Common Cardiac Anomalies Seen within the Cardiac

Catheterization Lab
Lindsey E. Walker, student at the University of North Carolina- Chapel Hill
Abstract
A cardiac catheterization procedure, whether diagnostic or interventional, sheds light on
both the normal and abnormal cardiac anatomy and conditions present within a small portion of
the general hospital. It is important to be familiar with the most common conditions as it can
determine the type of care and treatment that should be provided to the patient. This article will
provide the reader with a general overview of cardiac anatomy and physiology along with
common cardiac anomalies that are seen in the cardiac catheterization laboratory including
coronary artery anomalies, alcoholic cardiomyopathy and valve stenosis.
After completing this article, the reader should be able to:

State the main coronary arteries and their branches.

Trace the path of blood through the heart.
Describe the basics of a cardiac catheterization procedure.
List common indications and contraindications for a cardiac catheterization procedure.
Understand the differences between dilated, hypertrophic, restrictive and unclassified
cardiomyopathies.
State the definition of a coronary artery anomaly.
Discuss the difference between a cardiomyopathy and a cardiac anomaly.
Understand causes of both congenital and acquired mitral and aortic valve stenosis.
Explain the effects of stress induced Takotsubo syndrome on the heart muscle.
List and describe several coronary artery anomalies.

Anatomy
Overview and Function
The heart is one of the few organs that is absolutely critical in maintaining life. It
functions to pump blood through its many chambers, dropping off deoxygenated blood and
picking up oxygenated blood, and then out through vessels that deliver that blood to the systemic
circulation.1 The heart does this by acting as a muscular pump, contracting in order to push
blood out of the chambers and relaxing so that more blood can fill those now empty chambers. 1
It is a repetitive system that is continuously working to deliver oxygen and nutrients to the
widespread areas of the body up until your last heartbeat.
Despite the compact size, roughly the same as a fist, the heart has very in depth anatomy.1
Located in the mediastinum of the thoracic cavity, it sits between the left and the right lung

1
lobes, posterior to the sternum but anterior to the spine.1 The shape is almost like an upside down
triangle with rounded tips in the body, with the top, flatter portion as the base and the lower tip as
the apex.1
Layers of the Heart
Now we will look at the different layer of the heart from the outside-in. Encapsulating
and protecting the heart is the pericardium. The function of the pericardium is to anchor it
within the thoracic cavity.1 It has an outer wall called the pericardial sac or the parietal
pericardium and an inner wall call the epicardium or the visceral pericardium.1 The pericardial
sac is made of two layers: an outer, fibrous layer and the layer underneath that is a serous layer.
The outer wall then connects to the inner epicardium at the base, where it then goes to cover the
surface of the heart. The epicardium will also be discussed later with the other layers of the heart
wall. In between both the outer and inner wall, there lies both the pericardial cavity and
pericardial fluid that allows the heart to expand, contract and move freely without any friction.1
Moving deeper into the actual heart, the first layer of the heart wall is the previously discussed
epicardium, the thin layer that covers the outer portion of the heart. The heart wall is made up of
three layers. Saladin and McFarlands Essentials of Anatomy & Physiology describe it in relation
to a sandwich, describing it as a thick muscular myocardium sandwiched between two thin
serous membranes, the epicardium and endocardium.1 The epicardium is so thin, it resembles
tissue paper and is transparent so the underlying myocardium can be seen. 1 Below the
epicardium is the thick layer of myocardium, or heart muscle, which makes up most of the
weight of the heart. The thicknesses of the heart walls vary depending on how much work, or
contracting, that area of the heart does. For example the left ventricle wall is significantly thicker
than the right ventricle due to the sheer fact that the left ventricle is pumping blood out to the
whole body whereas the right is just pumping into the pulmonary circulation. As you will see in
several of the pathologies discussed later in this article, the myocardium can grow so large that it
can inhibit the normal function of the heart. Due to the coiling of the heart muscle, the
contracting motion of the heart muscle looks much like wringing out a towel, or a twisting
motion to squeeze the blood out of each chamber.1 Underneath the myocardial layer is the final
layer, the thin endocardium that covers the inside of the heart chambers and valves the same as
the epicardium does the outside.1
The Heart Chambers
There are in total, four chambers of the heart: two atria and two ventricles, each separated
out into the right heart and the left heart by the type of blood it receives. The atria sit above the

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ventricles and are the first to receive blood from their prospective systems. They are receiving
chambers for blood returning to the heart by way of great veins as Saladin and McFarland
described it.1 The right atrium receives deoxygenated blood from the superior and inferior vena
cava while the left atrium receives oxygenated blood from the pulmonary veins. They are both
relatively thin walled in comparison to their fellow ventricles due to the reduced force of
contraction needed to move the blood into the adjacent chamber. Once out of the atria the blood
moves into the ventricles. The ventricles are responsible for moving the blood into the arteries.
As hinted at earlier, the ventricles have much thicker walls due to the need for forceful
contraction to eject blood into the different systems.
When in comparison, the left side of the heart can be considered to be the stronger of the
two. The right heart circulates blood through the pulmonary system, where deoxygenated blood
is dropped off and fresh, oxygenated blood is picked up and brought back to the heart.
Meanwhile the left side of the heart takes that freshly oxygenated blood and circulates it through
the body or the so-called systemic circulation. There is much more tissue being served by the
systemic circulation than the pulmonary system, therefore there is a greater need for more
muscle on the left side of the heart, specifically the left ventricle. With one, strong contraction
from the left ventricle, there is enough force to push that blood all the way through the many
arteries of the body.
At the conjunction between the atria and ventricles and the ventricles and their
corresponding great arteries, there lies a one-way valve. Each valve has a set number of thin
cusps and is structured specifically to meet the needs of that chamber.1 The atrioventricular (AV)
valves sit between the atria and ventricles. They relax and flap open during atrial contraction so
that blood can flow into the ventricle but then are held shut by specialized papillary muscles and
tendinous cord strings when the ventricle contracts in order to prevent blood from flow
retrograde back into the atrium.1 The right AV valve has three cusps, therefore is also called the
tricuspid valve.1 The left AV valve or mitral valve is the inlet valve to the left ventricle.2 It is
called the mitral valve due to its similarities to a miter which is a headdress a church bishop
wears.1 It has only two leaflets or cusps, so it is called the bicuspid valve that are attached to two
papillary muscles that are anchored down to the walls of the left ventricle by small strings called
chordae tendineae, which keep the cusps from folding in on themselves during ventricular
systole.2 Semilunar valves connect the ventricles to the great arteries: the pulmonary valve
connects the right ventricle to the pulmonary trunk and the aortic valve connects the left ventricle

