Pathophysiology of

Left-to-Right Shunt Lesions

Before discussing the hemodynamic abnormalities of left-to-right shunt lesions, knowledge
of the model that will be used throughout this section is helpful. Figure 9-1 is a
block diagram of a normal heart in which one arrow represents a “unit” of normal cardiac
output. It is assumed that the cardiac chambers and great arteries and veins indicated
by one arrow are normal in size. If a cardiac chamber or great artery has more than one
arrow in it, that chamber or blood vessel is going to be dilated. A diagram of a normal
cardiac roentgenogram is presented in Chapter 4 (see Fig. 4-2). Modifications in appearance
of chest roentgenogram secondary to enlargement or reduction of cardiac chambers
or great vessels are presented in diagrammatic drawings to aid in the interpretation of
chest radiograph films.

Atrial Septal Defect

In acyanotic patients with atrial septal defects (ASDs), the direction of the shunt is from
left to right and the magnitude of the left-to-right shunt is determined by the size of
the defect and the relative compliance of the right ventricle (RV) and left ventricle (LV).
Because the compliance of the RV is greater than that of the LV, a left-to-right shunt is
present. The magnitude of the shunt is reflected in the degree of cardiac enlargement.
Let it be assumed that there is a left-to-right shunt of one arrow at the atrial level. As
seen in Figure 9-2, the right atrium (RA), RV, and main pulmonary artery (PA) and its
branches have two arrows and are therefore dilated. These findings are translated into
the chest radiographs (Fig. 9-3), which reveal enlargement of the RA, RV, and PA, as
well as an increase in pulmonary vascular markings. Note that the left atrium (LA) is
not enlarged (see Figs. 9-2 and 9-3). This is because the increased pulmonary venous
return to the LA does not stay in that chamber; rather, it is shunted immediately to the
RA. The absence of LA enlargement is one of the helpful radiographic signs for differentiating
an ASD from a ventricular septal defect (VSD) in patients with increased
pulmonary vascularity.
The dilated RV cavity prolongs the time required for depolarization of the RV because
of its longer pathway, producing either complete or incomplete right bundle branch block

(RBBB) pattern (with rsR′ in V1) in the electrocardiogram (ECG). The RBBB pattern in children

with ASDs is not the result of actual block in the right bundle. If the duration of the
QRS complex is not abnormally prolonged, the ECG may be read as mild right ventricular
hypertrophy (RVH). Therefore, either (complete or incomplete) RBBB pattern or mild RVH
is seen on the ECG of children with ASD.
The heart murmur in ASD is not caused by the shunt at the atrial level. Because the
pressure gradient between the atria is so small and the shunt occurs throughout the cardiac
cycle, both in systole and diastole, the left-to-right shunt is silent. The heart murmur in
ASD originates from the pulmonary valve because of the increased blood flow (denoted by
two arrows) passing through this normal-sized valve, producing a relative stenosis of the
pulmonary valve. Therefore, the murmur is systolic in timing and is maximal at the pulmonary
valve area (i.e., at the upper left sternal border). When the shunt is large, increased
blood flow through the tricuspid valve (denoted by two arrows) results in a relative stenosis

of this valve, producing a mid-diastolic murmur at the tricuspid valve area (i.e., lower left
sternal border). The widely split S2 that is a characteristic finding in ASD results partly
from RBBB. The RBBB delays both the electrical depolarization of the RV and the ventricular
contraction, resulting in delayed closure of the pulmonary valve. In addition, the large
atrial shunt tends to abolish respiration-related variations in systemic venous return to the
right side of the heart, resulting in a fixed S2.
It should be noted that infants and small children rarely manifest with clinical findings
described above even in the presence of a moderately large ASD (proved by echocardiographic
studies) until they are 3 to 4 years of age. It is because the compliance of the RV
improves slowly so that any significant shunt does not occur until that age.
Children with ASD rarely experience congestive heart failure (CHF) even in the presence
of a large left-to-right shunt. The PAs can handle an increased amount of blood flow
for a long time without developing pulmonary hypertension or CHF because there is no
direct transmission of the systemic pressure to the PA, and PA pressure remains normal.

