Arkansas Children’s Heart Center provides diagnosis, treatment
and follow-up care for every congenital heart disease encountered. Whether
these treatments require one or more open-heart surgeries, or can be treated
with less invasive technologies, the Heart Center provides the state-of-the-art
care that gives your child the best chances for the most successful outcome.
The goal in every case is to repair the defect as completely as possible and
make the circulation as normal as it can be.
Overview descriptions of major conditions treated follow. In addition, we provide
a description of significant diagnostic procedures and noninvasive corrective
procedures. For greater detail on these conditions, their diagnosis and treatment,
please see the excellent resources referenced in our further
reading section.
Abnormal Heart Valve
The heart has 4 valves that allow blood to flow through the chambers
of the heart in one direction. If a valve is abnormal, it will not fully open
or close and will impede the flow of blood. If the valve is stenotic, blood
is pumped through a smaller than normal opening; this leads to regurgitation
(blood leaking back through the valve) of the blood back into the chamber.
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Adult Congenital Heart Disease
With the revolution in caring for children with congenital heart defects
and the advances in diagnosis and surgery children are now reaching adulthood
and living full, active lives. Many continue to require the care of cardiologists
familiar with birth defects of the heart.
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AICD
Automatic implantable cardiac defibrillator device; an AICD is implanted
into a patient and is used to treat tachycardias (fast heart rates) that are
recurrent. The leads will sense the cardiac rhythm, deliver electrical shocks,
and pace the heart as needed. AICDS are very useful in preventing sudden death
in patients with known sustained tachycardia or fibrillation.
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ALCAPA
See anomalous left coronary.
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Annuloplasty
Surgical repair on the annulus of a heart valve that improves its mobility
and function or enlarged the opening.
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Anomalous Venous Return – See TAPVR
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Anomalous Left Coronary
In a normal heart, the left coronary artery arises from the aorta,
the large vessel that carries oxygen-rich blood from the heart to the body.
In this defect, the left coronary artery arises from the pulmonary artery instead
of the aorta. This leads to low pressure in the coronary artery that can lead
to a heart attack.
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Aorta
The large artery that receives the blood from the left ventricle and distributes
it to the body.
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Aortic Arch Reconstruction
A surgical intervention that requires the repair of the aortic arch.
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Aortic Hypoplasia
See Coarctation of the Aorta.
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Aortic Stenosis
Aortic stenosis occurs when the aortic valve did not form properly—it
narrows and constricts blood flow. As with pulmonary stenosis, the need for
surgery depends on the severity of the condition.
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Aortoplasty
The surgical repair of Coarctation of the Aorta. The narrowed area
can be surgically removed or made larger with the help of surrounding structures
or a patch.
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Atrial Ectopic Tachycardia
a rapid, often irregular heart beat that originates from areas of the
upper chambers of the heart. In some cases there are multiple sites of origin
of these abnormal fast beats.
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Atrial Septal Defect (ASD)
This condition occurs when there is a defect, or hole, between the left and
right atria. The child may have few, if any, symptoms, and is treated by open-heart
surgery.
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Atrioventricular Canal Defect (also Endocardial Cushion
Defect or Atrioventricular Septal Defect)
Caused by a defect, or hole, in the center of the heart, this condition affects
the upper and lower heart chambers and valves. The treatment requires open-heart
surgery.
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AV Canal Defect
Atrioventricular canal defect.
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Balloon Valvuloplasty
A procedure in which a narrowed heart valve is stretched open using
a balloon catheter to improve the valve function and allowing blood to flow
from chamber to chamber.
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Berlin Heart
The Berlin Heart is a ventricular assist device (VAD), or blood pump, which
is not available in the United States, but can be used on an emergency basis
to treat heart failure until a heart transplantation procedure can be performed.
This “bridge to heart transplant” with the Berlin Heart is allowed
by the FDA only on a case-by-case basis. At this point, 12 other pediatric patients
in the U.S. have had the Berlin Heart used as a bridge to transplant.
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Biventricular Pacemaker
Traditional pacemakers are used to treat slow heart rhythms. They regulate
the right atrium and right ventricle to maintain a good heart rate and keep
the atrium and ventricle working together. Biventricular pacemakers add a third
lead to help the left ventricle contract at the same time as the right
ventricle.