3
to the aorta. Unlike the AV valves, the semilunar valves each have three cusps, with each cusp
having a semilunar shape (hints the name) or a shape like shirt pockets as Saladin and
McFarland describe it.1 The force from the contraction of the ventricles causes the semilunar
valves to open and flatten against the artery.1 Once that force is removed and the ventricles move
into the relaxation phase, the cusps then relax back into their normal semilunar shape. As the
ventricle relaxes, blood from the arteries flows retrograde and fills the concave shaped cusps,
pushing them together to create a seal that allows very little blood to flow backwards.1
The Coronary Arteries
Since the heart is continuously working to supply blood to the many organs of the body, it
itself needs its own supply of blood in order to work sufficiently. This system is termed the
coronary circulation and is fully devoted to supplying the heart wall, as stated by Saladin and
McFarland.1 Because the heart works so hard, it needs ample amount of blood, more specifically
250 milliliters per minute. For a more descriptive way of getting this point across, Saladin and
McFarland state, This is about 5% of the circulating blood going to meet the metabolic needs of
the heart alone, even though the heart is only 0.5% of the bodys weight. It receives 10 times its
fair share to sustain its strenuous workload. 1 While there is a vast system of vessels in the
coronary circulation, as is for any circulation system, for the purpose of this paper, we will only
focus on the coronary arteries as those are the main points of view during a cardiac
catheterization procedure.
The coronary arteries can be described as both branching and variable from an
anatomical standpoint.1 Their structure resembles that of tree roots while the specificity of the
pattern of coronary artery placement is variable among each person.1 For example the supplier of
the posterior descending artery or PDA can vary between the left circumflex artery and the right
coronary artery. But for the most part, the pattern of coronary arteries described below represents
that of the majority of the population.
There are three main coronary arteries: the right coronary artery (RCA) and the left
circumflex artery (LCX) and left anterior descending (LAD) both of the which diverge off of the
left main coronary artery (LMCA).3 Both the left and the right coronary arteries branch off the
aorta after the aortic valve from their corresponding aortic sinus and then converge in the
direction of the apex of the heart.3 Although it would seem differently, the coronary arteries do
not receive blood supply with ventricular contraction, rather when the blood begins to flow back

4
against the aortic valve with ventricular relaxation, the coronary arteries receive blood flow.1
Once flow hits both main coronary arteries, it travels down through their branches.
The left main coronary artery originates from the left sinus of Valsalva and maneuvers
between the pulmonary artery and the left auricle before traveling down the coronary sulcus.3
There are no significant branches off the LMCA, only the bifurcation into the left circumflex and
left anterior descending arteries, which occurs quickly after the ostium.3 The LAD, more
commonly known as the left anterior descending, ventures around the left side of the pulmonary
artery and then moves through the anterior interventricular sulcus towards the apex of the
heart.1,3 The LAD can be divided into three different segments, with an average full length of 10-
13 cm.3 Along the full length, branches arise off diagonally towards the septum, called septal
perforator branches that work to supply the anterior two-thirds of the basal interventricular
septum and the mid- and apical level septum.3
Back at the bifurcation of the LMCA, the other branch is known as the left circumflex
(LCX), which travels in a circular motion, venturing to the left towards the diaphragmatic
cardiac surface along the coronary sulcus and stopping prior to the posterior interventricular
sulcus.3 The LCX is shorter than the LAD at only 5-8 cm but gives rise to numerous important
branches including the left marginal artery that continues around the heart to the obtuse margin
and the obtuse marginal branch which supplies a portion of the inferior wall.3
The right coronary artery arises from the right sinus of Valsalva of the ascending aorta
and is responsible for supplying blood to the right side of the heart including the right atrium,
right ventricle, sinoatrial and AV node along with numerous other right-sided territories.3 The
RCA travels over the anterior portion of the heart, between the right auricle and pulmonary
artery, vertically in the right atrioventricular sulcus and then moves posteriorly in the
diaphragmatic surface sulcus towards the base of the heart.3 It does not bifurcate like the LMCA,
however gives rise to other important branches. Initially after the right sinus of Valsalva is the
right conus branch which is responsible for supplying the right ventricle.3 This is a branch that
needs to be carefully avoided during a diagnostic cardiac catheterization due to its sensitivity and
can trigger a ventricular arrhythmia if sporadically and forcefully injected with contrast.
Branching off from the right coronary artery next is the right atrioventricular artery followed by
the right marginal and the posterior interventricular branch, also known as the posterior
descending artery (PDA).1 For more detail on the branches of coronary arteries and the areas they
supply to, see Table 1.

