ASD

Question: Ostium secondum type of ASD

 ASD is an opening in the atrial septum permitting shunting of blood between the
atria.

Prevalence:
o ASD (ostium secundum defect) occurs as an isolated anomaly in 5% to 10% of
all congenital heart diseases (CHDs).
o Sex: Female preponderance (male-to-female ratio of 1:2)
o About 30% to 50% of children with congenital heart defects have an ASD as part
of the cardiac defect..
Pathology / Types of ASD:
 During the embryologic development of the heart, a septum grows toward the
endocardial cushions to divide the atria. Failure of septal growth or excessive
reabsorption of tissue leads to ASDs
 Types of ASD
Three types of atrial septal defects (ASDs) occur in the atrial septum .
1. Ostium secundum ASD
2. Ostium primum ASD
3. Sinus venosus ASD
4. coronary sinus ASD (rare)
 Ostium secundum ASD
 Most common ASD (50% to 70% of all ASDs)
 The defect is present at the site of fossa ovalis (central portion of the septum)
 It usually occurs in isolation. Rarely, this may be associated with mitral valve
prolapse syndrome (MVPS) or MS in Lutembacher’s syndrome.
 Ostium primum ASD
 Isolated ostium primum ASD occurs in about 15% of all ASDs
 Ostium primum defects occur in about 30% of all ASDs, if those that occur as
part of complete ECD are included.
  located near the endocardial cushions (lower part of the septum), may be
part of a complete atrioventricular canal defect or may be present with an
intact ventricular septum.
 Sinus venosus ASD:
 Occurs in about 10% of all ASDs.
 The defect is near the entrance of the superior vena cava (SVC) or inferior
vena cava (IVC) to the right atrium (RA). (posterosuperior atrial septum)
 Partial anomalous pulmonary venous return (PAPVR) is common with a sinus
venous defect, as the location of the sinus venosus is intimately related to the
right upper pulmonary vein.
 Coronary sinus ASD:
 the defect is in the roof of the coronary sinus and the LA blood shunts through
the defect and the coronary sinus ostium into the RA, which produces clinical
pictures similar to those in other types of ASD.
Hemodynamics:
o In acyanotic patients with atrial septal defect (ASD), the direction of the shunt is
from left to right.
o The magnitude of the left-to-right shunt is determined by the size of the defect
and the relative compliance of the right ventricle (RV) and left ventricle (LV).
o Because the compliance of the RV is greater than that of the LV, a left-to-right
shunt is present.
o The magnitude of the shunt is reflected in the degree of cardiac enlargement.
o There is volume overload to the RA and right ventricle (RV) and an increase in
pulmonary blood flow.
o The structures enlarged are-RA, RV & main PA
o The dilated RV cavity prolongs the time required for depolarization of the RV
because of its longer pathway, producing a right bundle branch block (RBBB)
pattern (with rsR' in V1) in the ECG.
o Systolic ejection murmur at the pulmonary area: The heart murmur in ASD is
due to relative stenosis of the pulmonary valve because of the increased blood
flow passing through this normal-sized valve.
The murmur is systolic in timing and is maximal at the pulmonary valve area (i.e.,
at the upper left sternal border).
(Note: The heart murmur in ASD is not caused by the shunt at the atrial level.
Because the pressure gradient between the atria is so small and the shunt occurs
throughout the cardiac cycle, in both systole and diastole, the left-to-right shunt is
silent.)
o When the shunt is large, increased blood flow through the tricuspid valve results
in a relative stenosis of this valve, producing a diastolic murmur at the tricuspid
valve area (i.e., lower left sternal border).
o Fixed, widely split S2:
 Reason for widely split S2
 The widely split S2 in ASD results partially from RBBB. The RBBB delays
both the electrical depolarization of the RV and the ventricular contraction,
resulting in delayed closure of the pulmonary valve.
 The large volume also results in prolonged ejection phase of theRV. The
pulmonary valve closes late and P2 is delayed.
  Reason for fixed split:
The large atrial shunt tends to abolish respiration-related variations in
systemic venous return to the right side of the heart, resulting in a fixed S2.
Since the RV is fully loaded, further increase in the RV volume during
inspiration cannot occur.
o The cardiac apex is formed by the enlarged RV and the accentuated P2 is well
audible at the apex as well.
o The lung fields are plethoric.
o Infants and small children rarely manifest with the clinical findings even in the
presence of a moderately large ASD until they are 3 to 4 years of age. It is
because the compliance of the RV improves slowly so that any significant shunt
does not occur until that age.
o Children with ASD rarely experience congestive heart failure (CHF), even in the
presence of a large left-to-right shunt. The PAs can handle an increased amount
of blood flow for a long time without developing CHF because there is no direct
transmission of the systemic pressure to the PA, and PA pressure remains
normal. However, CHF and pulmonary hypertension eventually develop in the
third and fourth decades of life.
Clinical manifestations:
History:
o The patients are usually asymptomatic.
o Mild effort intolerance and frequent chest infections are the only symptoms
o CCF is rare
Physical examination:
o A relatively slender body build is typical
o CVS examination
o The cardiac enlargement is only mild to moderate
o Inspection:
 Apical impulse-normal
