Pathophysiology:

An atial septal defect (ASD) is an abnormal opening in the septal wall between the two atria.
This opening or communication allows blood to shunt from higher pressure left atrium to the
lower pressure right atrium (a.k.a. left-to-right shunt). This extra blood will cause a volume
overload of both the right atrium and the right ventricle. People with ASD are usually
asymptomatic. Some children will experience shortness of breath with activity as they grow
older because extra blood shunts through the ASD into the right side of the heart and into the
pulmonary circulation.

Any process that increases the pressure in the left ventricle can cause worsening of the left-to-
right shunt. This includes hypertension, which increases the pressure that the left ventricle has to
generate in order to open the aortic valve during ventricular systole, and coronary artery disease
which increases the stiffness of the left ventricle, thereby increasing the filling pressure of the
left ventricle during ventricular diastole.

The right ventricle will have to push out more blood than the left ventricle due to the left-to-right
shunt. This constant overload of the right side of the heart will cause an overload of the entire
pulmonary vasculature. Eventually the pulmonary vasculature will develop pulmonary
hypertension to try to divert the extra blood volume away from the lungs.

The pulmonary hypertension will cause the right ventricle to face increased afterload in addition
to the increased preload that the shunted blood from the left atrium to the right atrium caused.
The right ventricle will be forced to generate higher pressures to try to overcome the pulmonary
hypertension. This may lead to right ventricular failure (dilatation and decreased systolic
function of the right ventricle) or elevations of the right sided pressures to levels greater than the
left sided pressures.

When the pressure in the right atrium rises to the level in the left atrium, there will no longer be a
pressure gradient between these heart chambers, and the left-to-right shunt will diminish or
cease.

If left uncorrected, the pressure in the right side of the heart will be greater than the left side of
the heart. This will cause the pressure in the right atrium to be higher than the pressure in the left
atrium. This will reverse the pressure gradient across the ASD, and the shunt will reverse; a
right-to-left shunt will exist. This phenomenon is known as Eisenmenger's syndrome.

Once right-to-left shunting occurs, a portion of the oxygen-poor blood will get shunted to the left
side of the heart and ejected to the peripheral vascular system. This will cause signs of cyanosis.
 Congenital

Opening/fissure between left and right

atrium.

Left-to-right shunt

Right ventricle pushes more blood

Overloading of pulmonary
Vasculature

Pulmonary hypertension

Right ventricle: Increase preload and afterload.

Right Ventricular Failure

Pressure in the right atrium rises to the level in the left

atrium,
Ceased/ diminished Left-to-
right shunt

Right-to-left shunt

Poor Oxygen shunt blood

Cyanosis