PATENT DUCTUS ARTERIOSUS

The ductus arteriosus is a normal fetal connection between the left PA and the
descending aorta. During fetal life, blood flow is shunted away from the lungs
through the ductus arteriosus and directly into the systemic circulation. PDAs are
common in premature neonates who weigh less than 1,500 g. They account for 5% to
10% of CHDs, excluding premature neonates.

Pathophysiology and Etiology

• During fetal life, the ductus arteriosus allows blood to bypass the pulmonary
circulation (fetus receives oxygen from the placenta) and flow directly into the
systemic circulation.
• After birth, the ductus arteriosus is no longer needed. Functional closure
usually occurs within 48 hours after birth. Anatomic closure is completed by
age 2 to 3 weeks.
• When the ductus arteriosus fails to close, blood from the aorta (high pressure)
flows into the low-pressure PA, resulting in pulmonary overcirculation.
• Increased pulmonary blood flow leads to a volume-loaded LV.

Clinical Presentation
Small to Moderate-Sized PDA
Usually asymptomatic.

Large PDA
• CHF, tachypnea, frequent respiratory tract infections.
• Poor weight gain, failure to thrive.
• Feeding difficulties.
• Decreased exercise tolerance.

Diagnostic Evaluation
• Auscultation: continuous murmur heard best at left upper sternal border.
Hyperactive precordium with large PDAs.
• Wide pulse pressure; bounding pulses.
• Chest X-ray: varies; normal or cardiomegaly with increased pulmonary
vascular markings.
• ECG: varies; normal or LVH.
• Two-dimensional echocardiogram with Doppler study and color flow mapping
to visualize the PDA with left-to-right blood flow.
• Cardiac catheterization is not needed for the initial diagnosis.

Management
• In the symptomatic premature neonate: indomethacin given I.V.
• Medical management:
o Monitor growth and development.
o Reassess for spontaneous PDA closure.
o Increase caloric intake as needed for normal weight gain.
o Diuretics: furosemide (Lasix), spironolactone (Aldactone).
o Infective endocarditis prophylaxis for 6 months after surgery or coil
occlusion.
 • Cardiac catheterization:
o For small PDAs coil occlusion.
o For larger PDAs a closure device may be used.
• Surgical management through PDA ligation.

Complications
• CHF, pulmonary edema.
• Infective endocarditis.
• Pulmonary hypertension/pulmonary vascular occlusive disease.
• Recurrent pneumonia.

ATRIAL SEPTAL DEFECT

ASD is an abnormal communication between the left and right atrias. ASDs account
for 9% of CHDs. There are three types:
• Ostium secundum ASD: the most common type of ASD; abnormal opening in
the middle of the atrial septum.
• Ostium primum ASD: abnormal opening at the bottom of the atrial septum;
increased association with cleft mitral valve and atrioventricular defects.
• Sinus venosus ASD: abnormal opening at the top of the atrial septum;
increased association with partial anomalous pulmonary venous return.

Pathophysiology and Etiology

• Blood flows from the higher-pressure left atrium across the ASD into the
lower-pressure right atrium (left-to-right shunt).
• Increased blood return to the right heart leads to right ventricular volume
overload and right ventricular dilation.
• Increased pulmonary blood flow leads to elevated pulmonary artery pressures.

Clinical Manifestations
• Usually asymptomatic.
• Clinical symptoms vary depending on type of associated defects:
o CHF (usually not until the third or fourth decade of life).
o Frequent upper respiratory infections (URIs).
o Poor weight gain.
o Decreased exercise tolerance.

Diagnostic Evaluation
• Auscultation: soft systolic ejection murmur heard best at the left upper sternal
border; widely split, fixed second heart sound.
• Chest X-ray: varies; normal to right atrial and ventricular dilation, increased
pulmonary markings.
• ECG: varies; right axis deviation and mild RVH or right bundle-branch block.
• Two-dimensional echocardiogram with Doppler study and color flow mapping
to identify the site of the ASD and associated lesions and document left-to-
right flow across the atrial septum.
 • Cardiac catheterization usually not needed for initial diagnosis; performed if
defect can be closed using an atrial occlusion device (device can be used only
in ostium secundum defects).

