******* 1 - وزارة الصحة السورية

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Transcript ******* 1 - وزارة الصحة السورية

‫الدكتور‬
‫بشار عبد الوهيب الحاج علي‬
‫استشاري بطب األطفال والرضع وحديثي الوالدة‬
‫رئيس دائرة المشافي والهيئات العامة ‪ -‬وزارة الصحة‬
‫ما هو املقصود آبفات القلب الوالدية أو اخللقةة‬
‫ما هو سبب حدوث آفة القلب الوالدية‬
‫‪ ‬السبب الحقيقي غير معروف‬
‫‪ ‬عوامل وراثية بدئية ( شذوذات صبغية‪ -‬عوامل جينية )‬
‫‪ ‬عوامل بيئية ( كيمائية – أدوية – فيروسية – اشعة – مرض عند األم )‬
‫‪ ‬عوامل متداخلة ( متعددة العوامل )‬
Classification
 A cyanotic
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Interrupted Aortic Arch
Aortic Stenosis
Ventricular Septal Defect
Atrial Septal Defect
 Cyanotic Defects
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Hypo plastic left Ventricle
Pulmonary Stenos is
Tetra logy of Fallout
D transposition of the great vessels
Tricuspid Atresia
A cyanotic Congenital heart
Disease
Ventricular Septal Defect
 Defect in the septum separating the left and right
ventricles
 Most common type of congenital heart disease
accounting for 21% of all cases
 Can occur singly or in multiples anywhere along
the ventricular septum
 Small defects often close spontaneously in the
first 2 years of life while large defects require
surgical repair within the 1st year
Ventricular Septal Defect
Symptoms of Ventricular Septal Defects
 Rapid breathing
 Irritability
 Excessive Sweating
 Poor weight gain
 Congestive Heart Failure, usually within 6 to 8 weeks
of life if defect is large
 Pulmonary Hypertension if defect is large
Treatments for Ventricular Septal Defects
 Lasix and Aldactone to decrease symptoms of CHF
 Digoxin to increase effectiveness of myocardial
function
 If surgery needed, patching or suturing the defect can
be done
 Mortality from surgery is low
Atrial Septal Defect
 Defect in the septum separating the left and right atria
 Accounts for 5-10% of congenital heart disease
 Twice as frequent in girls versus boys
 Three types of atrial septal defects
Atrial Septal Defect
 Ostium Primum: Defect located in the lower part of
septum near tricuspid valve which separates the right
atrium and right ventricle
 Ostium Secundum: Defect located near center of atria
septum (most common accounting for 50-70% of atrial
defect)
 Sinus Venosus: Located near the SVC or IVC’s
entrances to the heart
Atrial Septal Defect
Symptoms of Atrial Septal Defects
 Slender build
 Heart murmur resulting from increased blood flow
through pulmonary valve
 Usually no significant exercise restriction unless defect
is large.
 SOB or palpitations are possible.
Treatment of Atrial Septal Defects
 If defect is small (less than 2mm), will usually resolves
spontaneously
 If defect is large, surgical correction is needed
 Minimally invasive procedures available
 Transcatheter devices, such as a septal occluder may be
used.
Patent Ductus Arteriosus
 The ductus arteriosus
connects the pulmonary
artery to the descending aorta
during fetal life.
 PDA results when the ductus
fails to close after birth.
Picture: www.lpch.org
Patent Ductus Arteriosus
Pathophysiology:
 Blood flows from aorta to the
pulmonary artery, creating a
left to right shunt, resulting
in left atrium and ventricle
overload.
 Increased pulmonary blood
flow can result in pulmonary
hypertension and reversal of
the shunt, which is known as
Eisenmenger’s Syndrome.
This results in flow of
desaturated blood to the
lower extremities.
Picture: www.lpch.org
Patent Ductus Arteriosus
 Symptoms:
 Children with small patent ductus are usually
asymptomatic.
 Large left to right shunts develop symptoms of
congestive heart failure such as tachypnea, tachycardia,
poor feeding and slow growth
 Physical exam:
 Continuous murmur heard best at the left sternal
border.