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The Flow of Blood
Starting from the right atrium, deoxygenated blood returns to the heart via the superior
and inferior vena cava after circulating through the body and dropping off oxygen and nutrients
to the tissues. It flows first through the tricuspid valve, and then into the right ventricle. From
there it will flow through the pulmonary circulation system but not before passing through the
pulmonary semilunar valve and out the pulmonary trunk. While in the pulmonary system, the
blood will drop off the carbon dioxide picked up from the tissues and replace it with oxygen
before returning to the heart. Traveling as fresh, newly oxygenated blood, it will first enter the
pulmonary arteries, then the left atrium, through the bicuspid or mitral valve and then into the
left ventricle. Before being sent back out to the tissues, the blood moves through the aortic
semilunar valve and out into both the aorta and the coronary arteries.
Cardiac Catheterization
What is cardiac catheterization?
Cardiac catheterization is a surgical procedure that is less invasive than most with the
intention of diagnosis and possible intervention or fixing of various cardiovascular disorders. The
procedure uses catheters and wires from a peripheral access site, up into the heart and its
corresponding vasculature. The procedure is done under dynamic x-ray or fluoroscopy using
contrast media in order to visualize the necessary structures.
There are many reasons why a cardiac catheterization procedure may be recommended
for a patient. Those reasons can vary from non-emergent to emergent situations such as during an
ST-segment elevation myocardial infarction (STEMI), sudden cardiovascular death, or
myocardial infarction with unstable angina post-infarction.4 Other indications that are not so
emergent, yet still important for diagnostic and treatment plan purposes include suspected or
known coronary artery disease with a positive stress test, evaluation of conditions pre-operation,
and valvular heart disease.4 With indications, there are also always contraindications that are
present that should sway the recommendation of a cardiac catheterization procedure. There are
absolute contraindications such as the facility or hospital is inadequately staffed with
knowledgeable personnel or equipment needed for such a risky procedure.4 Besides the lack of
staff or equipment, other relative contraindications relate mostly to the patient. The patient
should be deferred from the consideration of a cardiac catheterization procedure should they
have an infection, renal failure, an electrolyte imbalance, or uncontrolled arrhythmias.4 Those
conditions along with many others pose a pre-operative threat that may exacerbate other risks
that come along with the cardiac catheterization procedure.

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Cardiac Anomalies
Definition
The term cardiac anomaly is something that is not often heard but rather coronary artery
anomaly (CAA). Villa, et. al defines coronary artery anomalies as a coronary pattern or
morphological feature that is encountered in less than 1% of the general population.3 The
definition of a coronary artery anomaly is formed based on the definition of normal coronary
anatomy that determines what is normal based on what is seen in more than 1% of the general
population including normal anatomical variants which are also seen in greater than 1% of the
population.3 In the case of congenital coronary anomalies, these deviations in the normal
anatomy occur due to an error during coronary bud development.3 Due to the effects of both
acquired and congenital coronary artery anomalies, sudden cardiac death occurs in 20% of young
athletes.5 However, even with that statistic, they are considered relatively uncommon.3 This
makes coronary anomalies a significant topic as it points to the fact that these conditions arent
typical heart conditions that are seen in the aging population, but one that affects people of all
age ranges. Clinical symptoms of coronary anomalies may include chest pain, dyspnea,
palpation, syncope and arrhythmia.3
Anomaly vs. Cardiomyopathy
An anomaly is defined as, something that deviates from what is standard, normal or
expected by the Oxford Dictionary. Rather than limiting the topic of this paper to anomalies of
coronary arteries, it will rather be broadened to include anomalies throughout all aspects of the
heart including cardiomyopathies. For the sake of this paper, cardiomyopathies will be grouped
with cardiac anomalies, as they are also a condition that deviates from what is normal or
expected of the cardiac anatomy and function.
Cardiomyopathy is defined as a disease, whether acquired or congenital, that affects the
heart muscle or causes it to be abnormal.69 The affects to the heart muscle make it difficult for
the heart pump effectively enough to deliver blood to the body and can eventually lead to heart
failure.6 The disease often causes the heart muscle to become thickened and stiff making
contractions harder.8 As the disease progresses, the heart becomes weaker and less blood is
pumped through the body. That weakening can also cause valvular issues which can lead to
significant problems for the patient.8 Symptoms of a cardiomyopathy include fatigue, dizziness,
swollen lower extremities, shortness of breath with exertion, chest pain and/or arrhythmia.7 In
most cases it is a treatable condition, however the type of treatment depends solely on the type of
cardiomyopathy. Treatment options generally include lifestyle changes, medication, implantable

7
devices or possibly a heart transplant in severe cases of the condition.6,8 There are five main types
of cardiomyopathies including dilated, hypertrophic, restrictive, arrhythmogenic right ventricular
dysplasia and unclassified.6,7 In this organization, cardiomyopathies are described based on the
condition of the heart, however they can also be organized into primary and secondary
cardiomyopathies. A primary cardiomyopathy is one that a doctor is unable to find a specific
cause of the symptoms.7 A secondary cardiomyopathy is the exact opposite in which it doesnt
have a specific cause whether that be an ischemic cause, with chronically low oxygen to the
heart muscle due to coronary artery disease, or a non-ischemic cause.7 Refer to Table 2 for other
names for the common cardiomyopathies. All types of cardiomyopathies will be discussed in the
next section except for arrhythmogenic right ventricular dysplasia due to its rare nature.
Categories of Cardiomyopathies
Dilated Cardiomyopathy
The most common type of cardiomyopathy is dilated cardiomyopathy (DCM).7,10 It is a
type of non-ischemic cardiomyopathy characterized by a stretching of the heart muscle causing
the affected chamber, usually the left ventricle, to become enlarged, thinner and weaker.10 The
heart muscle stretches in order to fill with more blood and strengthen the weakened contractions
that push the blood through the systemic circulation. While this method seems to work at first,
eventually it causes the heart to become even weaker due to hypertrophy, decreasing the strength
of the chamber contractions which cause impaired systolic function.9,10 Without forceful
contractions to move blood through the body, blood backs up into the lungs or venous system.7
At this point the kidneys respond by retaining more fluid and sodium which contributes to
swollen lower extremities. The body then becomes congested and the development of congestive
heart failure or an arrhythmia is likely.10 The cause of dilated cardiomyopathy is often unknown
due to a range of diseases, conditions and substances that can contribute including alcohol,
toxins, coronary artery disease, myocardial infarction, diabetes and illegal drugs.8
Hypertrophic Cardiomyopathy
In hypertrophic cardiomyopathy, the heart muscle becomes thickened due to hypertrophy
and because of the increased thickness, blocks the flow of blood through the heart. In this case,
hypertrophic cardiomyopathy is usually caused by a hereditary agent or a variety of mutations
causing hypertrophy.7,9 This type of cardiomyopathy causes left ventricular volume to either be
normal or reduced along with the presence of diastolic dysfunction. Athletes heart is an example
of a hypertrophic cardiomyopathy, which can be described as a physiological increase in left
ventricular wall thickness due to the type of training and conditioning that athletes go through.

