Left to Right Shunts

The flow through the systemic and pulmonary circulations is normally balanced and equal. As the two
circulations are placed in series with each other; blood first makes its way through the systemic circulation,
then the pulmonary circulation, then back to the systemic circulation, and so on.
Left to right shunts are characterized by a "back-leak" of blood from the systemic to the pulmonary
circulation. As a consequence, the pulmonary circulation carries not only the blood that legitimately entered
the right atrium and ventricle through the superior and inferior vena cava, but also the additional blood
entering through an ASD, VSD, or a PDA. Blood volume and pressure in the pulmonary circulation become
abnormally high. If the shunt is significant, there is progressive damage to the pulmonary vasculature and
gradual development of irreversible pulmonary hypertension. The pressure in the pulmonary circuit may
ultimately exceed the systemic pressure with reversal of blood flow from the right side of the circulation to
the left (Eisenmenger syndrome).

Lesions resulting in left to right shunts include:

Ventricular septal defect (VSD)

Patent ductus arteriosus (PDA)

Atrial septal defect (ASD)

Atrioventricular defect (AVSD)

In VSD and PDA, the direction and magnitude of the shunt depends on the size of the communication and
the relative resistance in the pulmonary and systemic circuit.
In ASD and AVSD, the magnitude of the shunt depends largely on relative ventricular compliance
(elasticity).

A.

Ventricular Septal Defects

Ventricular septal defects (VSD) constitutes 25-30% of all congenital heart disease (CHD) in children. VSD
may be present as an isolated lesion or in combination with other defects, as in Tetrology of Fallot.
Anatomy
Approximately 70% of all VSD's are present in the membranous portion of the inter-ventricular septum;
about 20% are in the muscular portion, and the remaining defects are at either the inlet or the outlet
portion of the ventricular septum.
Pathophysiology

With a small or medium sized VSD, "restrictive VSD," the direction and magnitude of the shunt depends
on the size of the VSD and the pressure gradient between the left and right ventricle. Blood flow is usually
from left to right.
With a large VSD, the hole is not restrictive and the pressure in both ventricles is almost equal. The
direction and magnitude of the shunt depend on the difference of the relative pulmonary and systemic
vascular resistances. In the fetal life, the pulmonary resistance is higher than the systemic resistance. As
the lungs expand with the first breath, the pulmonary resistance drops and the pulmonary flow increases.
The pulmonary resistance continues to decrease until reaching the adult values by 6-8 weeks.
If the left to right shunt is large, pulmonary hypertension gradually develops, and the flow across the shunt
decreases. If the pulmonary vascular resistance exceeds the systemic vascular resistance, the direction of
the shunt reverses and cyanosis develops after a few years (Eisenmenger syndrome).
Clinical Presentation
Children with a smaller VSD are usually asymptomatic. An incidentally discovered holosystolic heart
murmur is the common presentation. The intensity of the murmur is inversely proportional to the size of
the VSD because of the increased flow velocity produced by a smaller defect. A thrill may be palpable.
An infant with a large VSD may be asymptomatic in the first few weeks of life until the pulmonary vascular
resistance drops. With the declining pulmonary resistance, the left-to-right shunt increases. The right
ventricle is thus subjected to high pressure and becomes hypertrophied while the left ventricle receives
more volume and becomes dilated. Congestive heart failure (CHF) may develop and presents as
tachycardia, tachypnea, exertional dyspnea, breathlessness and sweating while feeding. The child's growth
is also often delayed. In some infants, especially those with Down Syndrome, the pulmonary vascular
resistance may not drop. These infants may not develop CHF but may need earlier surgical intervention to
prevent worsening of pulmonary hypertension and early development of Eisenmenger syndrome.
Making the diagnosis

A holosystolic murmur is heard at the left sternal border due to turbulence across the VSD. The
intensity of the murmur is inverselyproportional to the magnitude of the shunt; the smaller the
shunt, the louder the murmur.

An apical mid-diastolic murmur may be heard due to increased flow across the mitral valve (relative
mitral stenosis).

A loud P2 indicates increased pulmonary vascular pressure.

Cardiomegaly and hepatomegaly may be present if congestive heart failure has developed.

Chest radiograph may show cardiomegaly and increased pulmonary vascularity.

EKG reflects the hemodynamic status and may show RVH, left atrial enlargement and LVH.

Echocardiogram shows the location, type and size of the VSD. It can also measure the pressure
gradient across the VSD.

Cardiac catheterization may be needed to measure the pulmonary pressure and to determine the
reactivity of the pulmonary vasculature.

