 What causes increased pulmonary blood flow?

Elevated pulmonary artery pressure in congenital heart disease

is caused by pulmonary overcirculation, pulmonary vasoconstriction,
and pulmonary vascular disease, either alone or in combination. One of the
most common defects associated with elevated pulmonary pressure is a
large ventricular septal defect (VSD).
Prolonged large left-to-right shunt leads to gradual increase in
the pulmonary pressure and pulmonary hypertension eventually develops.
As the pressure difference between the systemic and pulmonary systems
decreases, the flow across the shunt also decreases.

 What is the difference between a left to right shunt and a right to

left shunt?
A shunt is an abnormal communication between the right and left sides of
the heart or between the systemic and pulmonary vessels, allowing blood
to flow directly from one circulatory system to the other. A right-to-left
shunt allows deoxygenated systemic venous blood to bypass the lungs and
return to the body.

 ATRIAL SEPTAL DEFECT

An atrial septal defect is a birth defect of the heart in which there is a
hole in the wall (septum) that divides the upper chambers (atria) of the
heart. A hole can vary in size and may close on its own or may require
surgery. An atrial septal defect is one type of congenital heart defect.
a baby’s heart develops during pregnancy, there are normally several
openings in the wall dividing the upper chambers of the heart (atria). These
usually close during pregnancy or shortly after birth.

If one of these openings does not close, a hole is left, and it is called an
atrial septal defect. The hole increases the amount of blood that flows
through the lungs and over time, it may cause damage to the blood vessels
in the lungs. Damage to the blood vessels in the lungs may cause
problems in adulthood, such as high blood pressure in the lungs and heart
failure. Other problems may include abnormal heartbeat, and increased risk
of stroke.
  Secundum atrial septal defect (ASDII) is a common congenital
heart defect that causes shunting of blood between the systemic and
pulmonary circulations.

Atrial septal defect, congenital opening in the partition between the two
upper chambers (atria) of the heart. The most common atrial septal
defect is persistence of the foramen ovale, an opening in this partition
that is normal before birth and that normally closes at birth or shortly
thereafter. The opening in the atrial septum results in the flow of blood
from the left atrium to the right, causing enlargement of the right atrium
and ventricle and of the main pulmonary artery. The usual treatment, the
surgical closure of the defect, is sometimes made hazardous by
serious disease of the pulmonary vessels.

 What are the causes of atrial septal defect?

Some congenital heart defects may have a genetic link, either occurring
due to a defect in a gene, a chromosome abnormality, or environmental
exposure, causing heart problems to occur more often in certain families.
Most atrial septal defects occur sporadically (by chance), with no clear
reason for their development.

How the Heart Works

The heart is an organ, about the size of a fist. It is made of muscle and
pumps blood through the body. Blood is carried through the body in blood
vessels, or tubes, called arteries and veins. The process of moving blood
through the body is called circulation. Together, the heart and vessels make
up the cardiovascular system.

Structure of the Heart

The heart has four chambers (two atria and two ventricles). There is a wall

(septum) between the two atria and another wall between the two
ventricles. Arteries and veins go into and out of the heart. Arteries carry
blood away from the heart and veins carry blood to the heart. The flow of
blood through the vessels and chambers of the heart is controlled
by valves.

Blood Flow Through the Heart

(Abbreviations refer to labels in the illustration)

The heart pumps blood to all parts of the body. Blood provides oxygen and
nutrients to the body and removes carbon dioxide and wastes. As blood
travels through the body, oxygen is used up, and the blood
becomes oxygen poor.