8
The intensive nature of the training causes the wall to not only become thickened, but also an
increase in the cavity size and mass can be seen.9
Restrictive Cardiomyopathy
In cases of restrictive cardiomyopathy, the heart muscle becomes stiff and rigid, making it
hard for the ventricles to relax and adequately fill will blood, however the walls of the heart
never become thickened or enlarged.7,8 It is characterized by nondilated ventricles, impaired
ventricular filling and the absence of hypertrophy.9 As progression of the cardiomyopathy
continues, the heart muscle weakens and the ventricles lose the ability to pump efficiently which
can lead to heart failure and other valvular issues.8 The cause of restrictive cardiomyopathy is
thought to be another disease or condition. Some of the suspected conditions include,
amyloidosis which causes a buildup of proteins in the heart and other organs, hemochromatosis
in which there is an excess of iron in the body which can be toxic to the organs of the body
including the heart, and lastly sarcoidosis which causes inflammation of numerous organs and
small lumps of cells to grown in the heart.8
Unclassified Cardiomyopathy
Like the name indicated, unclassified cardiomyopathies are essentially all other types of
cardiomyopathies. These are the types that dont necessarily fit into any of the other categories
due to their specific phenotype.9 Common unclassified cardiomyopathies include left ventricular
noncompaction where the heart muscle projects inside of the ventricle, and stress-induced
Takotsubo cardiomyopathy, the latter of which we will talk about in great detail.8
Common Cardiac Anomalies
Acquired
Acquired cardiac anomalies are those to which the patient is not born with, but develops
throughout their lifetime due to a condition or factor.
Takotsubos Syndrome
Takotsubos syndrome is a type of acquired cardiomyopathy grouped under unclassified
cardiomyopathies due to the fact that it is stress-induced.11 It is more commonly known as
broken heart syndrome, seeing as many patients present with clinical symptoms and the
condition after the loss of a loved one, a time that can induce excessive stress on both the patient
emotionally and the patients heart physically. The condition simulates a heart attack due to the
seemingly identical clinical symptoms they both present including chest pain and shortness of
breath. However, Takotsubos syndrome shows no blocked arteries or lesions, only heart muscle
failure. The symptoms of broken heart syndrome are initiated by significant emotional or
physical stress such as grief or anger which researchers believe releases hormones that

9
temporarily affect normal heart function.11 The effects are described as a stun because its only
temporary and although the heart failure is severe, its short-term and most patients have a full,
quick recovery with no lasting damage.11 The muscle failure can be seen during a left
ventriculogram when contrast is injected quickly into the left ventricle and watched as the
ventricle contracts. Tako tsubo is Japanese for octopus trap because the bulging shape
resembles that of the left ventricle due to the weakness of the wall muscle.11
Non-Ischemic
Non-ischemic cardiomyopathy is another ways to classify cardiomyopathies other than
by the description of the actual condition as was done in the previous sections of the types of
cardiomyopathies. The characteristics of non-ischemic cardiomyopathy are just as the name
suggests: a condition not caused by a chronic lack of oxygen supply to the heart due to coronary
artery disease, rather it is caused by another causative factor that is often connected o diseases
of other organs.7 Two non-ischemic cardiomyopathies that will be discussed in this section are
drug induced cardiomyopathy, including methamphetamine and cocaine, and alcoholic
cardiomyopathy.
Alcoholic cardiomyopathy (ACM) is not triggered within a short period of time as
Takotsubos syndrome is, rather shows onset after excessive alcohol consumption over an
extended period of time. Although a low to moderate intake of alcohol on daily basis has proven
to reap benefits on the cardiovascular system, excessive daily intake has the potential to cause
cardiac dysfunction and/or heart failure that may be attributed to structural changes of cardiac
muscle cells.12 Studies have shown that under electron microscope investigation, cardiac muscle
cells in patients with high alcohol intake present with changes such as intracellular swelling,
mitochondria alteration, fibrosis and variations in cell size, all of which are also seen in
idiopathic dilated cardiomyopathy which can make deciphering between the two difficult.12
Excessive in the case of alcohol consumption is defined to be around 80 grams per day, over 7-8
drinks, while long-term is defined as greater than 5 years of daily drinking.13,14 Alcoholic
cardiomyopathy is currently one of the leading cause of non-ischemic dilated cardiomyopathy
that occurs in two stages, asymptomatic or the preclinical stage and a symptomatic stage.13 The
heavy consumption of alcohol causes a characteristic dilated left ventricle, an increase in left
ventricle mass and subsequent left ventricle systolic dysfunction, which are hallmarks of
acquired dilated cardiomyopathy.13 The left ventricular wall thickness can be found to either be
reduced or normal in patients with alcoholic cardiomyopathy, it all depends on the stage of the