 Precordial movement
 Hyperdynamic right ventricular impulse along the left sternal border due to
RVH.
 Pulsatile pulmonary trunk in 2nd left ICS
o Palpation:
 Parasternal heave on the left side
 Pulsations in 2nd left ICS due to dilation of the pulmonary trunk
 Systolic thrill may be palpable at the 2nd left interspace in 10% of patients.
o Auscultation
 S1 –normal or may be loud due to a loud tricuspid component of S1.
 S2- widely split and fixed split, P2 accentuated
 Murmurs
o Shunt murmur –absent
o Flow murmurs
1. Systolic ejection murmur at the pulmonary area, grade 2 to 3/6 is
characteristic of moderate-size ASD (resulting from relative PS)
2. Middiastolic murmur may be audible at the lower left sternal border
with a large L-R shunt (resulting from relative TS)
Investigations:
 ECG:
 Ostium secundum ASD
 shows right axis deviation (RAD) (+90 to +180 degrees)
 mild right ventricular hypertrophy (RVH) or
 right bundle branch block (RBBB) with an rsR´ pattern in V1.
 Ostium primum ASD
Left axis deviation beyond -300
 Chest x-ray
 cardiomegaly with enlargement of the RA and RV.
 prominent main pulmonary artery (MPA) segment & increased pulmonary
vascular markings(plethoric lung fields) are seen when the shunt is significant.
(Note-the LA is not enlarged because the increased pulmonary venous return to the
LA does not stay in that chamber. Rather it is shunted immediately to the RA. The
absence of left atrial enlargement is one of the helpful x-ray signs for differentiating
an ASD from a ventricular septal defect (VSD).
(RV enlargement in lateral view-obliteration of the retrosternal space)
 2 D Echo shows the position and the size of the defect. Increased size of RV with
paradoxical ventricular septal motion.
 Cardiac catheterization is usually not necessary.
Assement of the severity:
o The size of the L-R shunt is directly proportional to the intensity of the 2 murmurs
and the heart size.
o The larger the shunt the more the cardiomegaly and the louder the pulmonary
and tricuspid murmurs.
DDs
 PS
 High VSD
 Ebstein anomaly
 AS
 Partial /total anomalous venous drainage
 Acyanotic TOF
 Pulmonary htn
Natural history/complications:
o Spontaneous closure of the defect
 ASD < 3 mm in size diagnosed before 3 mo of age100% spontaneous
closure by 1 ½ yrs of age.
 ASD 3 to 8 mm in size80% spontaneous closure by 1 ½ yrs of age.
 ccurs more than 80% of the time in patients with defects 3 to 8 mm before 1/2
years of age.
o An ASD with a diameter > 8 mm rarely closes spontaneously. The defect may
reduce in size in some patients.
o Most children with an ASD remain active and asymptomatic. Rarely, congestive
heart failure (CHF) can develop in infancy.
o If the defect is large and left untreated, CHF & pulmonary hypertension develops
in the third and fourth decades of life.
o Cerebrovascular accident due to paradoxical embolization through an ASD is
possible.-rare complication.
o With or without surgery, atrial arrhythmias (flutter or fibrillation) may occur in
adults.
o Infective endocarditis does not occur in patients with isolated ASDs.
Management:
Medical
1. Exercise restriction is not required.
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 of ASD:
o Non surgical closure using a catheter-delivered closure device(Amplatzer septal
occluder) has become a preferred method, provided the indications are met.
o These devices are applicable only to secundum ASD with an adequate septal
rim.
o Indications:
 Secundum ASD, measuring 5 mm or more in diameter (but <32 mm) with an
adequate septal rim (4 mm), and
 Significant L-R shunt with clinical evidence of RA and RV volume overload.
o Timing of closure:
 not entirely clear
 Considering the possibility of spontaneous closure, it is wise not to use the
device in infancy unless the patient is symptomatic with heart failure.
o Post-device closure follow-up:
 Following the device closure, the patients are placed on aspirin 81 mg per day
for 6 months.
o Advantages of nonsurgical closure
 avoidance of cardiopulmonary bypass with its attendant risk
 avoidance of pain and residual thoracotomy scars
 a less than 24-hour hospital stay, and rapid recovery.
o Disadvantages: All these devices are associated with a higher rate of small
residual leak than is operative closure.
Surgical closure of ASD:
o For patients with primum ASD and sinus venosus defect, and some patients with
secundum ASD for which the device closure is considered inappropriate.
o Indications & timing:
 significant L-R shunt with Qp/Qs of 1.5:1 or greater. Surgery is usually
delayed until 2 to 4 years of age, unless congestive heart failure (CHF)
develops.
 If CHF does not respond to medical management, surgery is performed
during infancy, if device closure is considered inappropriate.
 If oxygen and other medical therapy are needed for infants with associated
bronchopulmonary dysplasia and the device closure is not considered
appropriate, surgery is performed during infancy.
o Open repair with a midsternal incision or minimally invasive cardiac surgical
technique (with a smaller skin incision) is used.
o Surgical mortality rate is less than 1%.
o Contraindications: High pulmonary vascular resistance (PVR >10 units/m2) is a
contraindication to surgery.
o Complications: Cerebrovascular accident and postoperative arrhythmias may
develop in the immediate postoperative period.
o Postsurgical follow-up. Atrial or nodal arrhythmias occur in 7% to 20% of patients.
Occasional sick sinus syndrome requires pacemaker therapy.