Adults With Congenital Heart Disease -- Glossary

Glossary

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A

Ablation ("RFA" or radiofrequency ablation)
A therapeutic method that physically destroys the heart tissue that causes or contributes to some types of fast heartbeats (tachycardia).

Anesthesiologist
A physician specializing in the practice of anesthesiology and the use of anesthetic medicines. These medications result in a loss of sensation, memory, pain and loss of consciousness, and are used in all surgical cases.

Arterial Switch Operation (ASO)
An open-heart procedure used to correct many forms of transposition of the great arteries.

Arteriovenous Malformations (AVMs)
An abnormal connection between the arteries and the veins. Pulmonary arteriovenous malformations are located in the lung, allowing oxygen-depleted blood to pass through the lungs without becoming oxygen-rich. In congenital heart disease patients, these malformations may be seen following modifications of the Glenn shunt, hemi-Fontan, Kawashima or Fontan procedures. Systemic arteriovenous malformations may occur in any organ and cause congestive heart failure. They typically occur in the abdomen or in the brain.

Attending (physician)
A more senior, supervising physician whose responsibilities include caring for a particular patient or special group of patients. Within teaching hospitals, the "attending" is typically a faculty member with teaching responsibilities.

Autograft (see "Ross procedure")

B

Baffle, Atrial (see Mustard or Senning)

Balloon Angioplasty (Blood Vessel Dilation)
An invasive catheterization procedure where a narrowed portion of a blood vessel (e.g., coarctation of the aorta, pulmonary artery stenosis) is enlarged by inflating a balloon that straddles the narrowed segment. Severe coarctation of the aorta or pulmonary artery narrowings resemble an hourglass. The balloon dilation intentionally tears part of the wall of the blood vessel.

Balloon Valvotomy (Valve Dilation)
An interventional catheterization technique in which an uninflated balloon is placed across a narrowed valve inside the heart. The balloon is inflated while situated across the narrowed valve, increasing the area available for blood flow and decreasing the degree of obstruction. Nearly all balloon valve dilations result in some degree of valve leakiness (regurgitation) after the procedure.

Blalock-Taussig ("B-T") Shunt
One of the first cardiac surgical procedures ever performed, the Blalock-Taussig shunt connects the blood vessel going to the arm ("subclavian artery") directly to the pulmonary artery. A "modified" Blalock-Taussig shunt involves a graft of Gore-texئ between the subclavian artery and the pulmonary artery.

C

Cardiac Catheterization
An "invasive" procedure in which thin plastic tubes ("catheters") are inserted into the bloodstream and advanced into the heart chambers using X-ray guidance. During a cardiac catheterization, measurements of oxygen levels and pressures are made in the heart chambers. In addition, X-ray pictures (angiograms) of the heart and blood vessels are made by injecting a dye into the bloodstream.

Cardiopulmonary Bypass
Also called the "heart/lung machine," cardiopulmonary bypass is a mechanical device that allows the heart to be safely stopped during cardiac surgical procedures. Blue (oxygenpoor) blood is drained from the patient to a reservoir where oxygen is added to it; this red (oxygen-rich) blood is then pumped back to the patient directly into the aorta. Hypothermia (lowering the body temperature) is used in most cases.

Case Management
The task of overseeing an individual patient's medical and social needs, ensuring that necessary services are obtained.

Clinical Nurse Specialist (CNS)
An advanced practice nurse with special expertise in patient care, family education and staff support.

Clinical Pathways
A process by which "routine" postoperative orders are placed into a standard protocol, minimizing variability in the ordering of tests by staff. Clinical pathways are especially useful in "straightforward" postoperative management. They have been shown to reduce hospital charges and length of stay by as much as 20 percent.

Closed-Heart Surgery
An operation on the heart (or more typically the great vessels) without the need for cardiopulmonary bypass. Examples of closed-heart surgery include repairs of coarctation of the aorta and patent ductus arteriosus.

Clubbing
Enlargement of the tips of the fingers and toes, especially visible in the nailbeds. Clubbing is usually associated with long-standing cyanosis and low oxygen levels in the blood.