10
disease.15 Patients with alcoholic cardiomyopathy and additional comorbidities may be
predisposed to develop heart failure than a patient with just the cardiomyopathy.16 ACM was seen
in both men and women alike but women have an increased sensitivity to cardiac toxicity
causing them to develop the condition at a much lower dose than of men.13 This was discovered
when comparing left ventricle ejection fraction (EF), which shows how much blood is being
pumped out with each contraction of the left ventricle, and it was found that women had a lower
EF than men.13
Another form of non-ischemic cardiomyopathy can be caused by drugs, most commonly
methamphetamine and cocaine, although there are several other drugs that can cause non-
ischemic cardiomyopathy including chemotherapy drugs and methadone. Methamphetamines are
sympathomimetic amines drugs that cause similar effects to endogenous agonists which are
naturally produced by the body targeting the sympathetic nervous system.17 The effects of
sympathomimetic amines are used to treat conditions such as cardiac arrest, which sheds light on
the reason why they can cause further damage to the heart. The drugs increase intra-synaptic
levels of monoamines, such as dopamine, serotonin and noradrenaline, through several
mechanisms, one of which can inhibit the reuptake and degradation of the drug.17 The effect of
these types of drugs manifest themselves as hypertension, dissections, and meth-related
cardiomyopathies which contribute to the statistic that cardiovascular complications are the
second leading cause of death in meth users.17 The use of methamphetamine can trigger the onset
of cardiomyopathy along with an increase in risk, severity and frequency of side effects that tend
to be more severe than those of non-users.18,19 For example, patients who are known to have a
history of meth use have shown increased frequency of moderately or severely reduced ejection
fractions, where an ejection fraction less than or equal to 40% has a strong correlation to the
likelihood of hospitalization or even death due to heart failure.19 One determinant of severity of
the condition is by the state of the patients left ventricular dysfunction which contributes to the
effectiveness of the chambers contraction which can affect the ejection fraction.19In an
experiment studying the effects of meth injected into animals, hypertension, tachycardia, and
myocardial toxicity with cellular death were commonly seen as a result.17 While both
methamphetamine and cocaine are known to cause cardiomyopathy alone, when taken together
the risks of cardiac toxicity greatly increase.17
In the case of cocaine, similar affects are seen including chronic adrenergic stimulation
and inhibited reuptake of norepinephrine and dopamine which can cause symptoms such as

11
hypertension, ischemia, increased heart rate, and ultimately the development of cocaine-related
cardiomyopathy.20 Cocaine, like methamphetamine, has a depressive effect on left ventricular
dysfunction, but has also been known to decrease calcium concentrations, which can reduce
myocardial function, and increase left ventricular mass.20 The good news about both meth-related
and cocaine-related cardiomyopathies is that they are proven to be reversible if abstinence from
the drug is strictly enforced.17,19,20 However, if it is not, the reoccurrence of cardiomyopathy
manifestations is high.21
Mitral Valve Stenosis
Mitral valve stenosis can be both an acquired or congenital condition that occurs when
the mitral valve that connects the left atrium to the left ventricle somehow obstructs the flow of
blood, triggering a domino effect of other sequelae.2 In the case of acquired mitral stenosis, the
condition has onset with the acquisition of another illness, most often rheumatic fever.
Rheumatic fever is an illness that can occur following an upper respiratory tract infection or as a
complication of group A streptococcal infection to genetically susceptible patients.2 Other causes
of acquired mitral stenosis exist including carcinoid causes, systematic lupus erythematosus, and
rheumatoid arthritis which are rare in most cases but all causes produce diffuse inflammation of
the connective tissue as a consequence of the disease.2 Specifically with rheumatic fever, the
leaflets of the valve acquire fibrous scarring and calcification that ultimately decrease orifice size
needed to effectively move blood from the left atrium to the left ventricle.2 Population
distribution of acquired mitral stenosis shows that the condition affecting those with a single
recessive gene that predisposes them to acquiring rheumatic fever via a group A streptococcal
infection.2 Rheumatic fever primarily affects children however the incidence of mitral valve
stenosis doesnt appear until around the age of 15-20 years old due to the progression of the
disease.2
When the mitral valve orifice size is decreased, the most immediate consequence is
reduced blood flow into the left ventricle from the left atrium.2 The reduction of blood flow to
the left ventricle is significant because it then reduces the amount of newly oxygenated blood
that is being delivered to the systemic circulation with each ventricular contraction. While blood
flow to the left ventricle has been reduced due to the valve size, the blood that would normally
be pushed into the ventricle is now backing up into the pulmonary system from the left atrium
and causing increased left atrial pressure.2 The pressure moves through to the pulmonary veins
and capillaries causing pulmonary congestion due to the elevated hydrostatic pressure, forcing