1. Oxygen-poor blood returns from the body to the heart through

the superior vena cava (SVC) and inferior vena cava (IVC), the two main
veins that bring blood back to the heart.
2. The oxygen-poor blood enters the right atrium (RA), or the right
upper chamber of the heart.
3. From there, the blood flows through the tricuspid valve (TV) into the
right ventricle (RV), or the right lower chamber of the heart.
4. The right ventricle (RV) pumps oxygen-poor blood through
the pulmonary valve (PV) into the main pulmonary artery (MPA).
5. From there, the blood flows through the right and left pulmonary
arteries into the lungs.
6. In the lungs, oxygen is put into the blood and carbon dioxide is taken
out of the blood during the process of breathing. After the blood gets
oxygen in the lungs, it is called oxygen-rich blood.
7. Oxygen-rich blood flows from the lungs back into the left atrium (LA),
or the left upper chamber of the heart, through four pulmonary veins.
8. Oxygen-rich blood then flows through the mitral valve (MV) into the
left ventricle (LV), or the left lower chamber.
9. The left ventricle (LV) pumps the oxygen-rich blood through
the aortic valve (AoV) into the aorta (Ao), the main artery that takes
oxygen-rich blood out to the rest of the body.

ASD: Diagnostic Testing

Electrocardiogram. In patients with ostium secundum ASD, the

electrocardiogram reveals right axis deviation and
incomplete right bundle branch block. Ostium primum
defects are accompanied by left axis deviation and
atrioventricular nodal conduction delay. The presence of an
ectopic atrial pacemaker is suggestive of a sinus venosus
ASD. With the development of pulmonary hypertension, right
ventricular hypertrophy becomes evident. Atrial arrhythmias
such as atrial fibrillation and supraventricular tachycardia
may appear in patients in the third or fourth decades of life.

Chest Roentgenogram. Prominent pulmonary vascular

markings, right atrial and ventricular enlargement, and
dilation of the pulmonary artery are typical findings in
patients with hemodynamically significant shunting.

Imaging. Echocardiographic findings include right chamber

enlargement and evidence of right ventricular volume
overload. Transthoracic echocardiography (TTE) is the
imaging study of choice for ostium primum and secundum
ASDs. Identification of sinus venosus defects usually requires
transesophageal echocardiography (TEE). Evaluation of the
location, size, and direction of shunt can be facilitated by use
of color flow Doppler and echocardiographic contrast agents.
Important information such as estimated pulmonary artery
pressure and presence of additional cardiac abnormalities
can also be obtained.
TEE is an essential tool in the selection of patients who are
candidates for percutaneous closure of secundum ASDs.
Although still not widely used, magnetic resonance imaging
and computed tomography are emerging imaging modalities
for the evaluation of patients with congenital heart disease.

Cardiac Catheterization. Invasive evaluation becomes

necessary when the results of the noninvasive studies are
inconclusive. It allows estimation of the magnitude of the
shunt (pulmonary to systemic flow ratio, Qp:Qs), and
measurement of the pulmonary artery pressure. Coronary
angiography is recommended in patients with suspected
coronary artery disease and in those over the age of 40
years.
ASD: Treatment

ASD repair is indicated in patients with hemodynamically

significant ASD (Qp:Qs > 1.5:1), in the absence of severe and
irreversible pulmonary hypertension. Consideration for ASD
repair may be given in patients with paradoxical embolism.
Surgical repair is the treatment modality of choice.
Percutaneous closure is the preferred treatment in patients
with ostium secundum ASD and suitable anatomy, in the
absence of other cardiac conditions requiring surgical repair.

The operative mortality is < 1% in the absence of significant

pulmonary hypertension. Surgical repair in young adults (<
25 years) results in long-term survival rates similar to those
of matched controls. Repair in patients older than 40 years
does not eliminate the risk of atrial arrhythmias and
cerebrovascular accidents. Long-term surveillance is
recommended in these patients.

PATHOPHYSIOLOGY
Small ASDs result in trivial shunting and have no
hemodynamic consequences. Larger defects are associated
with substantial shunting, which may lead to volume overload
of the right atrium, right ventricle, and pulmonary arteries.
The magnitude of left-to-right shunting depends on the size
of the ASD, the relative compliance of the 2 ventricles, and
the pulmonary and systemic vascular resistance. If left
untreated, this may result in pulmonary hypertension, right
ventricular failure, decreased right ventricular compliance,
and potentially right-to-left shunting.

Treatments

Treatment for an atrial septal defect depends on the age of diagnosis, the
number of or seriousness of symptoms, size of the hole, and presence of other
conditions. Sometimes surgery is needed to repair the hole. Sometimes
medications are prescribed to help treat symptoms. There are no known
medications that can repair the hole.