Ventricular Septal Defect

The direction of the shunt in acyanotic VSD is left to right. The magnitude of the shunt is
determined by the size, not the location, of the defect and the level of pulmonary vascular
resistance (PVR). With a small defect, a large resistance to the left-to-right shunt occurs
at the defect, and the shunt does not depend on the level of PVR. A decrease in the PVR
occurs normally in this situation. With a large VSD, the resistance offered by the defect is
minimal, and the left-to-right shunt depends largely on the level of PVR. The lower the
PVR, the greater the magnitude of the left-to-right shunt. This type of left-to-right shunt is
called a dependent shunt (in contrast to obligatory shunt, to be discussed later in this chapter).
Even in the presence of a large VSD in a newborn, the PVR remains elevated, and
therefore a large shunt does not occur until the infant reaches 6 to 8 weeks of age, when
the shunt increases and CHF may develop.
In a VSD of moderate size, the cardiac chambers or vessels with two arrows enlarge,
resulting in enlargement of the main PA, LA, and LV, as well as an increase in pulmonary
vascular markings (Fig. 9-4). In VSD, it is the LV that does volume overwork, not the RV.
This results in LV enlargement; the RV does not enlarge. Because the shunt of VSD occurs
mainly during systole when the RV also contracts, the shunted blood goes directly to the
PA rather than remaining in the RV cavity. Therefore, there is no significant volume overload
to the RV, and the RV remains relatively normal in size in a VSD with moderate shunt
(Figs. 9-4 and 9-5). It should be noted that LA enlargement is present only with VSD but
not with ASD. It should also be noted that both VSD and PDA produce an enlargement of
the LA and LV.
Figure 9-6 summarizes the hemodynamics of VSDs of varying sizes and helps in the
understanding of clinical manifestations. The size of a cardiac chamber directly relates to
the amount of blood (or the number of arrows) handled by the chamber. The total number of arrows in the heart
diagram also determines the overall size of the heart. Let us examine
Figure 9-6 for varying sizes of VSDs.
With a small VSD, there is only half an arrow coming from the LV to the main PA. In
addition, the degree of pulmonary vascular congestion and chamber enlargement is either
minimal or too small to result in a significant change in the chest x-ray films (see Fig. 9-6).
The degree of volume work imposed on the LV is also too small to produce left ventricular
hypertrophy (LVH) on the ECG. The shunt itself produces a heart murmur (regurgitant
systolic), and the intensity of the P2 is normal because the PA pressure remains normal.
With a VSD of moderate size, one arrow shunts from the LV to the RV, and all the chambers
that are enlarged handle two arrows. Therefore, the degree of cardiomegaly on the
radiographic film is significant. The volume overwork done by the LV is significant, so the
ECG shows LVH of the “volume overload” type. Although the shunt is large, the RV is not
significantly dilated, and the pressure in this chamber is elevated only slightly (see Fig.
9-6). In other words, in a moderate VSD, the RV is not under significant volume or pressure
overload; therefore, ECG signs of RVH are absent. As in a small VSD, a heart murmur
(regurgitant systolic type) is produced by the left-to-right shunt. The normal-sized mitral
valve handles two arrows. This relative mitral stenosis produces a mid-diastolic rumble at
the apex. The PA pressure is mildly elevated; therefore, the intensity of the P2 may increase
slightly.
With a large VSD, the overall heart size is larger than that seen with a moderate VSD
because there is a much greater shunt. Because there is direct transmission of the LV pressure
through the large defect to the RV, in addition to a much greater shunt, the RV becomes enlarged
and hypertrophied. Therefore, the chest radiograph shows biventricular enlargement, left atrial
enlargement, and greatly increased pulmonary vascularity (see Fig. 9-6). The ECG shows
biventricular hypertrophy (BVH) and sometimes left atrial hypertrophy (LAH). A large VSD
usually results in CHF in early infancy.
When a large VSD is left untreated, irreversible changes take place in the pulmonary
arterioles, producing pulmonary vascular obstructive disease (or Eisenmenger’s syndrome).
It may take years to develop this condition. When Eisenmenger’s syndrome occurs, striking
changes take place in the heart size, ECG, and clinical findings. Because the PVR is
notably elevated at this stage, approaching the systemic level, the magnitude of the left-toright
shunt decreases. This results in removal of the volume overload placed on the LV as
well as the LA. Therefore, the size of the LV and the overall heart size decrease, and the ECG
evidence of LVH disappears, leaving only RVH because of the persistence of pulmonary
hypertension. Although the heart size becomes small, the PA segment remains enlarged
because of persistent pulmonary hypertension. In other words, with the development of pulmonary vascular
obstructive disease, the heart size returns to normal except for a
prominent PA segment, and a pure RVH on the ECG results. A bidirectional shunt causes
cyanosis. Because the shunt is small, the loudness of the murmur decreases, or it may even
disappear. The S2 is loud and single owing to pulmonary hypertension.