12
fluid from the capillaries to the interstitial space and pulmonary hypertension develops.2 The
right ventricle is soon affected when the afterload increases due to pulmonary hypertension.2
Consequently, tricuspid valve regurgitation and right ventricular hypertrophy can occur.2 Mitral
valve stenosis can cause a wide range of associated issues that affects numerous organs once
cardiac output is reduced, especially with a severe case.2 Refer to Table 3 for a list of resulting
conditions associated with decreased cardiac output due to severe mitral valve stenosis.2
When mitral stenosis is left untreated, the outcome for the patient can be devastating.
Prognosis for untreated patients is a 10-year survival rate for 50-60% of patients, however as
symptoms develop, the prognosis worsens, moving from a 10-year survival rate to a 1-year
survival rate for 15% of patients if left untreated.2 If the condition is left untreated, there are
numerous complications that can occur including pulmonary edema, renal insufficiency, atrial
arrhythmias and thromboembolic events.2
Treatment of mitral valve stenosis is primarily dependent on the severity of the condition.
It can either be by medical or surgical intervention, however surgical is the most common option
in the case of symptomatic moderate to severe mitral stenosis patients, especially when the
resulting congestive heart failure is unresponsive to medical management.2 Medication
management for mild to moderate cases can include diuretics to help reduce symptoms of
congestive heart failure, antiarrhythmic medications for any episodes of atrial fibrillation or
flutter, an anticoagulant for potential thromboembolic events and statins, a
hydroxymethylglutaryl-coenzyme A reductase inhibitor, that has shown to reduce the progression
of acquired mitral stenosis.2 The surgical route can either be done via percutaneous mitral valve
balloon valvuloplasty, expansion of the valve via balloon which can be done in the cardiac
catheterization suite, valvotomy, removal of fused commissural cusps and thinning of leaflets,
and valve replacement with either a mechanical or prosthetic valve.2
Aortic Valve Stenosis
Like mitral valve stenosis, acquired aortic valve stenosis is the narrowing of the aortic
valve causing an outflow obstruction from the left ventricle into the aorta.22,23 The stenotic valve
causes heart to have to work harder in order to push the normal volume of blood out of the left
ventricle, which will eventually weaken the heart muscle, limiting the amount of blood that can
be pumped out further.22 That weakening of the heart is what can cause heart failure manifested
by fatigue, shortness of breath and swollen ankles.22 There are several causes of acquired aortic
stenosis including rheumatic fever which is also a cause of mitral stenosis, and calcium build up

13
on valve cusps which manifest symptoms of heart failure in addition to chest pain, tightness, and
heart palpitations.22 Complications begin to occur with aortic stenosis when the left ventricle wall
thickens and enlarges in an attempt to pump the higher volume of blood left in the chamber more
efficiently using more force.22 However, that compensation method only lasts for a short period
of time before the left ventricle hypertrophies.22 The methods used to treat aortic stenosis are
very similar to those used for mitral stenosis, only the area of focus is different. Surgical options
include balloon valvuloplasty and transcatheter aortic valve replacement or TAVR, both of which
are performed by an interventional cardiologist in the cardiac catheterization suite, along with
surgical valvuloplasty and aortic valve replacement which are done in the operating room.22
Inherited/Congenital
Congenital cardiac anomalies describe conditions to which the patient is born with,
passed on by a parents gene for the disease.
Coronary Artery Anomalies
Affecting about one percent of the general population, coronary artery anomalies are very
rare.3 However, no matter how rare they are, they should still be considered especially during a
cardiac catheterization procedure when the cardiologist has difficulty locating a coronary artery
or a portion of the myocardium is missing normal perfusion.4 It is a common error to assume that
the vessel that is missing is just occluded, as it can affect the treatment options that are pursued,
therefore it is the angiographers responsibility to visualize the origin and course of the
anomalous vessel.4 The corresponding risk of the coronary anomaly related to its location and
pathway from the origin to its perfusion area.3 There are five different pathways that an artery
can take from its origin within the contralateral sinus of Valsalva to its ultimate destination: pre-
pulmonic, retro-aortic, inter-arterial, trans-septal and retro-cardinal with inter-arterial being the
most dangerous due to its path between the pulmonary artery and the aorta. Coronary artery
anomalies with an inter-arterial course have the highest potential for causation of sudden cardiac
death in young adults, along with other manifestations of chest pain, shortness of breath,
myocardial infarction and pulmonary edema.4 The proposed mechanism for the lethalness of an
inter-arterial course is the compression of the anomalous artery by the aorta and pulmonary trunk
during strenuous activity when cardiac output is high which cuts off flow to one of the main
coronary arteries, causing ischemia to it corresponding myocardium.4,24 An anomalous coronary
artery is found 12-33% of time in young adults or athletes with sudden cardiac death presenting
with chest pain and syncope with exercise.25 Unfortunately the sudden cardiac death is often the

14
first symptom with no other clinical manifestations prior to the event.25 Below is an overview of
several of the most common coronary artery anomalies that are seen in cardiac catheterization
labs and across the United States.
Split RCA
A split right coronary artery or double RCA is the most common coronary artery
anomaly with 1.23% incidence.3 The name can be a bit misleading as there are not actually two
right coronary arteries, rather the posterior descending artery is just split into two different
sections.3 The sections take different routes, with an anterior portion perfusing the anterior free
wall of the right ventricle while the posterior bifurcation completes a normal route, through the
atrioventricular groove, making up the upper portion of the posterior descending artery branch.3
LMCA from RSV
In relation to the other anomalies presented in this article, the left main coronary artery
origin from the right sinus of Valsalva is very rare, with only a 0.02-0.05% incidence.3 In the
case of this anomaly, the entire left coronary system stems from the right sinus with either an
ostium separate or shared with the right coronary artery.3 Depending on the course of this
anomaly, it can either be benign, with an anterior course, or malignantly lethal, with an inter-
arterial course.3 This coronary anomaly, along with the anomalous origin of the right coronary
artery from the left coronary sinus, is the most common cause associated with sudden cardiac
death, most often found in patients after physical exertion.25
RCA from LSV
This presents as one of the most common anomalies to cause sudden cardiac death in
young athletes.25 It occurs when the right coronary artery develops from the left cusp or the
proximal left main coronary artery, following a dangerous path between the aorta and the
pulmonary trunk.4 It presents with symptoms including chest pain, dizziness, myocardial
infarction and ventricular tachycardia with the absence of atherosclerosis during a cardiac
catheterization proceudure.3 Due to its dangerous course, this anomaly is considered malignant
and should treatment via revascularization should be seriously considered when the patient is
presenting wiht myocardial infarction symptoms.4
Separate Origins of LCX and LAD
In the case of the left circumflex and the left anterior descending artery, there is no left
main coronary artery, therefore the LCX and LAD both have separate, but adjacent ostia from
the left sinus of Valsalva.3 It is seen with 0.41-0.67% incidence, increasing with the development
of aortic valve disease or left system dominance.3 The only time there could be potential
consequences of this anomaly would be in the case of surgery or cardiac catheterization if not