Natural history
More than half of small and medium sized VSDs will close spontaneously, but less than 10% of large VSDs
will close spontaneously.

Management
Asymptomatic children with a small or medium sized VSD need only supportive care, with the expectation
that the VSD will close in the first few years of life. If congestive heart failure develops, treatment consists
of diuretics, after-load reducing agents and possibly digoxin. Fluid restriction should be avoided as it
reduces the caloric intake and delays growth.
Oxygen therapy should also be avoided as it lowers the pulmonary vascular resistance, increases the left to
right shunt and worsens the symptoms of congestive heart failure. Patients with persistent CHF or those
who are developing pulmonary hypertension require surgical closure of the VSD. If a patient is not a
suitable candidate for surgery, pulmonary artery banding should be considered.

B. Patent Ductus Arteriosus

Anatomy
The ductus arteriosus, formed from the embryonic 6th aortic arch, connects the aorta to the pulmonary
artery. It normally closes within a few days after birth. A high oxygen tension and a decrease in
endogenous prostaglandins are important factors in inducing closure of the ductus arteriosus. For the same
reason, prostaglandin synthetase inhibitors such as indomethacin are effective in inducing ductal closure in
the newborn period, especially in premature infants.

Pathophysiology
Failure of closure of the ductus arteriosus leads to pathophysiologic changes similar to those seen with
VSD. The direction and extent of the shunt in the PDA depends on the size of the PDA and the relative
systemic and pulmonary arterial resistances.

Clinical presentation
PDA is more common in females, premature infants, and patients with Down Syndrome or congenital
rubella syndrome. The symptoms are similar to those found in VSD and depend on the size of the shunt
and direction of flow. While a small PDA is asymptomatic, a large PDA with significant shunting may lead to
CHF.

Making the diagnosis

In older children, PDA classically produces a continuous or "machinery" murmur due to flow across
the shunt throughout the cardiac cycle. In newborn infants, especially those born prematurely, the
murmur may be heard only during systole because the higher pulmonary arterial pressure prevents
flow during diastole.

An apical mid-diastolic murmur may be heard because of increased flow through the mitral valve.
This usually signifies a large PDA and is often associated with CHF. Remember that the flow through
the post-natal PDA is left to right.

Wide pulse pressure indicates a large left to right shunt

In a small PDA, the EKG is normal. In large PDA, the EKG will demonstrate left ventricular and left
atrial enlargement due volume overload. There may also be biventricular enlargement or
hypertrophy.

Chest radiograph may show increased pulmonary vascularity and cardiomegaly due to left atrial and
left ventricular enlargement. The heart size might be normal if the shunt is small.

Natural history and management

Small and moderate sized PDA often close spontaneously especially in full term infants. PDA in premature
infants may need indomethacin or surgical closure. In older children, a PDA is closed by placing a coil or a
device in the ductus via a catheter inserted through the femoral vein. Surgical ligation may be necessary.

C. Atrial Septal Defects (ASD)

Anatomy
Atrial septal defects involve many different parts of the inter-atrial septum. The septum secondum
defect is the most common and comprises 6-10% of all CHD. It is located in the fossa ovalis, in the
location of the foramen ovale.

Pathophysiology
Since the pressure difference between the left and right atria is small, the direction and magnitude of the
shunt across the ASD depends on the relative right and left ventricular compliances. In early infancy, the
right ventricular compliance is low and the shunt across the ASD is small. As the right ventricular
compliance increases, the left to right shunt increases.

Clinical presentation
ASD is usually asymptomatic and typically presents with a heart murmur in preschool age. CHF rarely
develops in patients with large ASD. Right atrial stretch may cause atrial arrhythmias. Prolonged volume

overload of the lungs eventually causes pulmonary hypertension, which usually takes 4 to 5 decades to
develop.

Making the diagnosis

A systolic ejection murmur may be heard in the pulmonary area due to increased flow across the
pulmonary valve (relative pulmonary stenosis).

A mid-diastolic murmur may be heard at the lower left sternal border due to increased flow across
the tricuspid valve.

There is wide splitting of S2 due to increased flow across the pulmonary valve.

The splitting of S2 is "fixed" and does not vary with respiration. This is due to diminished effects of
respiratory cycle on the volume of the blood in the right ventricle. In inspiration, the venous return
to the right atrium increases and the left to right shunt across the ASD decreases. In expiration, the
venous return to the right atrium decreases and the shunt across the ASD increases. In both cases,
the volume of blood in the right ventricle is increased and fixed in both inspiration and expiration.