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Cardiac Catheterization
The ACH Cardiac Catheterization suite has two fully staffed and equipped pediatric
biplane catheterization laboratories. In these labs, children and young adults
have specific measurements (chamber pressure, oxygen content) made on their
heart by placing narrow tubes (catheters) from veins or arteries into the heart
and lung blood vessels. Some cardiac disorders can be corrected in the catheterization
lab including closing Patent Ductus Arteriosus (PDA), coil occlusion of abnormal
blood vessels, enlarging narrow blood vessels, and opening poorly functioning
heart valves. In the near future, we will be closing some Atrial Septal Defects
(ASD) with a specialized occlusion device in the lab.
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Patients with rhythm problems (arrhythmia) are also diagnosed and treated in
the catheterization lab. This includes electrophysiology studies, radiofrequency
catheter ablation (RFCA), and placement of pacemakers or defibrillators.
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Coarctation of the Aorta
(Coarc)
A condition in which the walls of the aortic arch is narrowed constricting
the blood flow through the blood vessel that carries the oxygen-rich blood to
the body.
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Coarctation Ballooning
The opening or widening of a narrowed aortic arch using a balloon catheter.
This procedure is performed in the cardiac catheterization lab and does not
require a surgical incision.
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Congestive Heart Failure (CHF)
I nability of the heart to maintain an adequate amount of blood flow; this may
result from failure of the right or left ventricle or both; symptoms of CHF
are weakness, shortness of breath, stomach pain and swelling.
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CPB
Cardiopulmonary bypass (heart/lung machine); the bypass device provides blood
flow to the body and “bypasses” the patients heart and lungs allowing
the surgeon to operate on these structures. This provides continuous oxygenated
blood to the patient during heart surgery while misnaming blood loss.
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Cryoablation
A non-surgical procedure used to permanently disable arrhythmias by
using extremely cold temperatures to destroy the precise area of the heart that
is causing the arrhythmia.
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DeBakey VAD Child
The DeBakey VAD Child is the first VAD approved by the FDA for use
in children. The 1”x3” pump weighs only 4 ounces and is silent in
operation, utilizing the same technology of the implantable adult pump, also
manufactured by MicroMed Technology, Inc. Designed in collaboration with NASA,
the Baylor College of Medicine and Drs. Michael DeBakey and George Noon, the
DeBakey VAD is intended for end-stage heart failure patients who can no longer
provide necessary blood flow with their native heart. More than 240 DeBakey
VADs have been implanted in adults worldwide.
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Defibrillation
Delivery of an electric shock to the heart in order to stop dangerous
heart rhythms.
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DiGeorge Syndrome
A syndrome that may include all of the following: cardiac defects (especially
TA & IAA), immune deficiencies and a predilection to infection, chromosomal
abnormalities, abnormal levels of calcium in the blood, and distinct facial
features.
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Down’s Syndrome
A congenital problem characterized by varying degrees
of mental retardation and distinct facial features, sometimes associated with
congenital heart defects.
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ECMO – extracorporeal membrane oxygenation
A special procedure that uses an artificial heart-lung machine to take
over the work of the lungs and heart in patients with acute, reversible cardiac
or respiratory failure who are unresponsive to conventional medical or pharmacologic
management. ECMO is used most often in newborns and young children but can be
used in adult who have failing hearts and lungs.
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Ectopic Atrial Tachycardia or SVT
Also called SVT; this abnormal rhythm is a fast rhythm that involves
the atria (upper chambers) and ventricles (lower chambers) of the heart. SVT
can be recurring. In young infants the heart rate is usually more that 220 beats
per minute. Treatment for SVT may be cardioversion or medications.
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Fetal Echocardiogram
An ultrasound of a baby’s heart while in in-utero. Fetal echos
can be performed after the 18th week of pregnancy.
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Fontan Procedure
A two-staged open-heart surgery that is used in the repair of several
cardiac defects, namely when the right ventricle is missing or is too small.
This procedure involves the creation of a passageway that diverts venous blood
from the right atrium to the pulmonary arteries without passing through the
right ventricle.
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Glenn Shunt
An anastomosis of the superior vena cava (SVC) to the right pulmonary
artery to improve blood flow to the lungs; this is often a first stage leading
to the Fontan procedure.
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GUCH
Grown-up Congenital Heart Disease, see adult congenital heart disease.
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Heart Bypass
See CPB.
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Heart Failure
Inability of the heart to receive or pump blood effectively.
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Heart Transplantation
An operation to replace a diseased heart with a healthy one that is
donated by another person who has experienced brain death.
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Holter Monitoring
Holter is a recording or "Ambulatory ECG" of each heartbeat during
24 hours of normal activity. Abnormal rhythms, while awake or sleeping, are
recorded on a portable device and can be detected.