If a child is diagnosed with an atrial septal defect, the health care provider may
want to monitor it for a while to see if the hole closes on its own. During this
period of time, the health care provider might treat symptoms with medicine. A
health care provider may recommend the atrial septal defect be closed for a child
with a large atrial septal defect, even if there are few symptoms, to prevent
problems later in life. Closure may also be recommended for an adult who has
many or severe symptoms. Closure of the hole may be done during cardiac
catheterization or open-heart surgery. After these procedures, follow-up care will
depend on the size of the defect, person’s age, and whether the person has other
birth defects.

References

Pathophysiology of an Atrial Septal Defect

To understand an ASD, you must first understand the NORMAL blood flow of the heart!

Therefore, how should blood flow NORMALLY in the heart without an ASD?

Blood flow starts on the RIGHT SIDE of the heart

 The goal of the right side is to get the UNOXYGENATED blood to the LUNGS!
The un-oxygenated blood (this is blood that has been “used-up” by your body that
needs to be resupplied with oxygen) enters to the heart through the SUPERIOR AND
INFERIOR VENA CAVA.

Blood enters into the RIGHT ATRIUM

Then it is squeezed through the TRICUSPID VALVE

Blood then enters into the RIGHT VENTRICLE

Then it is squeezed into the PULMONIC VALVE

Blood is then shot-up through the PULMONARY ARTERY and then enters into the
lungs for some oxygen

Left Side of the Heart

 The goal of the left side of the heart is to get the richly, oxygenated blood to the body to
feed the brain, tissue, muscles, organs etc.
Blood enters from the lungs through the PULMONARY VEIN that has just been
refreshed with oxygen to go into the LEFT ATRIUM

Down through the BICUSPID VALVE (also called mitral valve)

Then blood is squeezed into the LEFT VENTRICLE

Up through the AORTIC VALVE

Lastly up through the AORTA, where it pumped throughout the body

Blood flow through the Atria in a Fetus’ Heart

Now in a baby’s heart, who is still in the womb, there is a natural opening in the
septum called the formen ovale (O-VALLEY).

This opening allows blood from the right atrium to flow into the left atrium. WHY is this
needed in a fetus’ heart?

While a baby is in utero, blood doesn’t need to pass through the lungs because they
don’t work yet….the placenta is providing the oxygen. So, this opening allows the blood
on the right side to bypass the lungs.
However, after birth the formen ovale will close. It does this when the baby starts
breathing own its own. There is a layer of tissue that will seal the opening as the
pressure changes in the heart after the baby starts breathing.

As the baby breathes (hence uses the lungs) the pressure in the right side of the heart
decreases (in the womb the pressure was the same on the right and left side of the
heart).

Therefore, in a normal heart,  the pressure is higher on the left side when compared to
the right. The purpose for this pressure difference is: The left side needs more pressure
to pump all that oxygenated blood throughout the body, while the right side just needs to
pump unoxygenated blood to the lungs.

***Now, in some people the formen ovale does not seal shut after birth, and this is
called a patent formen ovale (PFO). This “usually” doesn’t cause any issues.
However, some patients are at a higher risk for stroke.

Therefore, there are really two types of holes that can occur in the atrial septum: a PFO
or an ASD. A hole that has occurred during the formation of the atrial septum in utero is
called an ASD (these tend to be larger than a PFO), and the other type
occurs AFTER birth when a natural structure (the foramen ovale) fails to CLOSE, which
is a PFO.

VENTRICULAR
A ventricular septal defect (pronounced ven·tric·u·lar sep·tal de·fect) (VSD) is a
birth defect of the heart in which there is a hole in the wall (septum) that
separates the two lower chambers (ventricles) of the heart. This wall also is called
the ventricular septum.

What is a Ventricular Septal Defect

A ventricular septal defect happens during pregnancy if the wall that forms
between the two ventricles does not fully develop, leaving a hole. A ventricular
septal defect is one type of congenital heart defect. Congenital means present at
birth.