Patent Ductus Arteriosus
 Lab Studies:
 CXR: enlarged cardiac silhouette secondary to left atrial
and ventricular enlargement with prominent pulmonary
vascular markings.
 EKG: left atrial enlargement, LVH
 ECHO: doppler flow through the ductus
 Treatment:
 Surgical division or ligation of the PDA
Interrupted Aortic Arch
 Relatively rare occurs in 2 cases per 100,000 live births
 Consists of 2 different defects: divided aortic arch and
ventricular septal defect
 Divided Aortic arch results in continued blood flow to
the upper extremities, but none to the lower
extremities
Interrupted Aortic Arch
 Lower extremities oxygenated due to the combination
of patent ductus arteriosus and ventricular septal
defect
 PDA connects lower portion of the aortic to the
pulmonary artery
 VSD allows travel of oxygenated blood to the right
ventricle which in turns travels to the pulmonary
artery.
Interrupted Aortic Arch
Symptoms of Interrupted Aortic Arch
 Often discovered 3-4 days after birth when the patent
ductus arteriosus closes
 Symptoms of shock develops very rapidly as no
oxygenated blood flows to the lower extremities
 Rapid breathing, clammy sweating, and poor feeding
often develops during the first week
Symptoms of Interrupted Aortic Arch
 Most babies born at term with normal length and
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weight
Heart murmur usually heard
Liver may be enlarged
Left arm/leg pulses may be diminished or absent
Echocardiogram for diagnosis
Treatment of Interrupted Aortic Arch
 Before surgery, try to keep the PDA open to provide
oxygenated blood to the lower extremities
 Surgery to suture together the two ends of the aorta,
patch the VSD, and legate the PDA
 85-90% patients survive the hospital stay
Thnk you
Cyanotic Heart Diseases
TETRALOGY OF FALLOT
 It is the most common cyanotic congenital heart
defect
 VSD, pulmonary stenosis, overriding aorta and
right ventricular hypertrophy.
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Clinical Manifestations
 The degree of cyanosis depends on the amount of
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pulmonary stenosis.
Infants initially may be acyanotic.
A pulmonary stenosis murmur is the usual initial
abnormal finding.
If the pulmonary stenosis is more severe, or as it becomes
more severe over time, the amount of right-to-left shunting
at the VSD increases, and the patient becomes more
cyanotic.
With increasing severity of pulmonary stenosis, the
murmur becomes shorter and softer.
In addition to varying degrees of cyanosis and a murmur, a
single S2 and right ventricular impulse at the left
sternal border are typical findings.
Imaging Studies
 The ECG usually has right axis deviation and right
ventricular hypertrophy.
 The classic chest x-ray finding is a boot-shaped heart
created by the small main pulmonary artery and upturned
apex secondary to right ventricular hypertrophy.
 Echocardiography shows the anatomic features, including the
levels of pulmonary stenosis, and provides quantification of
the degree of stenosis.
 Coronary anomalies, specifically a left coronary artery
crossing the anterior surface of the right ventricular outflow
tract, are present in 5% of patients with tetralogy of Fallot.
Treatment
 Treatment of hypoxic spells consists of oxygen
administration, placing the child in the knee-chest
position (to increase venous return), and giving
morphine sulfate (to relax the pulmonary infundibulum
and for sedation).
 Complete surgical repair with closure of the VSD and
removal or patching of the pulmonary stenosis can be
performed in infancy.
 Occasionally, palliative shunt surgery between the
subclavian artery and pulmonary artery is performed for
complex forms of tetralogy of Fallot with more complete
repair at a later time.
 Subacute bacterial endocarditis prophylaxis is
indicated
TRANSPOSITION OF THE GREAT
ARTERIES
 Although dextroposed transposition of the great
arteries represents only about 5% of congenital heart
defects
 it is the most common cyanotic lesion to present
in the newborn period
 Without mixing of the two circulations, death
occurs quickly.
 Mixing can occur at the atrial (patent foramen
ovale/ASD), ventricular (VSD), or great vessel (PDA)
level.
