Pediatric heart defects - heart valve abnormality

Arkansas Children’s Heart Center provides diagnosis, treatment and follow-up care for every congenital heart disease encountered. Whether this congenital heart disease support and treatment requires 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. Our goal is to repair each of the pediatric and adult heart defects that we encounter 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.

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Abnormal Heart Valve	Heart Failure
Adult Congenital Heart Disease	Heart Transplantation
AICD	Holter Monitoring
ALCAPA	Hypertrophic Cardiomyopathy (HCM)
Annuloplasty	Hypoplastic Left Heart Syndrome
Anomalous Venous Return	Interrupted Aortic Arch (IAA)
Anomalous Left Coronary	Leaking Heart Valve
Aorta	Long QT Syndrome
Aortic Arch Reconstruction	LVAD
Aortic Hypoplasia	Marfan’s Syndrome
Aortic Stenosis	Mitral Valve Prolapse
Atrial Septal Defect (ASD)	Non-Invasive Diagnostics and Treatments
Atrioventricular Canal Defect	Norwood Procedure
AV Canal Defect	Pacemaker Evaluation
Balloon Valvuloplasty	Patent Ductus Arteriosus (PDA)
Berlin Heart	Protein Losing Enteropathy (PLE)
Biventricular Pacemaker	Pulmonary
Cardiac Catheterization	Pulmonary Atresia
Coarctation of the Aorta	Pulmonary Stenosis
Coarctation Ballooning	Radiofrequency Ablation
Congestive Heart Failure (CHF)	Ross Procedure
CPB	Stent
Cryoablation	Stress Testing
DeBakey VAD Child	Sudden Death
Defibrillation	Tachycardia
Down’s Syndrome	Tetralogy of Fallot (TOF/Tet)
ECMO – extracorporeal membrane oxygenation	Transposition of the Great Arteries
Ectopic Atrial Tachycardia or SVT	Tricuspid Atresia
Fetal Echocardiogram	Total Anomalous Pulmonary Venous Connection (TAPVC)
Echocardiography (ECHO)	Truncus Arteriosus
Electrophysiology	Turner Syndrome
Event Recording	Valvuloplasty
Fontan	Valve Repair
Glenn Shunt	Valve Replacement
GUCH	Ventricular Fibrillation
Heart Bypass	Ventricular Septal Defect (VSD)
	Ventricular Tachycardia (VT)

Heart Valve Abnormality
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.

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.

Robert Morrow, M.D., chief of Pediatric Cardiology at Arkansas Children's Hospital and professor of Pediatrics at the University of Arkansas for MedicalSciences College of Medicine tells why adults with congenital heart disease need to be treated in a children’s hospital, rather than an adult hospital, and what the program at Arkansas Children's Hospital can offer those patients. Click here to view the Virtual Appointment

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.

ALCAPA
See anomalous left coronary. For more information from the Cove Point Foundation click here

Annuloplasty
Surgical repair on the annulus of a heart valve that improves its mobility and function or enlarged the opening.

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.

Aorta
The large artery that receives the blood from the left ventricle and distributes it to the body.

Aortic Arch Reconstruction
A surgical intervention that requires the repair of the aortic arch.

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. For more information from the Cove Point Foundation click here.

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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.

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. For more information from the Cove Point Foundation click here.

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. For more information from the Cove Point Foundation click here.

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.

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.

Robert D.B. “Jake” Jaquiss, M.D., chief of Pediatric and Congenital Cardiothoracic Surgery at Arkansas Children's Hospital and professor of Surgery at the University of Arkansas for

Medical Sciences talks about the Berlin Heart technology, which is used at Arkansas Children's Hospital to “bridge” children waiting for heart transplants who might not otherwise survive until a donor heart becomes available. Click here to view the Virtual Appointment.

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.

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. For more information from the Cove Point Foundation click here.

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.

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. For more information from the Cove Point Foundation click here.

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.

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.

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.

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.

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.

Defibrillation
Delivery of an electric shock to the heart in order to stop dangerous heart rhythms.

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.

Down’s Syndrome
A congenital problem characterized by varying degrees of mental retardation and distinct facial features, sometimes associated with congenital heart defects.

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.

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.

Electrophysiology
Electrophysiology deals with cardiovascular defects such as an irregular heart beat, often using catheterization procedures. The ability to perform these procedures without X-ray creates a new field of cardiology patients. Patients who are pregnant or are being treated for cancer can now receive treatment for rhythm disturbances that need ablation, since the need for X-ray has been eliminated in majority of the cases.

Volkan Tuzcu, M.D., the only pediatric electrophysiologist in Arkansas, is the director of

Electrophysiology and Pacing at Arkansas Children’s Hospital and associate professor of Pediatrics at the University of Arkansas for Medical Sciences College of Medicine. Dr. Tuzcu discusses recent innovations in treating patients who have electrical problems within their heart, problems that can be corrected without the need for open heart surgery. Click here to view the Virtual Appointment.

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. For more information from the Cove Point Foundation click here.

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.

Heart Transplantation
An operation to replace a diseased heart with a healthy one that is donated by another person who has experienced brain death. For more information from the Cove Point Foundation click here.

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. For more information from the Cove Point Foundation click here.

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. For more information from the Cove Point Foundation click here.

Hypoplastic Left Heart Syndrome
Here, the left side of the heart — including the aorta, aorticvalve,left ventricle and mitralvalve — is underdeveloped. Although the defect itself is not correctable, some babies can be treated with a series of operations or with heart transplantation. For more information from Cove Point Foundation click here. Fontenot

Eudice Fontenot, M.D., pediatric cardiologist at Arkansas Children's Hospital and associate professor of Pediatrics at the University of Arkansas for Medical Sciences College of Medicine, discusses the rare birth defect known as Hypoplastic Left Heart Syndrome and how physicians at Arkansas Children's Hospital treat patients who have HLHS. Click here to view the virtual appointment.

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. For more information from Cove Point Foundation click here.

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.

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.

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.

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. For more information from Cove Point Foundation click here.

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. For more information from Cove Point Foundation click here.

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.

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.

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. For more information from Cove Point Foundation click here.

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 pediatric heart defect if it does not close naturally after birth. For more information from Cove Point Foundation click here.

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.

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. For more information from Cove Point Foundation click here.

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. For more information from Cove Point Foundation click here.

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.

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.

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.

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. For more information from Cove Point Foundation click here.

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.

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.

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. For more information from Cove Point Foundation click here.

Transposition of the Great Arteries, D-Type
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). For more information from Cove Point Foundation click here.

Transposition of the Great Arteries, L-Type
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). For more information from Cove Point Foundation click here.

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. For more information from Cove Point Foundation click here.

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.

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. For more information from Cove Point Foundation click here.

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).

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.

Valve Repair
A heart valve abnormality may be repaired by several procedures: percutaneous balloon valvuloplasty, valvulotomy, or through a minimally invasive heart valve procedure.

Valve Replacement
Surgical intervention to remove abnormal valve and replace with a mechanical or prosthetic valve.

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.

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. For more information from Cove Point Foundation click here.

Heart Conditions, Diagnosis and Treatment