15
previously recognized.3 For this reason it is necessary to cannulate the separate ostia of both the
LCX and LAD during a cardiac catheterization procedure, that way it is a confirmed anomaly
rather than a potential occlusion.4 Other than the anomalous origin of the coronary arteries, the
vessels maintain a relatively normal course and pattern, causing no defects in hemodynamics.3 In
most cases this coronary anomaly should be considered benign.3
Mitral Valve Stenosis
The difference between acquired and congenital mitral valve stenosis is not the
pathophysiology of the disease, but instead the morphology of it. As acquired mitral stenosis
causes an obstruction of blood to the left ventricle, so does congenital mitral stenosis, only the
resulting formation of the mitral valve following fetal development can take many forms.26 These
forms include hypoplasia of the valve, fusion of the commissural cusps, a double orifice valve,
shortened or thickened chordae tendinae and a parachute mitral valve.26 The pathophysiology and
complications of mitral stenosis are the same between acquired and congenital as the process is
still the same, only the development of the disease is different.26 Most cases of severe congenital
mitral stenosis present shortly after birth showing signs of pulmonary edema and respiratory
distress.26 Cases with only mild to moderate mitral stenosis present post-neonatal period with low
cardiac output and right ventricular failure in the form of exhaustion with feeding, pulmonary
infections, and chronic cough.26 In rare cases, the congenital mitral stenosis doesnt show up until
later in childhood when the child experiences dyspnea upon exertion.26 While still very rare, the
prognosis for a newborn with congenital mitral stenosis is not very good.26 The necessary
surgical intervention is put off for as long as possible due to the high-risk nature of the surgery.26
Studies have shown that while early mitral valve replacement can save lives for those with
severe cases of the disease, there are also palliative ways to manage the disease while the child
develops further, allowing for a larger and more anatomically fitting prosthetic valve to be put
in.27 This disease is one that could be found during a pediatric cardiac catheterization or through
a cardiac sonogram.
Aortic Valve Stenosis
As congenital mitral stenosis really only has changes in the development and morphology
of the valve, not so much the pathophysiology of the disease, so does congenital aortic stenosis.
In the congenital form, children are born with one, two or four valve cusps rather than the normal
three cusps, producing a wide range of complications for the child including arrhythmias and
dyspnea.22,23 Having a lower count of cusps than normal, cause more force and workload on the

16
one or two cusps, causing them to wear out more easily.22 There is also the possibility that the
patient may not present with any symptoms until early into adulthood, when the cusps begin to
narrow or leak due to years of overworking.22 The issue of too few cusps becomes greater when
calcium begins to build on the already congenitally deformed valves, causing them to become
stiff, narrowing the orifice of the valve and limiting the blood flow from the left ventricle as it
occurs in acquired aortic stenosis.22 The formation of calcium on the valve cusps may sound like
it should be classified as an acquired disease, because the cusps were damaged at birth due to
developmental issues, it is classified as a congenital disease; the calcification comes only
secondary to the congenital deformity. The primary risk factor in the case of congenital aortic
stenosis is genetics, which can cause a deformity of the aortic valve to run in the family.22 The
treatment options for cases of congenital aortic stenosis are the same as acquired aortic stenosis,
which can be performed in the cardiac catheterization suite depending on the treatment option
selected, while also following the precautions highlighted in the congenital mitral valve section.
Surgical intervention of an aortic valve is still a high- risk procedure for young children, and
therefore is still pushed back until necessary in order to preserve the original aortic valve and left
ventricular function.28
Authors Biography
Lindsey Walker is a senior at the University of North Carolina-Chapel Hill. She is majoring in
Radiologic Science with a modality focus in Cardiac Catheterization. She is a member of Tau
Sigma, a national honor society for transfer students, the events chair for Tar Heel Transfers, an
outreach organization for fellow transfer students and enjoys playing intermural soccer, reading,
and traveling in her spare time.
Bibliography

1. Saladin KS, McFarland RK. The Circulatory System II. In: Essentials of Anatomy &
Physiology. McGraw Hill; 2014:418-463.

2. Horenstein MS. Acquired Mitral Stenosis: Background, Pathophysiology, Prognosis.

Medscape 2014. Available at: http://emedicine.medscape.com/article/902351-overview.
Accessed February 28, 2017.

3. Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A. Coronary artery

anomalies overview: The normal and the abnormal. World J Radiol 2016;8(6):537-555.
doi:10.4329/wjr.v8.i6.537.

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4. Kern MJ, Sorajja P, Lim MJ. The Cardiac Catheterization Handbook. 6th ed. Elsevier;
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5. Young M-L, McLeary M, Chan K-C. Acquired and congenital coronary artery
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6. Cardiomyopathy - Mayo Clinic. Available at: http://www.mayoclinic.org/diseases-

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7. Heart Failure | Second Opinion. Available at: https://www.secondopinion-

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https://www.nhlbi.nih.gov/health/health-topics/topics/cm. Accessed March 27, 2017.

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10. Dilated Cardiomyopathy Treatment, Causes & More | Cleveland Clinic. Cleveland Clinic.
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March 30, 2017.

11. What is Broken Heart Syndrome - NHLBI, NIH. Available at:

https://www.nhlbi.nih.gov/health/health-topics/topics/broken-heart-syndrome. Accessed
March 26, 2017.

12. Guzzo-Merello G, Cobo-Marcos M, Gallego-Delgado M, Garcia-Pavia P. Alcoholic

cardiomyopathy. World J Cardiol 2014;6(8):771-781. doi:10.4330/wjc.v6.i8.771.