Clinical Manifestations
 A history of cyanosis is always present, although it may
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not be profound and depends on the amount of mixing.
Quiet tachypnea and a single S2 are typically present.
If the ventricular septum is intact, there may be no
murmur.
Children with transposition and a large VSD have
improved intra cardiac mixing.
They may present with signs of CHF.
The heart is hyperdynamic, with palpable left and right
ventricular impulses.
A loud VSD murmur is heard.
S2 is single.
Imaging Studies
 ECG findings typically include right axis deviation
and right ventricular hypertrophy.
 The chest x-ray reveals increased pulmonary
vascularity, and the cardiac shadow is classically an
egg on a string created by the narrow superior
mediastinum.
 Echocardiography shows the transposition of the
great arteries, the sites and amount of mixing, and any
associated lesions
Treatment
 Initial medical management includes prostaglandin
E1 to maintain ductal patency.
 If the infant remains significantly hypoxic on
prostaglandin therapy, a balloon atrial septostomy
is performed to improve mixing between the two
circulations.
 Complete surgical repair is most often an arterial
switch; the atrial switch is rarely done.
 The arterial switch usually is performed within the
first 2 weeks of life, when the left ventricle still can
maintain a systemic pressure.
TRICUSPID ATRESIA
 Tricuspid atresia accounts for approximately 2% of all
congenital heart defects.
 The absence of the tricuspid valve results in a
hypoplastic right ventricle.
 All systemic venous return must cross the atrial
septum into the left atrium.
 The presence of a PDA or VSD also is necessary for
pulmonary blood flow and survival.
Clinical Manifestations
 Infants with tricuspid atresia are usually severely
cyanotic and have a single S2.
 If a VSD is present, there may be a murmur.
 A diastolic murmur across the mitral valve may be
audible.
 Frequently there is no significant murmur.
Imaging Studies
 The ECG shows left ventricular hypertrophy and a
superior QRS axis (between 0 and -90 degrees).
 The chest x-ray reveals a normal or mildly enlarged
cardiac silhouette with decreased pulmonary blood
flow.
 Echocardiography shows the anatomy, associated
lesions, and source of pulmonary blood flow.
Treatment
 Management initially depends on the presence of a VSD
and the amount of blood flow across the VSD to the lungs.
 If there is no VSD, or it is small, prostaglandin E1 is used
to maintain pulmonary blood flow until surgery.
 Surgery is staged with an initial subclavian artery-to-
pulmonary shunt (Blalock-Taussig procedure) typically
followed by a two-stage procedure (bidirectional Glenn
and Fontan procedure), which directs systemic venous
return directly to the pulmonary arteries
TRUNCUS ARTERIOSUS
 Truncus arteriosus occurs in less than 1% of all
cases of congenital heart disease.
 It results from the failure of septation of the truncus,
which normally occurs during the first 3 to 4 weeks
of gestation.
 Anatomically a single arterial trunk arises from the
heart with a large VSD immediately below the truncal
valve.
 The pulmonary arteries arise from the single arterial
trunk either as a single vessel that divides or
individually from the arterial trunk to the lungs.
Clinical Manifestations
 Varying degrees of cyanosis depend on the amount of
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pulmonary blood flow.
If not diagnosed at birth, the infant may develop signs
of CHF as pulmonary vascular resistance decreases.
The signs include tachypnea and cough.
Peripheral pulses are usually bounding as a result of
the diastolic runoff into the pulmonary arteries.
A single S2 is due to the single valve.
There may be a systolic ejection click, and there is
often a systolic murmur at the left sternal border.
Imaging Studies
 ECG findings include combined ventricular
hypertrophy and cardiomegaly.
 Increased pulmonary blood flow is usually seen
on chest x-ray. Pulmonary arteries may appear
displaced.
 Echocardiography defines the anatomy, including
the VSD, truncal valve function, and origin of the
pulmonary arteries.
Treatment
 Medical management is usually needed and includes
anticongestive medications.
