prescription drugs and over-the-counter medicines, as well as alcohol and "street" drugs, may increase the
risk of having a baby with a heart defect. Researchers are studying other factors.
Most heart defects either obstruct blood flow in the heart or vessels near it, or cause blood to flow through the heart in an
abnormal pattern. Rarely defects occur in which only one ventricle (single ventricle) is present, or both the pulmonary artery and
aorta arise from the same ventricle (double outlet ventricle). A third rare defect occurs when the right or left side of the heart is
incompletely formed — hypoplastic heart.
The following defects are described in this section: (For information on congenital defects in Spanish, see the Web
site www.americanheart.org/Spanish/index.html)
This defect (PA'tent DUK'tus ar-te"re-O'sis) allows blood to mix between the pulmonary artery and the aorta. Before birth an open
passageway (the ductus arteriosus) exists between these two blood vessels. Normally this closes within a few hours of birth.
When this doesn't happen, some blood that should flow through the aorta and on to nourish the body returns to the lungs. A
ductus that doesn't close is quite common in premature infants but rather rare in full-term babies.
If the ductus arteriosus is large, a child may tire quickly, grow slowly, catch pneumonia easily and breathe rapidly. In some
children symptoms may not occur until after the first weeks or months of life. If the ductus arteriosus is small, the child seems
well. If surgery is needed, the surgeon can close the ductus arteriosus by tying it, without opening the heart. If there's no other
defect, this restores the circulation to normal.
Obstruction defects
An obstruction is a narrowing that partly or completely blocks the flow of blood. Obstructions called stenoses (sten-O'seez) can
occur in heart valves, arteries or veins.
The three most common forms are pulmonary stenosis, aortic stenosis and coarctation of the aorta. Related but less common
forms include bicuspid aortic valve, subaortic stenosis and Ebstein's anomaly.
• Pulmonary stenosis (PUL'mo-nair-e sten-O'sis) (PS) — The pulmonary or pulmonic valve is between the right
ventricle and the pulmonary artery. It opens to allow blood to flow from the right ventricle to the lungs. A defective
pulmonary valve that doesn't open properly is called stenotic (sten-OT'ik). This forces the right ventricle to pump
harder than normal to overcome the obstruction.
If the stenosis is severe, especially in babies, some cyanosis (si"ah-NO'sis) (blueness) may occur. Older children
usually have no symptoms. Treatment is needed when the pressure in the right ventricle is higher than normal. In
most children the obstruction can be relieved by a procedure called balloon valvuloplasty (VAL'vu-lo-plas-te). Others
may need open-heart surgery. Surgery usually opens the valve satisfactorily. The outlook after balloon valvuloplasty
or surgery is favorable, but follow-up is required to determine if heart function returns to normal.
People with pulmonary stenosis, before and after treatment, are at risk for getting an infection of the valve
(endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take
antibiotics before certain dental procedures.
• Aortic stenosis (a-OR'tik sten-O'sis) (AS) — The aortic valve, between the left ventricle and the aorta, is narrowed.
The heart has difficulty pumping blood to the body. Aortic stenosis occurs when the aortic valve didn't form properly. A
normal valve has three leaflets (cusps) but a stenotic (sten-OT'ik) valve may have only one cusp (unicuspid) or two
cusps (bicuspid), which are thick and stiff. (See bicuspid aortic valve below.)
Sometimes stenosis is severe and symptoms occur in infancy. Otherwise, most children with aortic stenosis have no
symptoms. Some children may have chest pain, unusual tiring, dizziness or fainting. The need for surgery depends on
how bad the stenosis is. In children, a surgeon may be able to enlarge the valve opening. Surgery may improve the
stenosis, but the valve remains deformed. Eventually the valve may need to be replaced with an artificial one.
Balloon valvuloplasty (VAL'vu-lo-plas-te) has been used in some children with aortic stenosis. The long-term results of
this procedure are still being studied. Children with aortic stenosis need lifelong medical follow-up. Even mild stenosis
may worsen over time, and surgical relief of a blockage is sometimes incomplete. Check with your pediatric
cardiologist about limiting some kinds of exercise.
People with aortic stenosis, before and after treatment, are at risk for getting an infection of the valve
(endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take
antibiotics before certain dental procedures.
• Coarctation (ko"ark-TA'shun) of the aorta ("Coarct") — The aorta is pinched or constricted. This obstructs blood
flow to the lower body and increases blood pressure above the constriction. Usually there are no symptoms at birth,
but they can develop as early as a baby's first week. A baby may develop congestive heart failure or high blood
pressure that requires early surgery. Otherwise, surgery usually can be delayed. A child with a severe coarctation
should have surgery in early childhood. This prevents problems such as developing high blood pressure as an adult.