Management
• Medical management:
o Monitor and reassess (spontaneous closure rate is small but may occur
up to age 2).
o Treatment with anticongestive therapy (digoxin and Lasix) may be
necessary if signs of CHF are present (usually not until third to fourth
decade of life if ASD unrepaired).
o Infective endocarditis prophylaxis for 6 months after surgery or atrial
occlusion devise is used.
• Cardiac catheterization for placement of an atrial occlusion device for ostium
secundam defects.
• Surgical intervention:
o Primary repair: suture closure of the ASD.
o Patch repair of the ASD.

Complications
• CHF (rare).
• Infective endocarditis.
• Embolic stroke.
• Pulmonary hypertension.
• Atrial arrhythmias.

VENTRICULAR SEPTAL DEFECT

A VSD is an abnormal communication between the right and left ventricles. It is the
most common type of congenital heart defect, accounting for approximately 25% of
all CHDs. VSDs vary in the size (small and restrictive to large and nonrestrictive
defect), number (single versus multiple), and type (perimembranous or muscular).

Pathophysiology and Etiology

• Blood flows from the high-pressure left ventricle across the VSD into the low-
pressure right ventricle and into the PA, resulting in pulmonary
overcirculation.
• A left-to-right shunt because of a VSD results in increased right ventricular
pressure and increased PA pressure.
• The increased pulmonary venous return to the left side of the heart results in
left atrial dilation.
• Long-standing pulmonary overcirculation causes a change in the pulmonary
arterial bed, leading to increased pulmonary vascular resistance. High
pulmonary vascular resistance (PVR) can reverse the blood flow pattern that
leads to a right-to-left shunt across the VSD (Eisenmenger's syndrome),
resulting in cyanosis. Once this develops, the child is no longer a candidate for
surgical repair.

Clinical Manifestations
 • Small VSDs—usually asymptomatic; high spontaneous closure rate during
the first year of life.
• Large VSDs.
o CHF: tachypnea, tachycardia, excessive sweating associated with
feeding, hepatomegaly.
o Frequent URIs.
o Poor weight gain, failure to thrive.
o Feeding difficulties.
o Decreased exercise tolerance.

Diagnostic Evaluation
• Auscultation: harsh systolic regurgitant murmur heard best at the lower left
sternal border (LLSB); systolic thrill felt at LLSB, narrowly split S2.
• Chest X-ray: varies; normal or cardiomegaly and increased pulmonary
vascular markings. Pulmonary vascular markings are directly proportionate to
the amount of left-to-right shunting.
• ECG: varies; normal to biventricular hypertrophy.
• Two-dimensional echocardiogram with Doppler study and color flow mapping
to identify the size, number, and sites of the defects, estimate pulmonary artery
pressure, and identify associated lesions.
• Cardiac catheterization usually not needed for initial diagnosis; may be needed
to calculate the size of the shunt or to assess PVR. May be performed if defect
can be closed using a ventricular occlusion device (device can be used only in
muscular defects).

Management
Small VSD
• Medical management:
o Usually no anticongestive therapy is needed.
o Infective endocarditis prophylaxis for 6 months after surgical
implantation of a ventricular occlusion device.
• Cardiac catheterization for placement of a ventricular occlusion device for
muscular defects (for Qp:Qs > 2:1).
• Surgical intervention is usually not necessary.

Moderate to Large VSD

• Medical Management:
o CHF management: digoxin and diuretics (furosemide, spironolactone)
and afterload reduction.
o Avoid oxygen; oxygen is a potent pulmonary vasodilator and will
increase blood flow into the PA.
o Increase caloric intake: fortify formula or breast milk to make 24 to 30
cal/oz formula; supplemental nasogastric feeds as needed.
o Infective endocarditis prophylaxis for 6 months after
surgery/ventricular device occluder.
• Cardiac catheterization for placement of a ventricular occlusion device for
muscular defects (for Qp:Qs > 2:1).
• Refer for surgical intervention.
o Usually repaired before age 1.
 o One-stage approach: preferred surgical plan; patch closure of VSD.
o Two-stage approach: first surgery is to band the PA to restrict
pulmonary blood flow; second surgery is to patch close the VSD and
remove the PA band.

Long-Term Follow-Up
• Monitor ventricular function.
• Monitor for subaortic membrane and double-chamber RV.

Complications
• CHF.
• Frequent URIs.
• Failure to thrive; poor weight gain.
• Infective endocarditis.
• Eisenmenger's syndrome.
• Pulmonary hypertension.
• Aortic insufficiency.