 Surgical repair includes VSD closure and placement
of a conduit between the right ventricle and
pulmonary arteries
TOTAL ANOMALOUS PULMONARY
VENOUS RETURN
 Total anomalous pulmonary venous return accounts
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for about 1% of congenital heart disease.
Disruption of the development of normal pulmonary
venous drainage during the third week of gestation
results in one of four abnormalities.
Anatomically, all of the pulmonary veins fail to
connect to the left atrium and return abnormally via
the right heart.
They may have supracardiac, cardiac, infracardiac,
or mixed drainage.
An atrial level communication is necessary for
systemic cardiac output and survival
Clinical Manifestations
 The most important determinant of presentation is the presence
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or absence of obstruction to the pulmonary venous drainage.
Infants without obstruction have minimal cyanosis and may be
asymptomatic. The pulmonary blood flow creates a continuous
murmur and reenters the right atrium and right ventricle.
There is a hyperactive right ventricular impulse with a widely
split S2 (owing to increased volume ejected from the right
ventricle) and a systolic ejection murmur at the left upper
sternal border.
Growth is relatively poor.
Infants with obstruction present with cyanosis, marked
tachypnea and dyspnea, and signs of right heart failure including
hepatomegaly.
The obstruction results in little, if any, increase in right
ventricular volume. There may be no murmur or changes in
S2
Imaging Studies
 For infants without obstruction, the ECG is consistent with
right ventricular volume overload.
 Cardiomegaly with increased pulmonary blood flow is
seen on chest x-ray.
 Infants with obstructed veins have right axis deviation
and right ventricular hypertrophy on ECG. On chest xray, the heart is normal or mildly enlarged with varying
degrees of pulmonary edema that can appear similar to
hyaline membrane disease or pneumonia.
 Echocardiography shows the volume-overloaded right
heart, right-to-left atrial level shunting, and common
pulmonary vein including site of drainage and degree of
obstruction
Treatment
 At surgery, the common pulmonary vein is opened
into the left atrium, and there is ligation of any vein or
channel that had been draining the common vein.
HYPOPLASTIC LEFT HEART
SYNDROME
 Hypoplastic left heart syndrome accounts for 1% of all
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congenital heart defects .
It is the most common cause of death from cardiac
defects in the first month of life.
Hypoplastic left heart syndrome occurs when there is
failure of development of the mitral or aortic valve or the
aortic arch.
A small left ventricle that is unable to support normal
systemic circulation is a central finding of hypoplastic left
heart syndrome, regardless of etiology.
Associated degrees of hypoplasia of the ascending aorta
and aortic arch are present.
Left-to-right shunting occurs at the atrial level
Clinical Manifestations
 After delivery, the infant is dependent on right-to-left
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shunting at the ductus arteriosus for systemic blood flow.
As the ductus arteriosus constricts, the infant becomes
critically ill with signs and symptoms of CHF from
excessive pulmonary blood flow and obstruction of
pulmonary venous return.
Pulses are diffusely weak or absent.
S2 is single and loud.
There is usually no heart murmur.
Cyanosis may be minimal, but low cardiac output gives
a grayish color to the cool, mottled skin.
Imaging Studies
 ECG findings include right ventricular hypertrophy
with decreased left ventricular forces.
 The chest x-ray reveals cardiomegaly (with right-
sided enlargement) and pulmonary venous congestion
or pulmonary edema.
 Echocardiography shows the small left heart, the
degree of stenosis of the aortic and mitral valves, the
hypoplastic ascending aorta, and the adequacy of leftto-right atrial flow.
Treatment
 Medical management includes prostaglandin E1 to open
the ductus arteriosus, correction of acidosis, and
ventilatory and blood pressure support as needed.
 Surgical repair is staged with the first surgery (Norwood
procedure) done in the newborn period.
 Subsequent procedures create a systemic source for the
pulmonary circulation (bidirectional Glenn and Fontan
procedures), leaving the right ventricle to supply systemic
circulation.
 There have been and continue to be many modifications to
all three stages of the surgical repair.
‫شكرا إلصغائكم‬
‫أي سؤال ؟‬