The outlook after surgery is favorable, but long-term follow-up is required. Rarely, coarctation of the aorta may recur.
Some of these cases can be treated by balloon angioplasty. The long-term results are still being studied. Also, blood
pressure may stay high even when the aorta's narrowing has been repaired.
People with coarctation of the aorta, before and after treatment, are at risk for getting an infection within the aorta or the
heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need
to take antibiotics before certain dental procedures.
• Bicuspid aortic (bi-KUS'pid a-OR'tik) valve — The normal aortic valve has three flaps (cusps) that open and close. A
bicuspid valve has only two flaps. There may be no symptoms in childhood, but by adulthood (often middle age or
older), the valve can become stenotic (sten-OT'ik) (narrowed), making it harder for blood to pass through it, or
regurgitant (allowing blood to leak backward through it). Treatment depends on how well the valve works.
People with bicuspid aortic valve, before and after treatment, are at risk for getting an infection within the aorta or the
heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need
to take antibiotics before certain dental procedures.
• Subaortic stenosis (sub"a-OR'tik sten-O'sis) — Stenosis means constriction or narrowing. Subaortic means below
the aorta. Subaortic stenosis refers to a narrowing of the left ventricle just below the aortic valve, which blood passes
through to go into the aorta. This stenosis limits the flow of blood out of the left ventricle. This condition may be
congenital or may be due to a particular form of cardiomyopathy (kar"de-o-mi-OP'ah-the) known as "idiopathic
hypertrophic (hi"per-TRO'fik) subaortic stenosis" (IHSS). Treatment depends on the cause and the severity of the
narrowing. It can include drugs or surgery.
People with subaortic stenosis, before and after treatment, are at risk for getting an infection within the aorta or the
heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need
to take antibiotics before certain dental procedures.
• Ebstein's anomaly (ah-NOM'ah-lee) is a congenital downward displacement of the tricuspid valve (located between
the heart's upper and lower chambers on the right side) into the heart's right bottom chamber (right ventricle). It's
usually associated with an atrial septal defect (see below).
People with Ebstein's anomaly, before and after treatment, are at risk for getting an infection within the heart valve
(endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take
antibiotics before certain dental procedures.
Septal defects
Some congenital cardiovascular defects let blood flow between the heart's right and left chambers. This happens when a baby is
born with an opening between the wall (septum) that separates the right and left sides of the heart. This defect is sometimes
called "a hole in the heart."
The two most common types of this defect are atrial septal defect and ventricular septal defect. Two variations are Eisenmenger's
complex and atrioventricular canal defect.
• Atrial septal (A'tre-al SEP'tal) defect (ASD) — An opening exists between the heart's two upper chambers. This lets
some blood from the left atrium (blood that's already been to the lungs) return via the hole to the right atrium instead of
flowing through the left ventricle, out the aorta and to the body. Many children with ASD have few, if any, symptoms.
Closing the atrial defect by open-heart surgery in childhood can prevent serious problems later in life.
• Ventricular septal (ven-TRIK'u-ler SEP'tal) defect (VSD) — An opening exists between the heart's two lower
chambers. Some blood that's returned from the lungs and been pumped into the left ventricle flows to the right
ventricle through the hole instead of being pumped into the aorta. Because the heart has to pump extra blood and is
overworked, it may enlarge.
If the opening is small, it doesn't strain the heart. In that case, the only abnormal finding is a loud murmur. But if the
opening is large, open-heart surgery is recommended to close the hole and prevent serious problems. Some babies
with a large ventricular septal defect don't grow normally and may become undernourished. Babies with VSD may
develop severe symptoms or high blood pressure in their lungs. Repairing a ventricular septal defect with surgery
usually restores normal blood circulation. The long-term outlook is good, but long-term follow-up is required.
People with unrepaired ventricular septal defects are at risk for getting an infection of the heart's walls or valves
(endocarditis). After a VSD has been successfully fixed with surgery, antibiotics should no longer be needed. If
surgery or other procedures do not completely repair the VSD, the person is still at risk for developing endocarditis.
Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before
certain dental procedures.
• Eisenmenger's complex is a ventricular septal defect coupled with pulmonary high blood pressure, the passage of
blood from the right side of the heart to the left (right to left shunt), an enlarged right ventricle and a latent or clearly
visible bluish discoloration of the skin called cyanosis (si"ah-NO'sis). It may also include a malpositioned aorta that
receives ejected blood from both the right and left ventricles (an overriding aorta).
People with Eisenmenger's complex, before and after treatment, are at risk for getting an infection within the aorta or
the heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will
need to take antibiotics before certain dental procedures.