Coil Embolization
The release of curled metal coils into abnormal blood vessels, resulting in non-surgical closure of these structures.

Collateral Vessels
Typically, an abnormal communication between the aorta and pulmonary arteries. Collateral vessels may also exist in the coronary circulation or large veins.

Conduit
An artificial (Gore-tex®) or human cadaver ("homograft") tube used in complex reconstructive surgery.

Congestive Heart Failure (CHF)
A combination of clinical signs and symptoms; in infantsmanifested as poor feeding, rapid breathing, sweatiness, rapid heart rate and failure to gain weight; in adults, manifested as fluid retention, shortness of breath, being easily fatigued and exercise intolerance. Congestive heart failure can be due to poor heart muscle function, abnormal communications or holes within the heart, or other abnormal burdens of the heart such as obstructed valves, leaky (regurgitant) valves, poorly controlled rhythm disturbances or shunts.

Cyanosis
A blue tinge to the skin, mucous membranes or nailbeds that results from abnormally low oxygen levels in the blood.

D

Defibrillator
A battery-driven, mechanical device used to treat life-threatening heart rhythm abnormalities. The battery and electrical circuits ("generator") are implanted in the body, usually under the shoulder or in the abdomen. Wires leading from the generator are placed on the heart muscle to deliver the electrical current needed to treat the abnormal rhythm.

E

Echocardiography
The use of ultrasound beams to visualize the heart and blood vessels. Complete studies may take over an hour to image all of the important structures; limited examinations may only require a few minutes. In small infants and toddlers, sedation may be necessary to obtain reliable information. "Doppler" techniques are often also used to look for obstructions in the heart or leakiness ("regurgitation") of any of the heart valves.

Eisenmenger Syndrome
If pulmonary hypertension (high blood pressure in the lungs) is caused by a pre-existing congenital heart defect, such as a ventricular or atrial septal defect, the pulmonary hypertension is considered secondary to the congenital heart defect. Then the term Eisenmenger Syndrome is often used.

Electrocardiogram (ECG or EKG) Measurement of the heart's electrical activity by placing electrodes on the arms, legs and chest. This test helps identify abnormally enlarged heart chambers or abnormalities of the heart's electrical system (arrhythmias).

F

Fellow A licensed physician who's already completed medical school (four years) and specialty training (three-seven years) in pediatrics, medicine, surgery, anesthesiology, etc., and is eligible for board certification in that specialty. Subspecialty training then takes place (for example, in cardiothoracic surgery, pediatric cardiology) during which time, the physician is called a "fellow."

Flutter, Atrial
Very rapid beating of the heart's upper chambers (atria).

Fontan Procedure
Originally described in the late 1960s, the Fontan procedure is used to separate the oxygen-poor (blue) blood from the oxygen-rich (red) blood in patients with single ventricle defect. Many modifications have been performed in the last 10-15 years, including direct connections of the atrium to the pulmonary artery, tunneling of the inferior vena cava inside the heart (lateral tunnel, or total cavopulmonary connection), or conduit insertion from the inferior vena cava to the pulmonary artery ("extracardiac Fontan"). A small hole may be intentionally left in this connection ("fenestrated Fontan").

G

Glenn Shunt
A connection between the superior vena cava and the right pulmonary artery.

Glenn Shunt, Bi-directional
Connection of the superior vena cava to the pulmonary artery, allowing blood from the upper half of the body to be directed to both ("bi-directional") pulmonary arteries.

H

Hemi-Fontan
Connection of the superior vena cava to both pulmonary arteries, similar to the bi-directional Glenn shunt. Homograft tissue is used to augment the connection and to separate the oxygen-poor (blue) blood from the upper half of the body from the oxygen-poor blood from the lower half of the body.

Holter Monitor
A long-term (24-48 hour) recording of the ECG.

Home-Based Healthcare
In-home care for ongoing medical conditions, usually by visiting nurses. This might include intravenous antibiotics, physical assessment, etc.

Homograft
Material (usually human heart valves and arteries) donated from a cadaver to be used during complex reconstructive surgery.

Hypoplasia
Underdevelopment of a cardiovascular structure, either a pumping chamber, valve or large blood vessel.