EKG may demonstrate right ventricular conduction delay (incomplete right bundle branch block),
right atrial enlargement, right axis deviation, and right ventricular dilation/hypertrophy

Chest radiograph shows increased pulmonary vascularity and right atrial and right ventricular
enlargement.

Management
Most patients with ASD are asymptomatic and no specific medical management is necessary. Management
(as in VSD) may be needed if CHF is present. Surgical closure is performed in patients with a large ASD. A
catheter-delivered occluding device can be placed in most patients older than 3 years of age with
secondum ASD.

D. Atrioventricular Defect (AVSD)

AVSD occurs in 2% of all CHD and is more prevalent in patients with Down Syndrome. Forty percent of
children with Down Syndrome have CHD, and 40 % of the defects are forms of AVSD.

Anatomy

Atrioventricular septal defect is also known as endocardial cushion defect (ECD) and atrioventricular
canal (AVC). It consists of a variety of defects in the endocardial cushions which form the lower part of
the atrial septum, upper part of the ventricular septum, and the medial parts of the tricuspid and mitral
valves.
The defect may be incomplete or complete.

Incomplete AVSD (primum ASD) consists of a defect in the lower part of the atrial septum and is
usually associated with a cleft in the anterior mitral leaflet causing mitral insufficiency.

Complete AVSD has a defect that extends from the lower part of the atrial septum to the upper
part (inlet) of the ventricular septum. The mitral and tricuspid valves lose their anchor points in the
ventricular septum and are instead attached to each other, forming a common AV valve that
overhangs the ventricular septum.

Pathophysiology
There is a left to right shunt at the atrial level due to increased relative right ventricular compliance leading
to right atrial enlargement. Left atrial enlargement occurs because of mitral insufficiency secondary to the
mitral valve cleft. There is varying degree of pulmonary hypertension as the pulmonary vasculature is
exposed to excess blood volume at higher pressure. The pulmonary hypertension may develop at about
one to two years in normal patients and at about six months to one year in patients with Down Syndrome.

Clinical presentation
The clinical presentation of AVSD is variable and depends on the size of the defect and the degree of the
left to right shunt. Patients with complete AVSD usually present with congestive heart failure in the first few
weeks of life, while those with incomplete AVSD (primum ASD) may be completely asymptomatic.
Symptoms of CHF include poor feeding, shortness of breath, diaphoresis during feeding, and poor weight
gain. Mild cyanosis may be present because of right to left shunt due to excessive pulmonary over
circulation or due to preferential streaming of the venous blood from the IVC to the left atrium.

Making the diagnosis

Physical findings are variable and depend on the presence or the absence of congestive heart
failure. The precordium may be hyperdynamic and the cardiac apex might be displaced inferiorly
and to the left.

The auscultatory findings are also variable and may include a systolic ejection murmur due to
increased flow through the pulmonary valve with wide and fixed splitting of S2 as in ASD. A loud,
single S2 indicates pulmonary hypertension.

Additional auscultatory findings include a mid-diastolic murmur at the lower left sternal border due
to increased flow through the tricuspid valve, apical holosystolic murmur radiating to the left axilla
due to mitral insufficieny, apical mid-diastolic murmur of severe mitral insufficiency and a
holosystolic murmur of VSD.

EKG is characterized by left axis deviation, right atrial enlargement, bi-ventricular hypertrophy and
an incomplete right bundle branch block (rSR in V1 indicating right ventricular volume overload). A
prolonged PR interval (first degree heart block) probably due to abnormal AV node may be present.

Chest radiograph shows varying degrees of cardiomegaly and increased pulmonary vascularity.

Echocardiography is useful in demonstrating the anatomical lesions and associated abnormalities. It

is essential to assess the integrity of the AV valves.

Management
Congestive cardiac failure is managed with diuretics, ACE inhibitors, and digoxin. A high caloric-density
formula should be used with no fluid restriction.
Almost all patients with AVSD will need surgery. The timing of surgery depends on the size of the patient,
the presence of CHF, response to medical management, and the presence or absence of pulmonary
hypertension. In asymptomatic patients with partial AVSD, surgery may be delayed to preschool age.
Corrective surgery is usually performed in symptomatic patients with intractable CHF in the first three
months of life. Even in patients who respond to medical management, the presence of Down Syndrome
necessitates early surgery to prevent development of pulmonary hypertension. If corrective surgery can
not be performed in a symptomatic patient, pulmonary arterial banding may help limit the pulmonary blood
flow until corrective surgery is possible.

