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".
us to submit items you would like to see included on this
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
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
A term meaning enlargement of the heart,
usually used in describing a chest x-ray.
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.
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
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
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.
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