Hypothermic Circulatory Arrest
During open-heart surgery, especially in neonates and young children, the body temperature can be lowered to 60ƒF-65ƒF and the heart/lung machine turned off ("circulatory arrest"). This allows the surgeon to most precisely operate on a still (non-beating) heart, in an operative field where cardiac structures can best be seen.

I

Intern
A term for a physician in first-year training after medical school. The first year of a residency is typically called an internship.

Intravascular Ultrasound
A technique in which an ultrasound catheter is placed in the bloodstream during a heart catheterization to visualize blood vessels "from the inside." This technique is particularly helpful in cases of complex narrowing (stenosis), as may occur in the aorta (coarctation) or pulmonary arteries.

J

K

Konno Procedure
A surgical procedure used to enlarge the area below the aortic valve, by cutting through and patching the muscular wall that separates the left and right ventricles (ventricular septum).

L

M

Magnetic Resonance Imaging (MRI)
In congenital heart disease, MRI technology uses the body's magnetic field and high-tech computers to "reconstruct" images of the heart, blood vessels, lungs and trachea. MRI can be used in virtually all patients, unless metal structures (e.g., a pacemaker) are in the chest.

Maze Procedure
A surgical procedure to control atrial fibrillation and/or atrial flutter. A number of incisions are made in the atria to block the path of the arrhythmia.

Mustard Procedure
An intra-atrial baffle procedure (similar in many ways to the Senning procedure) used for transposition of the great arteries and similar congenital heart defects. The oxygen-poor (blue) blood is directed to the left ventricle and then to the lungs, while the oxygen-rich (red) blood is directed to the right ventricle and then to the body.

N

Norwood Procedure
A complex surgical procedure used for hypoplastic left-heart syndrome (and similar variants) where reconstruction of the absent or small aorta is accomplished by using the patient's own pulmonary artery. This allows unobstructed blood flow to be delivered to the body. As part of the Norwood procedure, the wall between the upper chambers of the heart is removed (atrial septectomy), and a small Gore-tex® tube (shunt) is inserted from the aorta to the pulmonary arteries.

Nurse Practitioner (NP)
An advanced practice nurse with special training and an advanced degree in nursing. Pediatric nurse practitioners (PNPs) may perform examinations, order medications and diagnostic procedures, educate staff and families and provide continuity of care between inpatient and outpatient settings.

O

Occlusion Devices (atrial and ventricular septal defect occluders)
A number of investigators are designing devices that can be delivered through a catheter to close holes in the upper chambers (ASDs) and lower chambers (VSDs). Although a number have been successfully used in clinical trials supervised by the Food and Drug Administration, the follow-up is necessarily quite short at this time.

Open-Heart Surgery
A cardiac surgical procedure using cardiopulmonary bypass.

P

PAC
"Premature atrial contraction"; an early beat of the upper chamber of the heart, which may result in the feeling of the heart "skipping." (See also Palpitations.)

PVC
"Premature ventricular contraction"; an early beat of the lower chamber of the heart, which may result in the feeling of the heart "skipping." (See also Palpitations.)

Pacemaker, Mechanical
A battery-driven, mechanical device used to treat heart rhythms that are too slow, too fast or irregular. The battery and electrical circuits ("generator") are implanted in the body, usually under the shoulder or in the abdomen. Wires leading from the generator are placed on the heart muscle to deliver the small amount of electrical current needed to treat the abnormal rhythm.

Palpitations
The sensation of the heart beating rapidly or irregularly.

Perfusionist
A specially trained individual who manages the heart/lung machine.

Phlebotomy
Removal of blood from the vein. This term applies to routine laboratory blood tests, but in patients with high blood counts (see Polycythemia), a larger amount of blood is removed and replaced with intravenous fluid to lower the blood count.

Polycythmia
An elevated number of red blood cells, also referred to as a "high hematocrit" or "thick blood." Polycythemia is often seen in patients with lower-than-normal levels of oxygen in the blood.

Potts Shunt
A direct connection between the aorta and the left pulmonary artery.

