ACYANOTIC CONGENITAL

HEART DISEASE:
Obstructive Lesions
Pulmonary Valve Stenosis with
Intact Ventricular Septum
 Pulmonary Valve Stenosis with
Intact Ventricular Septum
• Of the various forms of right ventricular
outflow obstruction with an Intact ventricular
septum, the most common is isolated
valvular pulmonary stenosis, which
accounts for 7-10% of all congenital heart
defects.
• The valve cusps are deformed to various
degrees and, as a result, the valve opens
incompletely during systole.
 Pulmonary Valve Stenosis with
Intact Ventricular Septum

• Pulmonary stenosis as a result of valve

dysplasia is the most common cardiac
abnormality in Noonan syndrome, and is
associated, in approximately 50% of
cases, with a mutation in the gene
PTPN11, encoding the protein tyrosine
phosphatase SHP-2 on chromosome 12.
• The mechanism for pulmonic stenosis is
unknown, although maldevelopment of the
distal portion of the bulbus cordis and the
sequelae of fetal endocarditis have been
suggested as etiologies.
PATHOPHYSIOLOGY
 CLINICAL MANIFESTATIONS
• Patients with mild or moderate stenosis usually
do not have any symptoms.

• Growth and development are most often normal.

If the stenosis is severe, signs of right ventricular
failure such as hepatomegaly,peripheral edema,
and exercise intolerance may be present.

• In a neonate or young infant with critical

pulmonic stenosis, signs of right ventricular
failure may be more prominent, and cyanosis is
often present because of right-to-left shunting at
the foramen ovale.
 MILD PULMONARY STENOSIS
• Venous pressure and pulse are normal.
• The heart is not enlarged, the apical impulse is
normal, and the right ventricular impulse is not
palpable.
• The only abnormality demonstrable
radiographically is usually poststenotic dilation
of the pulmonary artery.
• 2D-echo shows righ ventricular hypertrophy and
a slightly thickened pulmonic valve, which
domes in systole
• Doppler studies demonstrate a right ventricle to
pulmonary artery gradient of ≤30 mm Hg.
 MODERATE PULMONARY
STENOSIS
• Venous pressure may be slightly elevated
• In older children, a prominent a wave may be
noted in the jugular pulse.
• The electrocardiogram reveals right ventricular
hypertrophy, sometimes with a prominent spiked
P wave.
• Radiographically, the heart can vary from normal
size to mildly enlarged with uptilting of the apex
• because of the prominence of the right ventricle;
• Pulmonary vascularity may be normal or slightly
decreased.
 SEVERE PULMONARY
STENOSIS
• mild to moderate cyanosis may be noted in patients with
an interatrial communication (ASD or patent foramen
ovale)
• In the absence of any intracardiac shunt, cyanosis is
absent.
• If hepatic enlargement and peripheral edema are
present, they are an indication of right ventricular failure.
• The electrocardiogram shows gross right ventricular
hypertrophy, frequently accompanied by a tall, spiked P
wave.
• Radiographic studies confirm the presence of cardiac
enlargement with prominence of the right ventricle and
right atrium.
 TREATMENT
• Patients with moderate or severe isolated pulmonary
stenosis require relief of the obstruction.
• Balloon valvuloplasty is the initial treatment of choice
for the majority of patients
• Patients with severely thickened pulmonic valves,
especially common in those with Noonan syndrome,
may require surgical intervention.
• In a neonate with critical pulmonic stenosis, urgent
treatment by either balloon valvuloplasty or surgical
valvotomy is warranted.
• Excellent results are obtained in most instances.
• Recurrence is unusual after successful treatment except
in those patients with extremely dysplastic valves.
TREATMENT
AORTIC STENOSIS
 AORTIC STENOSIS
• Congenital aortic stenosis accounts for
≈5% of cardiac malformations recognized
in childhood
• Bicuspid aortic valve, one of the most
common congenital heart lesions overall,
is identified in up to 1.5% of adults and
may be asymptomatic in childhood.
• Aortic stenosis is more frequent in males
(3 : 1)
 Valvular Aortic Stenosis
• the most common form, the leaflets are
thickened and the commissures are fused
to varying degrees.
• Left ventricular systolic pressure is
increased as a result of the obstruction to
outflow.
• The left ventricular wall hypertrophies in
compensation; as its compliance
decreases, end-diastolic pressure
increases as well.
Subvalcular (Subaortic) Stenosis

• This lesion is frequently associated with other

forms of congenital heart disease such as mitral
stenosis and coarctation of the aorta (Shone
syndrome) and may progress rapidly in severity.
• It is less commonly diagnosed during early
infancy and may develop despite previous
documentation of no left ventricular outflow tract
obstruction.
• May become apparent after successful surgery
for other congenital heart defects (coarctation
• of the aorta, PDA, VSD)
 Supravalcular Aortic Stenosis

• The least-common type, may be sporadic,

familial, or associated with Williams
syndrome, which includes mental
retardation (IQ range: 41-80), elfin facies
(full face, broad forehead, flattened bridge
of the nose, long upper lip, and rounded
cheeks), and idiopathic hypercalcemia of
infancy.
WILLIAM SYNDROME
 CLINICAL MANIFESTATION

• Symptoms in patients with aortic stenosis

depend on the severity of the obstruction.

• Severe aortic stenosis that occurs in early

infancy is termed critical aortic stenosis and is
associated with left ventricular failure and signs
of low cardiac output.

• Heart failure, cardiomegaly, and pulmonary

edema are severe, the pulses are weak in all
extremities, and the skin may be pale or grayish.
Urine output may be diminished.
 Laboratory Findings Diagnosis

• The diagnosis can usually be made on the

basis of the physical examination.

• The electrocardiogram may occasionally

be normal even with more severe
obstruction, but evidence of left ventricular
hypertrophy and strain (inverted T waves
in the left precordial leads) is generally
present if severe stenosis is long-standing.
 TREATMENT

• Balloon valvuloplasty is indicated for children

with moderate to severe valvular aortic stenosis
to prevent progressive left ventricular
dysfunction and the risk of syncope and sudden
death.

• Valvuloplasty should be advised when the peak-

to-peak systolic gradient between the left
ventricle and aorta exceeds 60-70 mm Hg at
rest, assuming normal cardiac output, or for
lesser gradients when symptoms or
electrocardiographic changes are present.
 TREATMENT

• For more rapidly progressive subaortic

obstructive lesions, a gradient of 40-50
mm Hg or the presence of aortic
insufficiency is considered an Indication
for surgery.

• Balloon valvuloplasty is the procedure of

choice even in the neonatal period.
THANK YOU!