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(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.
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.