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Hypertrophic Cardiomyopathy (HCM)
A thickening of the heart muscle that leads to stiffening of the heart
walls and abnormal heart function. This thickening also leads to obstruction
of blood flow out of the heart and heart rhythm problems.
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Hypoplastic Left Heart Syndrome
Here, the left side of the heart — including the aorta, aortic valve,
left ventricle and mitral valve — is underdeveloped. Although the defect
itself is not correctable, some babies can be treated with a series of operations
or with heart transplantation.
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Interrupted Aortic Arch (IAA)
A life threatening lesion that occurs if the aorta does not develop
properly and connect the aorta leaving the heart (ascending aorta) with the
descending aorta supplying blood to the lower body; life is sustained because
of the PDA that stays open after birth; the child becomes symptomatic as the
ductus begins to close and requires surgery during the first few days of life.
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Leaking Heart Valve
An abnormal valve that does not fully close allowing blood to flow
back into the chamber it is leaving. Another name for this is regurgitation.
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Long QT Syndrome
The QT interval is a measure of time required for depolarization and
repolarization to occur. In long QT syndrome, the duration of the repolarization
is longer that normal. An interval above 440msec is considered prolonged. This
syndrome is due to overload of myocardial cells with positive charged ions during
ventricular repolarization. This electrical defect predisposes one to a very
fast heart rhythm (Torsade de Pointes) which leads to sudden loss of consiousness
and may cause sudden cardiac death.
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LVAD
Left ventricle assist device; a mechanical pump that is surgically
implanted to help maintain the pumping ability of a failing heart. This device
is a “bridge to transplant” meaning keeping a patient alive until
a donor heart is found.
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Marfan’s Syndrome
Marfan’s is a congenital disorder of connective tissue, characterized
by abnormal length of extremities (especially fingers and toes) and cardiovascular
abnormalities (commonly dilation of the ascending aorta). Open-heart surgery
is required, usually in stages.
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Mitral Valve Prolapse
A condition that leads to the heart valve between the left atrium and
the left ventricle (the main pumping chamber of the heart) bowing backwards
with each heart beat. This can lead to leaking of the mitral valve and heart
rhythm problems.
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Non-Invasive Diagnostics and Treatments
Advances in cardiology now allow us to do many diagnostic studies without surgery.
The ACH Heart Station and/or Cardiac Catheterization Laboratory offers:
- Electrocardiography (ECG/EKG)
Pediatric ECG’s are performed routinely by technicians who have a special
touch with young children and adolescents alike. ECG’s provide the cardiologists
with specific information about the heart rhythm and electrical forces.
- Event Recording
An event monitor (loop recorder) is a recording device that is worn by the
patient for up to one to two months. Two wires from the monitor are attached
to the patient throughout much of each day. When the patient feels an unusual
heart rhythm, a button on the monitor is pressed. The patient can then call
our 24-hour receiver and send the stored rhythm over the phone. The event
recorder can then be reused.
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Norwood Procedure
A staged open-heart sugery that is used in the surgical management
of infants born with hypoplastic left heart syndrome (HLHS). HLHS occurs when
there is an absence or underdevelopment of the left ventricle. It is also associated
with aortic atresia, aortic arch hypoplasia, and coarctation of the aorta.
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Pacemaker Evaluation
Regular telephone transmissions are received from pacemaker patients to assess
function and remaining battery life. This is in addition to yearly pacemaker
clinic visits.
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Patent Ductus Arteriosus (PDA)
A blood vessel connecting the aorta to the pulmonary artery; this structure
is necessary for the fetus while the heart and lungs develop, but is considered
a heart defect if it does not close naturally after birth.
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Protein Losing Enteropathy (PLE)
A disorder of the gastro-intestinal tract that involves excessive loss
of important plasma proteins into the intestines. The result is low blood levels
of protein with swelling and fluid collection.
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Pulmonary
Refers to the lungs.
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Pulmonary Atresia
This condition means there is no pulmonary valve, causing a lack of blood flow
from the right ventricle into the pulmonary artery and on to the lungs. Multi-stage
open-heart surgery is indicated.
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Pulmonary Stenosis
Pulmonary stenosis occurs when the pulmonary valve narrows and causes the heart
to pump harder in order to get blood past the blockage. In most children, balloon
vulvoplasty can be used during cardiac catheterization. For some children, surgery
is necessary.
