Atrial septal defect
5-10% of all CHD
More in females (3 times males)
Types
Secundum ASD: 50-70%
Primum ASD: 30%
Sinus venosus ASD: 10%. Associated with partial anomalous pulmonary venous
drainage.
Coronary sinus ASD
ASD secundum:
Ostium secundum defect is the most common type of ASD, accounting for 50% - 70% of all
ASDs, allowing left-to-right shunting of blood from the left atrium (LA) to the right atrium
(RA) and cause Rt. side volume overload. Mitral valve prolapse (MVP) occurs in 20% of
patients with either ostium secundum or sinus venosus defects. ASD secundum associated
with Holt -Oram -syndrome (limb abnormality, prolong PR interval and ASD secundum)
which autosomal dominant disease
Clinical manifestation:
History:
Infants and children with ASDs are usually asymptomatic.
Physical Examination:
1-A relatively slender body build is typical. (The body weight of many is less than the
10th percentile.)
2-A widely split and fixed S2 and a grade 2 to 3/6 systolic ejection murmur are
characteristic findings of ASD in older infants and children.
3-loud first hear sound tricuspid component
ECG:
Right axis deviation + right bundle branch block (RBBB) with an rsR'
pattern in V1 are typical finding
ly with enlargement of the RA and right ventricle
Cardiomeg
CXR:
(RV) may be present
Natural History:
1-Spontaneous closure occurs more than 80% of the time in patients with defects
between 3 and 8 mm before 1½ years of age. An ASD with a diameter greater than 8
mm rarely closes spontaneously.
2-Most children with an ASD remain active and asymptomatic. Rarely, congestive
heart failure (CHF) can develop in infancy.
3- If a large defect is untreated, CHF and pulmonary hypertension develop in adults
who are in their 20s and 30s.
4-With or without surgery, atrial arrhythmias (flutter or fibrillation) may occur in
adults
5-Infective endocarditis does not occur in patients with isolated ASDs.
6- Cerebrovascular accident, resulting from paradoxical embolization through an
ASD, is a rare complication
Management
Medical:
1-Exercise restriction is unnecessary.
2-Prophylaxis for infective endocarditis is not indicated unless the patient has
associated MVP or other associated defects. Prophylaxis is indicated in patients with
primum ASD.
3-In infants with CHF, medical management is recommended because of its high
success rate and the possibility of spontaneous closure of the defect.
Nonsurgical Closure.
1-using a catheter-delivered closure device has become a preferred method
2-Surgical Closure is usually delayed until 2 to 4 years of age because the possibility
of spontaneous closure exists and because children tolerate the defect well.
Partial Endocardial Cushion Defect
1-ASD primum with or without MR
2-Patients with ostium primum ASD are usually asymptomatic during childhood.
3-A history of symptoms such as dyspnea, easy fatigability, recurrent respiratory
infections, and growth retardation may be present early in life if associated with
major MR
Physical Examination
Cardiac findings are the same as those of secundum ASD , with the exception of a
regurgitate systolic murmur of MR (owing to a cleft mitral valve), which may be
present at the apex
=LAD+RBBB
ECG
findings are the same as those of a secundum ASD, except for enlargement
ray
-
The x
of the LA and LV when MR is significant
Complete Endocardial Cushion Defect
1-Failure to thrive, repeated respiratory infections, and signs of CHF are common.
2-Infants with ECD are usually undernourished and have tachycardia and tachypnea
(signs of CHF). This defect is common in infants with Down syndrome.
3- Patients with complete ECD, heart failure occurs 1 to 2 months after birth and
recurrent pneumonia is common.
3-Without surgical intervention, most patients die by the age of 2 to 3 years
PDA
PDA occurs in 5% to 10% of all congenital heart defects, excluding premature infants. It is
more common in females than in males (male/female ratio of 1:3). PDA is a common problem
in premature infants.Patients are usually asymptomatic when the ductus is small, large-shunt
PDA may cause a lower respiratory tract infection and CHF.Bounding peripheral pulses with
wide pulse pressure (with elevated systolic pressure and lower diastolic pressure) are
characteristic findings. With a small shunt, these findings do not occur.
A systolic thrill may be present at the upper left sternal border. The P2 is usually normal, but
its intensity may be accentuated if pulmonary hypertension is present. A grade 1 to 4/6
continuous (“machinery”) murmur is best audible at the left infra-clavicular area or upper left
sternal border. If pulmonary vascular obstructive disease develops, a right-to-left ductal shunt
results in cyanosis only in the lower half of the body (i.e., differential cyanosis).