Protein-losing Enteropathy (PLE)
The abnormal loss of protein in the stool, resulting in crampy abdominal pain, diarrhea, fluid retention and swelling of the abdomen, extremities and eyelids. PLE is a rare complication of the Fontan operation, and occasionally other forms of congenital heart disease.

Q

R

Rastelli Procedure
A complex open-heart procedure used for children with complex congenital heart disease that usually includes (1) a ventricular septal defect, and (2) obstruction of flow to the pulmonary arteries. In this procedure, the VSD is closed with a large patch or baffle to direct oxygen-rich (red) blood to the aorta and then out to the body. An artificial conduit is typically inserted to direct oxygen-poor (blue) blood to the lungs.

Resident
A licensed physician completing training in a primary specialty (e.g., pediatrics, surgery, internal medicine, obstetrics/gynecology, etc.) following medical school.

Ross Procedure
Surgical technique used to manage complex obstruction or regurgitation (leakiness) of the aortic valve. The procedure involves using the patient's own pulmonary valve, an autograft, to replace the abnormal aortic valve. Typically, a homograft conduit is used in the space left by the removed pulmonary valve.

S

Senning Procedure
An intra-atrial baffle procedure (similar in many ways to the Mustard procedure) used for transposition of the great arteries and similar congenital heart defects. The oxygen-poor (blue) blood is directed to the left ventricle and then to the lungs, while the oxygen-rich (red) blood is directed to the right ventricle and then to the body.

Shunt

1. An abnormal flow pattern of blood through the chambers of the heart or through the large arteries leaving the heart. A "left-to-right" shunt results in extra blood flow entering the lungs, while a "right-to-left" shunt results in decreased blood flow to the lungs, low oxygen levels and cyanosis.
2. A surgically created connection designed to increase the delivery of blood to the lungs. The most common types of shunts are named for the physicians who popularized them (Blalock-Taussig, Waterston, Potts, Glenn). A "modified" shunt may involve the use of artificial material, such as Gore-tex®.

Social Worker
Within the hospital setting, a person specially trained to counsel and assist in the emotional, social, environmental and financial needs of the cardiac patient and family. This person often interacts as a liaison of need, working closely with medical staff and the family, both during and after hospitalization.

Stents (endovascular, endobronchial or tracheal)
Cylindrical metal devices that are placed on a balloon catheter, and are used to enlarge narrowed areas of blood vessels or the airway. When expanded, stents have the appearance of a "chicken wire" cylinder, and resist collapsing. When non-expanded, the stents are long and thin. Endobronchial stents are used inside the airways (trachea) and endovascular stents can be used in blood vessels (pulmonary arteries or veins, major systemic veins, atrial baffles, or coarctation of the aorta).

Stroke
Damage to the brain that may result from a blood clot or rupture in a vessel, depriving part of the brain of the blood supply it needs to function.Strokes may result in the loss of sensation, speech, memory or muscle movement. Some patients may have seizures related to the stroke.

SVT (supraventricular tachycardia)
Very rapid beating of the heart's upper chambers (atria).

Syncope
Passing-out, loss of consciousness or fainting.

T

Thoracoscopic Surgery
Similar to arthroscopic surgery for joint surgery or laparoscopic surgery in the abdomen, thoracoscopic surgery is performed by using small incisions and video cameras to do procedures typically done through larger open incisions.

Three-dimensional ("3-D") Echocardiography
Current echo technology allows the echo to be viewed in only two dimensions. Three-dimensional echocardiography allows the physician to "reconstruct" the heart and view the structural defects at any angle.

Transannular Patch
A patch placed across a narrow (stenotic) pulmonary valve to enlarge the passage through which blood travels to the lung. This type of patch is often used in repair of tetralogy of Fallot

Transesophageal Echocardiography (TEE)
An ultrasound technique in which the ultrasound probe (about as large as a pinky finger) is placed in the esophagus to "look" at the heart from behind. Transesophageal echocardiography is much more sensitive than transthoracic (across the chest) echocardiography, as overlying structures (bone and lungs) do not obscure the view. This technique requires sedation in almost all cases.

U

V

W

Waterston Shunt
A direct connection between the aorta and the right pulmonary artery.

X

Y

Z