In a baby without a congenital heart defect, the right side of the heart pumps
oxygen-poor blood from the heart to the lungs, and the left side of the heart
pumps oxygen-rich blood to the rest of the body.
In babies with a ventricular septal defect, blood often flows from the left ventricle
through the ventricular septal defect to the right ventricle and into the lungs. This
extra blood being pumped into the lungs forces the heart and lungs to work
harder. Over time, if not repaired, this defect can increase the risk for other
complications, including heart failure, high blood pressure in the lungs (called
pulmonary hypertension), irregular heart rhythms (called arrhythmia), or stroke.

Learn more about how the heart works »

Types of Ventricular Septal Defects

Click here to view a larger image

An infant with a ventricular septal defect can have one or more holes in different
places of the septum. There are several names for these holes. Some common
locations and names are (see figure):

1. Conoventricular Ventricular Septal Defect

In general, this is a hole where portions of the ventricular septum should
meet just below the pulmonary and aortic valves.
2. Perimembranous Ventricular Septal Defect
This is a hole in the upper section of the ventricular septum.
3. Inlet Ventricular Septal Defect
This is a hole in the septum near to where the blood enters the ventricles
through the tricuspid and mitral valves. This type of ventricular septal
defect also might be part of another heart defect called an atrioventricular
septal defect (AVSD).
4. Muscular Ventricular Septal Defect
This is a hole in the lower, muscular part of the ventricular septum and is
the most common type of ventricular septal defect.
Occurrence

In a study in Atlanta, the Centers for Disease Control and Prevention (CDC)
estimated that 42 of every 10,000 babies born had a ventricular septal
defect.  This means about 16,800 babies are born each year in the United States
1

with a ventricular septal defect. In other words, about 1 in every 240 babies born
in the United States each year are born with a ventricular septal defect.

Causes and Risk Factors

The causes of heart defects (such as a ventricular septal defect) among most
babies are unknown. Some babies have heart defects because of changes in
their genes or chromosomes. Heart defects also are thought to be caused by a
combination of genes and other risk factors, such as the things the mother comes
in contact with in the environment or what the mother eats or drinks or the
medicines the mother uses.

Read more about CDC’s work on causes and risk factors »

Diagnosis

A ventricular septal defect usually is diagnosed after a baby is born.

The size of the ventricular septal defect will influence what symptoms, if any, are
present, and whether a doctor hears a heart murmur during a physical
examination. Signs of a ventricular septal defect might be present at birth or
might not appear until well after birth. If the hole is small, it usually will close on
its own and the baby might not show any signs of the defect. However, if the
hole is large, the baby might have symptoms, including:

 Shortness of breath,
 Fast or heavy breathing,
 Sweating,
 Tiredness while feeding, or
 Poor weight gain.

During a physical examination the doctor might hear a distinct whooshing sound,
called a heart murmur. If the doctor hears a heart murmur or other signs are
present, the doctor can request one or more tests to confirm the diagnosis. The
most common test is an echocardiogram, which is an ultrasound of the heart that
can show problems with the structure of the heart, show how large the hole is,
and show how much blood is flowing through the hole.

Treatments

Treatments for a ventricular septal defect depend on the size of the hole and the
problems it might cause. Many ventricular septal defects are small and close on
their own; if the hole is small and not causing any symptoms, the doctor will
check the infant regularly to ensure there are no signs of heart failure and that
the hole closes on its own. If the hole does not close on its own or if it is large,
further actions might need to be taken.

Depending on the size of the hole, symptoms, and general health of the child, the
doctor might recommend either cardiac catheterization or open-heart surgery to
close the hole and restore normal blood flow. After surgery, the doctor will set up
regular follow-up visits to make sure that the ventricular septal defect remains
closed. Most children who have a ventricular septal defect that closes (either on
its own or with surgery) live healthy lives.

Medicines
Some children will need medicines to help strengthen the heart muscle, lower
their blood pressure, and help the body get rid of extra fluid.

Nutrition
Some babies with a ventricular septal defect become tired while feeding and do
not eat enough to gain weight. To make sure babies have a healthy weight gain,
a special high-calorie formula might be prescribed. Some babies become
extremely tired while feeding and might need to be fed through a feeding tube.