•Atrial Septal Defects

ASD
Presented by Dr. Maysa Abdul Haq
Directed by Dr. Ali Halabi
Jordan Hospital
11-9-2005
 Background:

An atrial septal defect (ASD) is a deficiency of the atrial

septum. ASDs account for about 10-15% of all congenital
cardiac anomalies .

Types of ASDs:
1-Ostium secundum defect→70% of ASDs.
2-Ostum primum defect→20% of ASDs.
3-sinus venosus defect.→10%of ASDs.
4-coronary sinus septal defect→ < 1% of ASDs .
Normal heart for comparison.
Cardiac Development:
Cardiac tissues are first detectable on the 18th or
19th day of fetal life. Cardiac development continues
for the next several weeks. The atrial septum begins
to form during the fourth week and is complete by the
end of the fifth week.
Frequency:

Research indicates that the incidence of

congenital heart disease (CHD) is 0.8% of the US
population. Approximately 7% of these individuals,
or about 1 in 1500 live births, have an ASD. An
estimated 15-30% of healthy adults have an
unfused foramen ovale in which the valve
functions normally but has failed to fuse. I
PFO is of no haemodynamic significant and not
considered as ASD. Does not need surgical repair
but increase risk of paradoxical syst. embolization.
Age of presentation:
ASD, is present at birth; however, in most cases, a
murmur is not audible until the child is a few months
old. Symptoms usually do not occur in individuals
with ASD until late childhood, adolescence, or
adulthood.
Secundum type, sinus venosus, and unroofed
coronary sinus defects sometimes are not diagnosed
until the third decade of life.
Ostium primum ASDs usually are diagnosed in the
first few years of life because of the presence of a
mitral regurgitation murmur.
Ostium Secundum:
A defect in the region of the fossa ovalis
The most common form of ASD
Associated with structurally normal AV valves.
It may be single or multiple ( fenestrated atrial
septum)
Female: male 3:1 incidence.
Associated lesions :partially anomalous pulmonary
venous return .& Holt- Oram-syndrome.
Ostium secundum ASD is the most
common type of ASD. It occurs in the
center of the septum between the right
and left atrium. A variant of this type of
ASD is called a patent foramen ovale
(PFO) and is very small
Holt-Oram syndrome:
Holt-Oram syndrome (HOS) is a heart–upper limb
malformation complex with an autosomal dominant
inheritance.
Musculoskeletal defects:
♦Upper limbs are affected. The most severe form is
phocomelia with rudimentary limbs.
♦Hypoplasia of the radius , The most common
defects are radial thumb anomalies ranging from
absent thumbs to displaced (distally placed),
duplicated, or triphalangeal thumbs.
Continue…

Heart defects :The most common lesion is a

secundum ASD. Others include ventriculoseptal
defect (VSD), atrioventricular (AV) block, pulmonic
stenosis (including peripheral arterial), and mitral
valve prolapse.
Continue…

right hand of a 6-month-

 
old infant with Holt-Oram
syndrome, showing
hypoplastic right thumb.
                 
Pathophysiology:
The degree of left to right shunt depends on :
1-the size of the defect
2-compliance of right and left ventricle
3- vascular resistance in pulmonary and
systemic circulation.
With large defect , the ratio of pulmonary to systemic
blood flow Qp:Qs between 2:1 and 4:1
ASD in infants is asymptomatic :Right atrial mascular
wall is thick and less compliant thus limiting the left to
right shunt as infant becomes older , PVR drops ,
right ventricular wall becomes thinner and left to right
shunt increases .
Cont…
Large blood flow through the right side of the heart
cause enlargement of right atrium and ventricle ,
dilatation of the pulmonary artery but pulmonary atrial
BP is usually normal ( absence of high pressure
communication between Pulm. and Sys. Circulation.)

PVR may increase in adulthood and result in right to

left shunt.
Clinical Manifistation:
Most often is aymptomatic.
Subtle failure to thrive , exercise intolerance .
 
Physical Findings:
Usually discovered incidentally during general physical
examination.

1-  Right ventricular impulses palpable at the left sternal border.

2-  Loud S1.

  3- Fixed and widely split S2 .

 Continue…

4-Systolic ejection murmur at middle and upper left

sternal border.

3- Mid diastolic rumbling murmur at the lower left

sternal border.

These findings indicate Qp:Qs ratio at least 2:1 .

Diagnosis:
Chest X-Ray : shows
*variable degrees of right ventricle and atrial enlargement .
* Enlargement of pulmonary artery.
*Increase pulmonary vascularity .
 
2-ECG: signs of right ventricle volume overload.
Right axis deviation
Right ventricular conduction delay
(rsR’).

 
3-Echocardiogram : Increased right ventricular end diastolic dimensions.
Abnormal motion of ventricular septum
Location and size of ASD
Confirmation of the shunt.
 
4- Cardiac Catheterization: confirmation of the defect
Measuring of the shunt and PVP. ( it’s of considerable volume when if more than or equals 20/min/m2.
Note : in the case of classical features of ASD on physical examination , CXR and ECHO which Identify isolated seccundum
ASD , there is no need for the cardian cath before surgery.
Continue…
3-Echocardiogram :
* Increased right ventricular end diastolic dimensions.
*Abnormal motion of ventricular septum .
*Location and size of ASD.
*Confirmation of the shunt.

4- Cardiac Catheterization:
*confirmation of the defect
*Measuring of the shunt and PVP.
Continue..