• Atrioventricular (A'tre-o-ven-TRIK'u-ler) (A-V) canal defect (also called endocardial cushion defect or
atrioventricular septal defect) — A large hole in the center of the heart exists where the wall between the upper
chambers joins the wall between the lower chambers. Also, the tricuspid and mitral valves that normally separate the
heart's upper and lower chambers aren't formed as individual valves. Instead, a single large valve forms that crosses
the defect. The large opening in the center of the heart lets oxygen-rich (red) blood from the heart's left side – blood
that's just gone through the lungs – pass into the heart's right side. There, the oxygen-rich blood, along with venous
(bluish) blood from the body, is sent back to the lungs. The heart must pump an extra amount of blood and may
enlarge. Most babies with an atrioventricular canal don't grow normally and may become undernourished. Because of
the large amount of blood flowing to the lungs, high blood pressure may occur there and damage the blood vessels.
In some babies the common valve between the upper and lower chambers doesn't close properly. This lets blood leak
backward from the heart's lower chambers to the upper ones. This leak, called regurgitation or insufficiency, can occur
on the right side, left side or both sides of the heart. With a valve leak, the heart pumps an extra amount of blood,
becomes overworked and enlarges.
In babies with severe symptoms or high blood pressure in the lungs, surgery usually must be done in infancy. The
surgeon closes the large hole with one or two patches and divides the single valve between the heart's upper and
lower chambers to make two separate valves. Surgical repair of an atrioventricular canal usually restores the blood
circulation to normal. However, the reconstructed valve may not work normally.
Rarely, the defect may be too complex to repair in infancy. In this case, the surgeon may do a procedure called
pulmonary artery banding to reduce the blood flow and high pressure in the lungs. When a child is older, the band is
removed and corrective surgery is done. More medical or surgical treatment is sometimes needed.
People with atrioventricular canal defect, before and after treatment, are at risk for getting an infection within the heart's
walls or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will
need to take antibiotics before certain dental procedures.
Cyanotic defects
Another type of heart defect is congenital cyanotic (si"ah-NOT'ik) heart defects. In these defects, blood pumped to the body
contains less oxygen than normal. This causes a condition called cyanosis (si"ah-NO'sis), a blue discoloration of the skin. Infants
with cyanosis are often called "blue babies."
Examples of cyanotic defects are tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, pulmonary atresia,
truncus arteriosus and total anomalous pulmonary venous connection.
• Tetralogy of Fallot (TE'TRAL'o-je of fal-O') has four components. The two major ones are a large hole, or ventricular
septal defect, that lets blood pass from the right to the left ventricle without going through the lungs; and a narrowing
(stenosis) at or just beneath the pulmonary valve. This narrowing partially blocks the blood flow from the heart's right
side to the lungs. The other two components are: the right ventricle is more muscular than normal; and the aorta lies
directly over the ventricular septal defect.
This results in cyanosis (blueness), which may appear soon after birth, in infancy or later in childhood. These "blue
babies" may have sudden episodes of severe cyanosis with rapid breathing. They may even become unconscious.
During exercise, older children may become short of breath and faint. These symptoms occur because not enough
blood flows to the lungs to supply the child's body with oxygen.
Some infants with severe tetralogy of Fallot may need an operation to give temporary relief by increasing blood flow to
the lungs with a shunt. This is done by making a connection between the aorta and the pulmonary artery. Then some
blood from the aorta flows into the lungs to get more oxygen. This reduces the cyanosis and allows the child to grow
and develop until the problem can be fixed when they are older.
Most children with tetralogy of Fallot have open-heart surgery before school age. The operation involves closing the
ventricular septal defect and removing the obstructing muscle. After surgery the long-term outlook varies, depending
largely on how severe the defects were before surgery. Lifelong medical follow-up is needed.
People with tetralogy of Fallot, before and after treatment, are at risk for getting an infection within the aorta or the heart
valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take
antibiotics before certain dental procedures.
• Transposition of the great arteries — The positions of the pulmonary artery and the aorta are reversed. The aorta is
connected to the right ventricle, so most of the blood returning to the heart from the body is pumped back out without
first going to the lungs. The pulmonary artery is connected to the left ventricle, so most of the blood returning from the
lungs goes back to the lungs again.
Infants born with transposition survive only if they have one or more connections that let oxygen-rich blood reach the
body. One such connection may be a hole between the two atria, called atrial septal defect, or between the two
ventricles, called ventricular (ven-TRIK'u-ler) septal defect. Another may be a vessel connecting the pulmonary artery
with the aorta, called patent ductus arteriosus (PA'tent DUK'tus ar-te"re-O'sis). Most babies with transposition of the
great arteries are extremely blue (cyanotic) (si"ah-NOT'ik) soon after birth because these connections are inadequate.