Patent Ductus Arteriosus

The hemodynamics of PDA are similar to those of VSD. The magnitude of the left-to-right
shunt is determined by the resistance offered by the ductus (i.e., diameter, length, and
tortuosity) when the ductus is small and by the level of PVR when the ductus is large
(i.e., dependent shunt). Therefore, the onset of CHF with PDA is similar to that with VSD.
The chambers and vessels that enlarge are the same as those in VSD except for an
enlarged aorta to the level of the PDA (i.e., enlarged ascending aorta and transverse arch),
which also handles an increased amount of blood flow (Fig. 9-7). Therefore, in PDA, chest
radiographic films show enlargement of the LA and LV, a large ascending aorta and PA, and
an increase in pulmonary vascular markings (Fig. 9-8). Although the aorta is enlarged, it
usually does not produce an abnormal cardiac silhouette because the ascending aorta does
not normally form the cardiac silhouette. Therefore, chest radiographic films of PDA are
indistinguishable from those of VSD.
Hemodynamic consequences of PDA are similar to those of VSD. In PDA with a small
shunt, the LV enlargement is minimal; therefore, the ECG and chest radiographic findings
are close to normal. Because there is a significant pressure gradient between the aorta and
the PA in both systole and diastole, the left-to-right shunt occurs in both phases of the
cardiac cycle, thereby producing the characteristic continuous murmur of this condition.
With a small shunt, the intensity of the P2 is normal because the PA pressure is normal.
In PDA with a moderately large shunt, the heart size is moderately enlarged with
increased pulmonary blood flow. The chambers enlarged are the LA, LV, and PA segments.
The ECG shows LVH as in moderate VSD. In addition to the characteristic continuous murmur, there may be an
apical diastolic flow rumble as a result of relative stenosis of the
mitral valve. The P2 slightly increases in intensity if it can be separated from the loud heart
murmur.
In a large PDA, marked cardiomegaly and increased pulmonary vascular markings are
present. The volume overload is on the LV and LA, which produces LVH and occasional
LAH on the ECG. The free transmission of the aortic pressure to the PA produces pulmonary
hypertension and RV hypertension, with resulting RVH on the ECG. Therefore, the
ECG shows BVH and LAH, as in a large VSD. The continuous murmur is present, with an
apical diastolic rumble owing to relative mitral stenosis. The P2 is accentuated in intensity
due to pulmonary hypertension.
An untreated large PDA can also produce pulmonary vascular obstructive disease, with
a resulting bidirectional (i.e., right-to-left and left-to-right) shunt at the ductus level. The
bidirectional shunt may produce cyanosis only in the lower half of the body (i.e., differential
cyanosis). As in VSD with Eisenmenger’s syndrome, the heart size returns to normal
because of the reduced magnitude of the shunt. The peripheral pulmonary vascularity
decreases, but the central hilar vessels and the main PA segment are greatly dilated owing
to severe pulmonary hypertension. The ECG shows pure RVH because the LV is no longer
under volume overload. Auscultation no longer reveals the continuous murmur or the apical
rumble as a result of the shunt reduction. The S2 is single and loud due to pulmonary
hypertension.