Note : in the case of classical features of ASD on

physical examination , CXR ,and ECHO which
Identify isolated secundum ASD , there is no need for
the cardiac cath before surgery.

left- to- right shunt, is of considerable volume when if

more than or equals 20L/min/m2.
Prognosis&Complications:
15% ostium secundum in term infants close
spontaneously .
Symptoms don’t usually appear until the
third decade in life or later :
Pulmonary hypertension, atrial
dysrhythmias, AV valve insufficiency, and
heart failure , initially appears during volume
overload in pregnancy .
Infective Endocarditis is extremely rare and
there is no need for antibiotic prophylaxis.
 Treatment:

Surgery or transcatheter device closure.

Indications : 1-all symptomatic patients.

2- asymptomatic patients with QP:QS
ratio of at least 2:1.

Timing : elective closure after one year and before

entry of school.
Continue…
Post Op complications : (HF , AF )
The risk is greater if done after 20 years of age.
 
Post Op prognosis :
1- symptoms disappear rapidly.
2- enhanced physical development and activity.
3-Heart size decreases to normal .
Ostium primum:

An ostium primum atrial septal defect (ASD) is

located in the most anterior and inferior aspect of
the atrial septum.

Cleft of anterior leaflet of the mitral valve is

noticed.

Tricuspid valve is usually functionally normal.

Ostium primum is
the next most common
type and is located in the
lower portion of the atrial
septum. This type often
have a mitral valve defect
associated with it called a
mitral valve cleft. A mitral
valve cleft is a slit-like or
elongated hole in anterior
leaflet
Development:
During fetal development, the rudimentary atrium is
divided by the septum primum, except for an anterior
and inferior space that is the ostium primum. The
ostium primum is sealed by fusion of the superior and
inferior endocardial cushions around the fifth week of
gestation. Failure to do so results in an ostium
primum ASD.
Ostium primum ASDs are most commonly associated
with Down syndrome (trisomy 21).
Continue…
Sex: The male-to-female ratio is 1:1.

Age: Patients with smaller defects and little

or no mitral regurgitation may present at any
age with a murmur and/or an abnormal
electrocardiogram (ECG). Those with more
severe mitral regurgitation typically present
with CHF in the first 1-2 years of life.
Pathophysiology:
Shunting is predominantly left-to-right in the absence
of pulmonary vascular disease or significant right
ventricular outflow tract obstruction.
This results in volume overload of the right atrium
and ventricle and pulmonary overcirculation.
If the mitral valve cleft causes significant mitral
regurgitation, the left side of the heart becomes
volume overloaded. A left ventricle to right atrium
shunt can be present, which further overloads both
the right and left heart.
Clinical presentation:

Usually asymptomatic and discovered incidentally during general

physical examination.
Exercise intolerance , easy fatiguabiltiy , recurrent pneumonia
may occur with large shunt and severe mitral insufficiency .

Physical Findings :
1-Loud S1
2-Fixed widely splitted S2
3-Pulmonary systolic ejection murmur and mid diastolic rumbling
murmur
4-Apical harsh holosystolic mumur that radiates to the axilla ( mitral
insufficiency )
 
Treatment:
the primum form of ASD is not amenable to device
closure in the cardiac catheterization laboratory. The
device is unable to be adequately seated secondary
to an inadequate inferior rim of atrial septal tissue.

Definitive management of hemodynamically

significant primum ASDs and partial AV canal defects
is operative repair.
Continue…
Timing:Patients with an isolated ostium primum ASD
usually are referred for elective repair when aged 3-5
years. repair may be recommended at an earlier age
because of significant CHF or because of failure to
thrive,

all patients with documented mitral regurgitation be

referred upon presentation, as once regurgitation
develops, making repair less successful.
Coplications& prognosis:
Infective endocarditis remains both a
preoperative and a postoperative
complication. In a study from the Oregon
Health Sciences University, the 30-year
postoperative incidence of infective
endocarditis was 2.8% among patients with
ostium primum atrial septal defects.

Surgical repair generally improves life

expectancy and alters the natural course of
the disease.
Sinus venosus:

Situated in the upper part ofAS,in close

relation to entry of SVC
.
Sinus venosus ASDs account for only 10% of
ASDs

always associated with anomalous pulmonary

venous drainage of the right upper pulmonary
vein into the SVC. .
A sinus venosus defect is the least
common type of ASD and is located
in the upper portion of the atrial
septum. A sinus venosus ASD often
has an abnormal pulmonary vein
connection associated with it. Four
pulmonary veins, two from the right
lung and two from the left lung,
normally return red blood to the left
atrium. Usually with a sinus venosus
ASD, a pulmonary vein from the
right lung will be abnormally
connected to the right atrium instead
of the left atrium. This is called an
anomalous pulmonary vein.
Continue…
Almost all patients with sinus venosus ASD becom
symptomatic when they are younger than 40 years.
The haemodynamic disturbance, clinical picture;ECG;
C-X ray are similler to ostium secundum.

Diagnosis by Echocardiography & cardiac cath.

Anatomical correction: the insertion of a patch to close
the defect.

excellent surgical results with a mortality rate near 0%

can be expected. If repair done before age 15 years.
Coronary sinus:
The least common type of ASD is termed an
unroofed coronary sinus or coronary sinus
septal defect. A portion of the roof of the
coronary sinus is missing, allowing shunting
of blood from the left atrium into the coronary
sinus and subsequently into the right atrium.
usually associated with other forms of
congenital heart disease(eg.persistant LSVC.
also may be associated with other forms of
ASD, such as the secundum or primum types,
Continue…

Patients usually present with symptoms typical of

other ASDs or with symptoms related to their
concomitant defects. In the presence of a LSVC,
these fenestrations can result in a history of brain
abscess or cerebral embolism.