To improve the body's oxygen supply, a special procedure called balloon atrial septostomy (sep-TOS'to-me) is used.
Two general types of surgery may be used to help fix the transposition. One is a venous switch or intra-atrial baffle
procedure that creates a tunnel inside the atria. Another is an arterial switch. After surgery, the long-term outlook
varies quite a bit. It depends largely on how severe the defects were before surgery. Lifelong follow-up is needed.
People with transposition of the great arteries, before and after treatment, are at risk for getting an infection on the
heart's walls or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child
will need to take antibiotics before certain dental procedures.
• Tricuspid atresia (tri-KUS'pid ah-TRE'zhuh) — In this condition, there's no tricuspid valve. That means no blood can
flow from the right atrium to the right ventricle. As a result, the right ventricle is small and not fully developed. The
child's survival depends on there being an opening in the wall between the atria called an atrial septal defect and
usually an opening in the wall between the two ventricles called a ventricular (ven-TRIK'u-ler) septal defect. Because
the circulation is abnormal, the blood can't get enough oxygen, and the child looks blue (cyanotic) (si"ah-NOT'ik).
Often a surgical shunting procedure is needed to increase blood flow to the lungs. This reduces the cyanosis. Some
children with tricuspid atresia have too much blood flowing to the lungs. They may need a procedure (pulmonary artery
banding) to reduce blood flow to the lungs. Other children with tricuspid atresia may have a more functional repair
(Fontan procedure). Children with tricuspid atresia require lifelong follow-up by a cardiologist.
People with tricuspid atresia, before and after treatment, are at risk for getting an infection of the valves (endocarditis).
Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before
certain dental procedures.
• Pulmonary atresia (PUL'mo-nair-e ah-TRE'zhuh) — No pulmonary valve exists, so blood can't flow from the right
ventricle into the pulmonary artery and on to the lungs. The right ventricle acts as a blind pouch that may stay small
and not well developed. The tricuspid valve is often poorly developed, too.
An opening in the atrial septum lets blood exit the right atrium, so venous (bluish) blood mixes with the oxygen-rich
(red) blood in the left atrium. The left ventricle pumps this mixture of oxygen-poor blood into the aorta and out to the
body. The baby appears blue (cyanotic) (si"ah-NOT'ik) because there's less oxygen in the blood circulating through the
arteries. The only source of lung blood flow is the patent ductus arteriosus (PA'tent DUK'tus ar-te"re-O'sis) (PDA), an
open passageway between the pulmonary artery and the aorta. If the PDA narrows or closes, the lung blood flow is
reduced to critically low levels. This can cause very severe cyanosis.
Early treatment often includes using a drug to keep the PDA from closing. A surgeon can create a shunt between the
aorta and the pulmonary artery to help increase blood flow to the lungs. A more complete repair depends on the size
of the pulmonary artery and right ventricle. If they're very small, it may not be possible to correct the defect with
surgery. In cases where the pulmonary artery and right ventricle are a more normal size, open-heart surgery may
produce a good improvement in how the heart works.
If the right ventricle stays too small to be a good pumping chamber, the surgeon can compensate by connecting the
right atrium directly to the pulmonary artery. The atrial defect also can be closed to relieve the cyanosis. This is called
a Fontan procedure. Children with tricuspid atresia require lifelong follow-up by a cardiologist.
People with pulmonary atresia, before and after treatment, are at risk for getting an infection on the heart's walls or
valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take
antibiotics before certain dental procedures.
• Truncus arteriosus (TRUN'kus ar-te"re-O'sis) — This is a complex malformation where only one artery arises from the
heart and forms the aorta and pulmonary artery. Surgery for this condition usually is required early in life. It includes
closing a large ventricular (ven-TRIK'u-ler) septal defect within the heart, detaching the pulmonary arteries from the
large common artery, and connecting the pulmonary arteries to the right ventricle with a tube graft. Children with
truncus arteriosus need lifelong follow-up to see how well the heart is working.
People with truncus arteriosus, before and after treatment, are at risk for getting an infection on the heart's walls or
valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take
antibiotics before certain dental procedures.
• Total anomalous pulmonary venous (ah-NOM'ah-lus PUL'mo-nair-e VE'nus) (P-V) connection —The pulmonary
veins that bring oxygen-rich (red) blood from the lungs back to the heart aren't connected to the left atrium. Instead,
the pulmonary veins drain through abnormal connections to the right atrium.