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Radiofrequency Ablation
A non-surgical treatment for abnormal heart rhythms where a long, flexible
wire is passed into the heart to ablate (eliminate) the precise area of the
heart that is causing the arrhythmia. The heart must be “mapped”
to identify the area of inappropriate conduction and then positioned at this
spot to deliver energy that will destroy the tissue.
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Ross Procedure
An open heart surgical procedure for the correction of aortic valve
disease; the native aortic valve is removed and is replaced with the native
pulmonary valve; a homograph valve (donor valve) is then placed into the pulmonary
valve position.
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Stent
A procedure in which a small, expandable wire mesh tube is inserted
into an artery or vein to hold it open. This procedure is performed in the cardiac
catheterization lab.
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Stress Testing
Complete metabolic cardio-pulmonary stress testing (bike or treadmill) is performed
daily. This is a valuable testing method to evaluate heart and lung function
under exercise and recovery situations. Rhythm problems can also be assessed.
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Sudden Death
Sudden cardiac death results from an abrupt loss of heart function
(cardiac arrest). The time and mode of death are unexpected and occurs within
minutes after symptoms appear. The most common underlying reason is coronary
heart disease. Sudden death occurs when the electrical impulses in the diseased
heart become rapid or chaotic or both. The irregular heart rhythm causes the
heart to suddenly stop beating. Some cardiac arrests are due to extreme slowing
of the heart.
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Tachycardia
A rapid heart rate; rates depend upon the child’s age.
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TAPVR
Total anomalous pulmonary venous return; pulmonary veins normally bring
oxygenated blood back from the lungs to the left atrium. In TAPVR, the pulmonary
veins drain incorrectly into the right atrium, superior vena cava, inferior
vena cava, or hepatic vein.
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Tetralogy of Fallot - (TOF/Tet)
A combination of four defects 1) VSD, 2) overriding aorta, 3) narrowing of the
pulmonary artery, 4) thickness of the right ventricle; this is the most common
form of cyanotic heart disease.
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Transposition of the Great Arteries
In a normal heart, the pulmonary artery carries blood from the right ventricle
to the lungs to get oxygen, and the aorta carries blood from the left ventricle
to the body. In this case, the vessels are reversed. This causes oxygenated
blood to travel only to the lungs — not the body. Initially, a balloon
atrial septostomy is done to improve the body’s oxygen supply. After the
blood supply is improved, a surgeon performs either an arterial switch or a
venous switch (also known as the Mustard procedure or Senning procedure).
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Tricuspid Atresia
In this case, the tricuspid valve is missing. No blood can flow from the right
atrium to the right ventricle. This causes the right ventricle to be underdeveloped
and small. Multi-stage open-heart surgery may be required.
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Total Anomalous Pulmonary Venous Connection (TAPVC)
This condition occurs when the pulmonary veins that bring oxygenated blood from
the lungs back to the heart are abnormally drained through the right atrium
instead of the left. This defect must be repaired surgically and may require
future procedures.
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Truncus Arteriosus
Sometimes only one artery develops from the heart and forms both the aorta and
pulmonary artery. This complex malformation requires open-heart surgery, possibly
in stages.
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Turner Syndrome
A genetic disorder only affecting females. The patient will have one
X chromosome in some or all cells or two chromosomes in which one is damaged.
Signs of Turner’s include short stature, delayed growth of the skeleton,
shortened fourth and fifth fingers, broad chest, and has associated heart abnormalities.
Diagnosis is made by blood test (karyotype).
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Valvuloplasty
Surgical repair of an abnormal valve. Valves can be repaired or replaced
with a mechanical or prosthetic valve. This will require open heart surgery.
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Valve Repair
Abnormal heart valves may be repaired by several procedures: percutaneous
balloon valvuloplasty, valvulotomy, or through a minimally invasive heart valve
procedure.
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Valve Replacement
Surgical intervention to remove abnormal valve and replace with a mechanical
or prosthetic valve.
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Ventricular Fibrillation
A life threatening condition in which the heart ceases to beat regularly.
The ventricles will “quiver” or fibrillate very rapidly. A person
cannot survive this condition for long. The patient must be defibrillated with
an AED, defibrillation machine or anti-arrhythmic medication.
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Ventricular Septal Defect (VSD)
Sometimes a defect, or hole, happens between the heart ventricles. VSD symptoms
may occur several weeks after birth and can be treated with open-heart surgery
that restores the blood circulation to normal.
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Ventricular Tachycardia (VT)
Fast heart beat originating in the ventricles.
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