Obstructive Cardiac Lesions

This section deals primarily with ventricular outflow tract lesions. Congenital mitral and tricuspid valve
stenoses are relatively rare in children, thus are not discussed here.
Outflow tract obstruction leads to an increase in the pressure proximal to the lesion and secondary
myocardial hypertrophy. Turbulent flow across the obstruction produces an ejection systolic murmur.
Myocardial hypertrophy leads to increased oxygen consumption with decreased exercise tolerance and
possible myocardial ischemia and fibrosis.
Ventricular dilatation is not a usual response to obstruction, and if present suggests ventricular failure.
Children with mild or moderate obstructive lesions usually have few symptoms. Severe obstructive may
lead to early CHF symptoms and are often ductus dependent (the patient often requires a patent ductus
arteriosus to bypass the obstruction).

A. Pulmonary Stenosis (PS)

Anatomy
Pulmonary stenosis can be valvular, subvalvular (infundibular), or supravalvular.

Valvular PS. The valve leaflets are thickened and/or narrowed.

Infundibular PS is usually associated with VSD as in Tetralogy of Fallot. The muscle under the valve
area is thickened, narrowing the outflow tract from the right ventricle.

Supravalvular PS is rare and may be associated with congenital rubella or Williams Syndrome. The
portion of the pulmonary artery just above the pulmonary valve is narrowed.

Peripheral pulmonary artery stenosis may result from congenital rubella or Alagille syndrome. The
right or left pulmonary artery is narrowed, or both may be narrowed.

Clinical presentation
Patients with mild or moderate PS are usually asymptomatic. Severe PS may present with decreased
cardiac output symptoms such as exertional dyspnea and subsequently symptoms of congestive cardiac
failure.

Making the diagnosis

The physical findings depend on the severity of the stenosis.

A right ventricular tap and a systolic thrill may be present at the ULSB.

An ejection systolic murmur is heard best at the ULSB radiating to the back. Wide splitting of S2 is
usually present. An ejection systolic click indicates post-stenotic dilation of the pulmonary artery.

Hepatomegaly may be present if CHF develops.

The EKG is normal in mild PS. RVH and right axis deviation are present in moderate or severe cases.
Right atrial enlargement indicates elevated right ventricular filling pressure. Severe PS may cause
RV strain pattern (deep inverted T wave and ST segment depression) in right precordial leads.
Newborns with critical PS may show LVH because of hypoplastic RV and large LV.

Chest radiograph usually shows normal heart size with a prominent pulmonary artery segment
because of post-stenotic dilatation. Cardiomegaly indicates CHF.

Echocardiography demonstrates the valvular lesion, the gradient across the stenotic valve, and the
presence of PDA.

Management
Restriction of activity is not necessary in the older child. Balloon valvoplasty may be done to relieve the

valvular obstruction in moderate or severe cases. Surgery is indicated in supravalvular and subvalvular PS
or if valvoplasty is unsuccessful.
Infants with critical PS need PGE1 to maintain the ductal patency.

B. Aortic Stenosis (AS)

Anatomy
Aortic stenosis may be valvular, supravalvular or subvalvular.

Supravalvular AS may be associated with Williams Syndrome.

A discrete fibrous ring, or membrane, may cause subvalvular obstruction.

Bicuspid aortic valve is a common cause of AS in patients with Turner Syndrome.

Clinical presentation
Aortic stenosis is more common in males. Children with mild or moderate AS are usually asymptomatic.
Severe AS may be accompanied by exertional chest pain, decreased exercise tolerance or episodes of
syncope. Infants with critical AS may present with CHF early in life.

Making the diagnosis

Patients with AS are usually acyanotic unless critical AS is present.

The pulse pressure may be narrow in severe AS. Patients with supravalvular AS may have higher BP
in the right arm than the left (Coanda effect).

A systolic thrill may be palpable at the URSB or the supra-sternal notch and radiates to the carotid
arteries.

An ejection systolic murmur is heard at the ULSB and URSB and radiates to the carotid arteries.

An early diastolic murmur may indicate the development of aortic insufficiency.

A newborn with critical AS who presents with CHF and poor peripheral pulses may have a faint
murmur or no murmur at all.

"Elfin facies" associated with mental retardation and personality changes suggest Williams
Syndrome and supravalvular

EKG is usually normal in mild AS. LVH indicates moderate or severe AS and may be associated with
a strain pattern (inverted T waves in left precordial leads).