In the right atrium, oxygen-rich (red) blood from the pulmonary veins mixes with venous (bluish) blood from the body.
Part of this mixture passes through the atrial septum (atrial septal defect) into the left atrium. From there it goes into
the left ventricle, to the aorta and out to the body. The rest of the poorly oxygenated mixture flows through the right
ventricle, into the pulmonary artery and on to the lungs. The blood passing through the aorta to the body doesn't have
enough oxygen, which causes the child to look blue (cyanotic) (si"ah-NOT'ik).
This defect must be surgically repaired in early infancy. The pulmonary veins are reconnected to the left atrium and the
atrial septal defect is closed. When surgical repair is done in early infancy, the long-term outlook is very good. Still,
lifelong follow-up is needed to make sure that any remaining problems, such as an obstruction in the pulmonary veins
or irregularities in heart rhythm, are treated properly. It's important to make certain that a blockage doesn't develop in
the pulmonary veins or where they're attached to the left atrium. Heart rhythm irregularities (arrhythmias) also may
occur at any time after surgery.
People with total anomalous pulmonary venous connection, before and after treatment, are at risk for getting an
infection on the heart's walls or valves (endocarditis). Please see the section on “Endocarditis” below to determine
whether your child will need to take antibiotics before certain dental procedures.
In hypoplastic (hi"po-PLAS'tik) left heart syndrome, the left side of the heart is underdeveloped – including the aorta, aortic valve,
left ventricle and mitral valve. Blood returning from the lungs must flow through an opening in the wall between the atria, called an
atrial septal defect. The right ventricle pumps the blood into the pulmonary artery, and blood reaches the aorta through a patent
ductus arteriosus (PA'tent DUK'tus ar-te"re-O'sis). (See above.)
The baby often seems normal at birth, but will come to medical attention within a few days as the ductus closes. Babies with this
syndrome become ashen, have rapid and difficult breathing and have difficulty feeding. This heart defect is usually fatal within the
first days or months of life without treatment.
This defect isn't correctable, but some babies can be treated with a series of operations or with a heart transplant. Until an
operation is performed, the ductus is kept open by intravenous (IV) medication. Because these operations are complex and
different for each patient, you need to discuss all the medical and surgical options with your child's doctor. Your doctor will help
you decide which is best for your baby.
If you and your child's doctor choose surgery, it will be done in several stages. The first stage, called the Norwood procedure,
allows the right ventricle to pump blood to both the lungs and the body. It must be performed soon after birth. The final stage(s)
has many names including bi-directional Glenn, Fontan operation and lateral tunnel. These operations create a connection
between the veins returning blue blood to the heart and the pulmonary artery. The overall goal is to allow the right ventricle to
pump only oxygenated blood to the body and to prevent or reduce mixing of the red and blue blood. Some infants require several
intermediate operations to achieve the final goal.
Some doctors will recommend a heart transplant to treat this problem. Although it provides the infant with a heart that has normal
structure, the infant will require lifelong medications to prevent rejection. Many other problems related to transplants can develop.
You should discuss these with your doctor.
Children with hypoplastic left heart syndrome require lifelong follow-up by a cardiologist for repeated checks of how their heart is
working. Virtually all the children will require heart medicines.
People with hypoplastic left heart syndrome, before and after treatment, are at risk for getting an infection on the heart's inner
lining or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take
antibiotics before certain dental procedures.
Endocarditis
The American Heart Association has recently changed its guidelines for prevention of endocarditis. Some children who used to
take antibiotics before going to the dentist no longer have to do so.
The AHA now recommends these routine antibiotics before dental visits for patients who are at the highest risk for
adverse outcomes if they develop endocarditis. This includes:
a. Unrepaired cyanotic congenital heart defects, including palliative shunts and conduits
b. Completely repaired congenital heart defects with prosthetic material or device, whether placed by surgery or by catheter
intervention, during the first six months after the procedure (prophylaxis is recommended for first six months because
endothelialization of prosthetic material occurs within six months after the procedure)
c. Repaired congenital heart defect with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic
device (which inhibit endothelialization)
Except for the conditions listed above, antibiotic prophylaxis is no longer recommended for any other form of congenital
heart disease.
Good dental hygiene can help lower the risk of endocarditis. For more information about dental hygiene and preventing
endocarditis, ask your pediatric cardiologist.
Routine antibiotics before gastrointestinal or genitourinary tract procedures solely to prevent endocarditis are no longer
recommended for any patients.
(For more information on children and heart disease, see the American Heart Association Web site
www.americanheart.org/children. For information on congenital heart defects in Spanish, see
www.americanheart.org/Spanish/index.html