13. Alcoholic cardiomyopathy - UpToDate. Available at:

https://www.uptodate.com/contents/alcoholic-cardiomyopathy?
source=search_result&search=drug%20cardiomyopathy%20adult&selectedTitle=20~150.
Accessed March 3, 2017.

14. Rezaei-Adaryani M, Ahmadi F, Mohamadi E, Asghari-Jafarabadi M. The effect of three

positioning methods on patient outcomes after cardiac catheterization. J Adv Nurs
2009;65(2):417-424. doi:10.1111/j.1365-2648.2008.04889.x.

15. Mili S, Luli D, timac D, Rui A, Zaputovi L. Cardiac manifestations in alcoholic

liver disease. Postgrad Med J 2016;92(1086):235-239. doi:10.1136/postgradmedj-2015-
133755.

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16. Piano MR. Alcoholic cardiomyopathy: incidence, clinical characteristics, and
pathophysiology. Chest 2002;121(5):1638-1650.

17. Paratz ED, Cunningham NJ, MacIsaac AI. The cardiac complications of
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18. Methamphetamine Use and Cardiomyopathy - Drug Addiction Treatment. Available at:
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19. Neeki MM, Kulczycki M, Toy J, et al. Frequency of methamphetamine use as a major
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20. Cocaine-Related Cardiomyopathy: Overview, Cardiac Effects of Cocaine, Epidemiology.

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23. Otto CM. Clinical manifestations and diagnosis of aortic stenosis in adults. UpToDate
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diagnosis-of-aortic-stenosis-in-adults. Accessed March 27, 2017.

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http://emedicine.medscape.com/article/897204-overview. Accessed March 26, 2017.

25. Oppido G, Davies B, McMullan DM, et al. Surgical treatment of congenital mitral valve
disease: midterm results of a repair-oriented policy. J Thorac Cardiovasc Surg
2008;135(6):1313-20; discussion 1320. doi:10.1016/j.jtcvs.2007.09.071.

Table 11,3

Coronary Artery Branches Area of Supply

Left Coronary Artery
a. Anterior a. Both ventricles and
interventricular/ left anterior two-thirds of
anterior descending septum.
(LAD) - follows b. To the left ventricle
straight down the and left atrium.

19
 anterior portion of the
heart, to the apex, and
slightly curves up the
posterior side.
b. Circumflex curves
around the left side,
then branches off to
the left (obtuse)
marginal artery and
posterior artery of left
ventricle.
Right Coronary Artery a. Right atrioventricular a. AV node
nodal branch- b. Right atrium and right
branches on the ventricle.
anterior surface of the c. Posterior wall of left
heart, moving towards and right ventricle and
the middle of the heart posterior portion of
b. Right marginal- interventricular
branches prior to the septum.
right diaphragmatic
curve; wraps around
slightly to the side and
then runs towards the
apex.
c. Posterior
interventricular artery/
Posterior descending
artery (PDA)- wraps
around to the posterior
side and then runs
down to the apex.

20
Table 28

Table 32

Consequences of decreased cardiac output End organ failure with shock

Metabolic acidosis
Renal insufficiency
Hepatic insufficiency
Consequences of Systemic Venous Hepatomegaly
Congestion due to Right Ventricular Failure Ascites
Pedal edema

Table 1: Branches of the Coronary Arteries and Corresponding Supply

Saladin KS, McFarland RK. The Circulatory System II. In: Essentials of Anatomy & Physiology.
McGraw Hill; 2014:418-463.
Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A. Coronary artery anomalies
overview: The normal and the abnormal. World J Radiol 2016;8(6):537-555.
doi:10.4329/wjr.v8.i6.537.

Table 2: Other Names for the Types of Cardiomyopathies

What Is Cardiomyopathy? - NHLBI, NIH. Available at: https://www.nhlbi.nih.gov/health/health-
topics/topics/cm. Accessed March 27, 2017.
Table 3: Conditions associated with decreased cardiac output due to severe mitral valve stenosis

21
Horenstein MS. Acquired Mitral Stenosis: Background, Pathophysiology, Prognosis. Medscape
2014. Available at: http://emedicine.medscape.com/article/902351-overview. Accessed
February 28, 2017.

Quiz Questions

1. Which coronary artery has no major branches but rather a bifurcation?

a. Left main coronary artery
b. Right coronary artery
c. Left circumflex artery
d. Left anterior descending artery

2. What object is the left atrioventricular or mitral valve named after?

a. a hat or headdress
b. an instrument or tool
c. a surgical technique
d. a scientist

3. An absolute contraindication for performing a cardiac catheterization procedure is _________.

a. an acute GI bleed
b. renal failure
c. an uncooperative patient
d. inadequate equipment and facility

4. What percent of the population must have a morphological abnormality in order for it to be
considered a normal deviation from anatomy?
a. 50%
b. less than 1%
c. greater than 1%
d. 20%

5. Where does the blood flow directly after it leaves the right ventricle?
a. the pulmonary veins
b. the pulmonary semilunar valve
c. the pulmonary arteries
d. the aortic semilunar valve

6. Which type of drug is used to slow the progression of mitral stenosis?

a. calcium-channel blockers
b. statins
c. antiarrhythmics
d. anticoagulants

22
7. What is the main difference between acquired and congenital mitral stenosis?
a. morphology
b. pathophysiology
c. prognosis
d. surgical treatment options

8. There are six different pathways that an anomalous coronary artery can take from its origin on
the contralateral sinus of Valsalva to it perfusion area.
a. True
b. False

9. What is another name for the split right coronary artery anomaly?
a. Two RCA
b. Double PDA
c. Double RCA
d. Two PDA

10. How often is an anomalous coronary artery found in a sudden cardiac death patient?
A. 12-33%
b. 50%
c. 20%
d. 75%

Answer Key

1. B
2. A
3. D
4. C
5. B
6. B
7. A
8. B
9. C
10. A

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