:
Managment
Medical
Indomethacin is more effective in premature neonate.
Standard anticongestive measures with digoxin and diuretics are indicated when CHF
develops.
No exercise restriction is needed in the absence of pulmonary hypertension.
Prophylaxis for subacute bacterial endocarditis (SBE) is indicated when indications arise.
Catheter closure of the ductus
Surgical Closure
Pulmonary Stenosis
8% to 12% of all congenital heart defects.
Physical Examination:
1-Most patients are a cyanotic and well developed.
2-Newborns with critical PS are cyanotic and tachypnea.
3-systolic thrill may be present at the upper left sternal border
4-An ejection-type systolic murmur (grade 2 to 5/6) is best audible at the upper left
sternal border
5-The louder and longer the murmur, the more severe the stenosis.
Natural History
1-The severity of stenosis is usually not progressive in mild PS CHF may develop in
patients with severe stenosis.
2-Infective endocarditis occasionally occurs.
3-Sudden death is possible in patients with severe stenosis during heavy physical
activities.
4-Without appropriate management, most neonates with critical PS die
Management
1-Newborns with critical PS and cyanosis require emergency treatment to reduce
mortality. These babies may temporarily improve with prostaglandin E
1
(PGE
1
)
infusion, which reopens the ductus arteriosus, and other supportive measures. Balloon
valvuloplasty is the procedure of choice in critically ill neonates
2-Balloon valvuloplasty, which is performed at the time of cardiac catheterization for
moderate and severe PS
s
Aortic Stenosi
Represents up to 10% of all CHDs
Clinical manifestation
History
Neonates with critical or severe stenosis of the aortic valve may develop signs of
hypoperfusion or respiratory distress related to pulmonary edema within days to
weeks after birth.
Most children with mild to moderate AS are asymptomatic. Occasionally, exercise
intolerance may be present.
Exertional chest pain, easy fatigability, or syncope may occur in a child with a severe
degree of obstruction.
Physical Examination
1. Infants and children with AS are acyanotic and are normally developed. Except for
neonates with critical AS, blood pressure is normal in most patients, but a narrow
pulse pressure is present in severe AS. A systolic thrill may be palpable at the upper
right sternal border, in the suprasternal notch, or over the carotid arteries. An ejection
click may be heard
Management
Medical
For critically ill newborns with CHF, the patients are stabilized before surgery or
balloon valvuloplasty by the use of rapidly acting inotropic agents (usually dopamine)
and diuretics to treat CHF and intravenous infusion of PGE
1
to reopen the ductus.
Mechanical ventilation may be useful. Neonates and young infants with CHF from
critical AS require balloon valvuloplasty (or surgery) on an urgent basis.
Percutaneous balloon valvuloplasty is now regarded as the first step in the
management of symptomatic neonates in many centers. It is also the first
interventional method for children older than 1 year.
Coarctation of aorta
Occurs in 8% to 10% of all cases of congenital heart defect. It is more common in
males than in females (male/female ratio of 2:1). Among patients with Turner's
syndrome, 30% have COA,
as many as 85% of patients with COA have a bicuspid
aortic valve
Infants with COA are pale and experience varying degrees of respiratory distress.
Oliguria or anuria, general circulatory shock, and severe acidemia are common.
Peripheral pulses may be weak and thready as a result of CHF. A blood pressure
differential may become apparent only after improvement of cardiac function with
administration of rapidly acting inotropic agents;
a loud S3 gallop is usually present.
A nonspecific ejection systolic murmur is audible over the precordium. The heart
murmur may become louder after treatment
,
a normal or rightward QRS axis and
RVH or right bundle branch block (RBBB) are present in most infants with COA,
rather than LVH; LVH is seen in older children
Management
Medical
1-In symptomatic neonates, PGE
1
infusion should be started to reopen the ductus
arteriosus and establish flow to the descending aorta and the kidneys during the first
weeks of life.
2-Intensive anticongestive measures with short-acting inotropic agents (e.g.,
dopamine, dobutamine), diuretics, and oxygen should be started.
3-Balloon angioplasty can be a useful procedure for sick infants in whom standard
surgical management carries a high risk.
4-Surgery