Chest radiograph usually shows normal heart size. A prominent aortic knob indicates post-stenotic
dilation in valvular AS. Cardiomegaly is usually present in severe AS associated with CHF.

Echocardiography is useful in determining the type and the severity of the lesion.

Cardiac catheterization helps measure the gradient across the aortic valve.

Management
Mild or moderate AS requires only supportive care. Severe valvular AS can be treated by balloon
valvoplasty. Subvalvular and supravalvular aortic stenosis are not amenable to balloon valvoplasy and will
need surgical intervention.
PGE1 infusion is essential to maintain the ductal patency in neonates with critical AS.
Surgery may involve simple valvotomy or valve replacement with a prosthesis or homograft. Another
alternative is the Ross autograft procedure in which the patient's normal pulmonary valve is excised and
placed in the aortic position and a homograft replaces the pulmonary valve.

Cyanotic Cardiac Lesions

Cardiac cyanosis generally results from either right to left shunt or complete mixing of both systemic and
pulmonary circulations with obstruction of the pulmonary flow.

A. Tetralogy of Fallot (TOF)

Tetology of Fallot (TOF) is the most common cyanotic CHD (over 50% of all cases of cyanotic CHD; ~ 10%
of all cases of CHD).

Anatomy
TOF has four abnormalities (hence the term 'tetrology'):

ventricular septal defect (VSD)

over-riding aorta

right ventricular outflow tract obstruction (RVOTO)

right ventricular hypertrophy (RVH).

Pathophysiology
The VSD is nonrestrictive (both ventricles have equal pressures). The direction of the blood flow across the
VSD depends on the relative systemic and RVOT resistances.

Clinical manifestations
The patient presents with a history of a heart murmur at birth with variable degree of cyanosis (depending

on the degree of RVOTO). Some patients may be acyanotic. There is a right ventricular tap at the left
sternal border, systolic ejection murmur due to RVOTO, and single S2. The VSD is usually unrestrictive and
does not produce a heart murmur. Clubbing of fingers may be seen in untreated TOF.

Making the Diagnosis

EKG: RAD, RVH

CXR: 'Boot shaped' heart, due to concave main pulmonary artery segment and upturned RV apex
due to RVH.

Echocardiography: diagnostic. 25% of patients have right aortic arch and 5% have coronary artery
anomalies.

Tet spells (hypercyanotic, or hypoxic spells) (hence the term 'blue baby')

Tet spells are characterized by paroxysmal cyanosis, tachycardia, hyperpnea and irritability. These spells
are caused by reversal of the shunt across the VSD so the blood flows from the right to the left ventricle.
Reversal of the shunt is due to an increase in the RVOTO resistance. Initially, the systolic murmur gets
louder and then diminishes as the RVOTO progresses to near complete obstruction. Tet spells are more
common in the warm morning hours when the systemic vascular resistance is low due to increased
parasympathetic tone and decreased intravascular volume; a warm bath can have a similar effect. The
RVOTO is worsened by increased catecholamines as may occur with irritability, hunger, or wet diapers.
Hypercyanotic spells need urgent treatment because if left untreated, metabolic acidosis, seizures or
cerebrovascular accidents or even death may occur. The concept of treatment is to reverse the mechanism
of the hypoxic spells. It is important to remove any source of irritation of the infant to decrease the
catecholamine surge.
While the mother is comforting the baby (nursing, bottle feeding), the baby is placed in a knee-to-chest
position to increase systemic vascular resistance. Blow-by O2 is provided. If the spell continues, morphine
sulfate (0.2 mg/kg) may be given intramuscularly or subcutaneously. An IV line should be inserted and
isotonic fluid boluses should be administered and may be repeated until the heart rate becomes normal.
Acidosis should be treated with sodium bicarbonate. If the spell continues, vasoconstrictors such as
phenylephedrine may be tried. Intravenous beta blockers (propranolol or esmolol) may be used to relieve
the RVOTO. If the spell continues despite these measures, endotrachial intubation and general anesthesia
are indicated in preparation for urgent surgical intervention.

Management
If the patient is pink and has never had a cyanotic spell, elective surgery is usually performed in the first
few months by closing the VSD and relieving the RVOTO. If the patient is cyanotic or develops a cyanotic
spell, intervention is necessary in the neonatal period. If neonatal repair cannot be done because of
prematurity or an unstable medical condition, palliative Blalock-Taussig shunt (anastamosis between the

subclavian artery and pulmonary artery) or its modification, a shunt between the innominate and
pulmonary arteries, is performed to bypass the RVOTO.