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 Glossary of Childhood Onset Heart Disease






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Glossary of Childhood Onset Heart Disease

Written by:
Gil Wernovsky, M.D.
The Children's Hospital of Philadelphia
William B. Moskowitz, M.D.
Medical College of Virginia
Robert J. Sommer, M.D.
Mount Sinai Medical Center
Alan Tong, M.D.
Cedars-Sinai Medical Center

Compiled and edited by: Mona Barmash

Posted: December 9, 1998

Updated: October 27, 1999

Based upon the needs and requests of our readers, we have created this glossary to provide accurate and concise information for families and individuals. The following represents a "work in progress". Please contact us to submit items you would like to see included on this page.

Associated Conditions

Accessory Connection
An abnormal (accessory) connection of electrical tissue from the upper chambers of the heart (atria) to the lower chambers (ventricles). Abnormal conduction of electrical impulses through the accessory connection can result in very rapid heart rates; also called "reentrant supraventricular tachycardia".

A weakness of the wall of a cardiac chamber or blood vessel. Often associated with previous surgical or transcatheter interventions.

Arteriovenous Malformations (AVMs)
Abnormal connections between the arteries and the veins. Pulmonary arteriovenous malformations are located in the lung, allowing blue blood to pass through the lungs without becoming oxygen-rich. In congenital heart disease patients, they 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.

A fluid collection in the abdomen, usually due to poor cardiac function, most often involving inadequate pumping ability of the right sided chambers. Often associated with swelling (edema) of the legs.

A rare genetic condition in which the patient is born without a spleen. This usually occurs in the setting of complex malformations of other organ systems including all of the abdominal and thoracic organs. Cardiac disease is a significant part of this syndrome.

A term meaning enlargement of the heart, usually used in describing a chest x-ray.

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

Congestive Heart Failure (CHF)
Clinical spectrum of symptoms that result in poor feeding, rapid breathing, sweatiness, rapid heart rate, and failure to gain weight. 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, or shunts.

Term meaning literally, "heart on the right". Used to describe patients who, for a variety of reasons, have their hearts predominantly in the right chest rather than the left. Dextrocardia can be associated in some cases with a normal heart.

Eisenmenger’s Complex
The combination of a ventricular septal defect, pulmonary vascular disease (damage to the lung vessels from high pressure and/or volume of blood flow due to the VSD), and cyanosis.

Eisenmenger’s Syndrome
Any situation where increased pressure/flow in the lung vessels has resulted in cyanosis, due to shunting from the blue to the pink circulation (right to left shunt).

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Literally, blood in the respiratory tract secretions (coughing up blood). Hemoptysis is usually caused by very high pressures in the venous (right sided) circulation (such as in Eisenmenger's), resulting in the breakage of smaller blood vessels supplying the lungs. It can vary from trace to life-threatening amounts.

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A thickening of the muscle of the pumping chamber (ventricle) of the heart. This results from increased work of that chamber (the heart muscle gets large and thick with an increased work-out, just like the biceps muscle). Usually associated with obstructions in the valves or blood vessels through which the ventricle must push the blood, or with high blood pressure.

Obstructive airway disease
In pediatrics, obstuctive airway disease usually refers to an anatomic obstruction in the air passages proper. Examples include foreign bodies, very large tonsils/adenoids, tracheal/bronchial stenoses/webs/rings. In older people, the most common cause is smoking

Literally, excess red blood cells in the bloodstream, which may be due to chronic cyanosis among other reasons. People with Eisenmenger's are almost always polycythemic unless they are iron-deficient. Polycythemia can be a problem because the blood is literally very thick, and may cause a stroke or other obstruction elsewhere in the circulation.

Pulmonary Hypertension – primary
A condition where the lung vessels have high resistance to blood flow (or are "tight"), for no identifiable reason. Pulmonary hypertension is usually treated with oxygen or vasodilating medications such as calcium channel blockers, nitrates, prostacyclin, or others that directly or indirectly relax blood vessels. Occasionally, an experimental procedure to create a small opening in the atrial septum using a balloon dilation catheter is performed in the cardiac catheterization lab, to allow the high pressure in the right (blue) side of the heart to decompress to the left (pink) side of the circulation. This maintains cardiac output because more blood can be pumped out to the body, but since this extra blood is deoxygenated (unable to go through the lungs), this comes at the expense of increased cyanosis. This procedure is only done in people who are severely ill and awaiting lung transplant. For cases of severe, irreversible pulmonary hypertension, lung transplantation is currently the only treatment.

Pulmonary Hypertension – secondary
Tight lung vessels with an identifiable cause (see Eisenmenger's). Secondary pulmonary hypertension can be reversible if detected early in the process, and appropriate treatment started.

Reactive airway disease
More or less the same as "asthma"; a condition of constriction of the air passages due to a body's hyperactive response to various environmental stimuli.

Other Articles in This Section
Associated Conditions
Cath Lab Procedures
Imaging Techniques
Medical Personnel /Services
New Strategies / Techniques
Surgical Complications
Surgical Procedures

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