Endocardial Cushion Defect

During fetal life, the endocardial cushion tissue contributes to the closure of both the lower
part of the atrial septum (i.e., ostium primum) and the upper part of the ventricular septum
in addition to the formation of the mitral and tricuspid valves. The failure of normal development
of this tissue may be either complete or partial. A simple way of understanding
the complete form of endocardial cushion defect (ECD) is that the tissue in the center of
the heart is missing, with resulting VSD, the primum type of ASD, and clefts in the mitral
and tricuspid valves. In the partial form of the defect, only an ASD is present in the ostium
primum septum (primum type of ASD), often associated with a cleft in the mitral valve.
Hemodynamic abnormalities of primum-type ASD are similar to those of secundumtype
ASD, in which the RA and RV are dilated with increased pulmonary blood flow (Fig.
9-9). These changes are expressed in the chest radiographs (see Fig. 9-3). The cleft mitral
valve is usually insignificant from a hemodynamic point of view because blood regurgitated
into the LA is immediately shunted to the RA, thereby decompressing the LA. The physical
findings are also similar to those of secundum ASD: a widely split and fixed S2, a systolic
ejection murmur at the upper left sternal border, and a mid-diastolic rumble of relative
tricuspid stenosis at the lower left sternal border. In addition, a systolic murmur of mitral
regurgitation (MR) is occasionally present. The ECG findings are also similar: RBBB (with

rsR′ in V1) or mild RVH. One exception, which is important in differentiating between the

two types of ASDs, is the presence of a “superior” QRS axis or left anterior hemiblock (with
the QRS axis in the range of −20 to −150 degrees) in primum-type ASD. The abnormal
QRS axis seen in ECD (both partial and complete forms) is not the result of axis deviation
or any of the hemodynamic abnormalities mentioned; rather, the abnormal QRS axis
occurs as a result of the primary abnormality in the development of the bundle of His and
the bundle branches.
Hemodynamic changes seen with complete ECD are the sum of the changes seen in ASD
and VSD. There is volume overload of the LA and LV as in VSD and partially due to MR.
In addition, it has volume overload of the RA and RV as in ASD (see Fig. 9-9). The result is
biatrial and biventricular enlargement (Fig. 9-10). The magnitude of the left-to-right shunt
in complete ECD is determined by the level of PVR (i.e., dependent shunt). The ECG also
reflects these changes as BVH and occasional biatrial hypertrophy (BAH). “Superior” QRS
axis is also characteristic of ECD as discussed earlier. Physical examination is characterized
by a hyperactive precordium and regurgitant systolic murmurs of VSD and MR, loud
and narrowly split S2 (because of pulmonary hypertension), apical or tricuspid diastolic
rumble (or both), and signs of CHF. Those who survive infancy may develop pulmonary
vascular obstructive disease, as already discussed for large VSD and large PDA A direct communication between
the LV and RA may occur as part of ECD (or as an
isolated defect unrelated to ECD). The direction of the shunt is from the high-pressure LV
to the low-pressure RA. The magnitude of the shunt is determined by the size of the defect,
regardless of the state of PVR; blood shunted to the RA must go forward through the lungs
even if the PVR is high. This type of shunt, which is independent of the status of PVR, is
called an obligatory shunt (see Fig. 9-9). When an LV-RA shunt is present as part of complete
ECD, CHF may occur within a few weeks, which is earlier than in the usual VSD. The
enlarged chambers are identical to those of the complete form of ECD. Therefore, the chest
radiographs and ECG findings are similar to those seen in complete ECD. Physical findings
also resemble those of complete ECD, although the holosystolic murmur (resulting from
the LV-RA shunt) may be more prominent at the mid-right sternal border.