Atrial septal defect
David Carroll ◉ and [Link] Frank Gaillard ◉ ◈ et al.
Atrial septal defects (ASDs) are the second most common congenital heart defect after
ventricular septal defects and the most common to become symptomatic in adulthood.
They are characterized by an abnormal opening in the atrial septum allowing communication
between the right and left atria. Due to the low pressures of the atria, the lesion is typically
asymptomatic until adulthood despite 2-4 times the normal pulmonary blood flow. Gradual (high
output) congestive cardiac failure eventually develops, usually becoming symptomatic by the age
of 30.
Epidemiology
ASD accounts for ~10% of congenital heart disease 7. There may be greater female predilection.
Clinical presentation
Most patients are asymptomatic but as cardiac failure develops they may present with shortness
of breath, palpitations and weakness 7. Chest auscultation classically reveals an ejection systolic
murmur heard at the left upper sternal border, attributed to increased flow across the pulmonary
valve rather than blood shunting across the defect itself 7.
ECG
incomplete right bundle branch block (RBBB morphology with QRS duration between
110-120 ms)
o increased specificity with crochetage sign in the inferior (II, III, aVF) leads
right precordial (V1-3) "defective T waves"
o describes biphasic morphology, initial T wave flattening or inversion and sharp,
positive upstroke to terminal positive deflection 10
o peak of the T wave delayed when compared to lateral precordial leads
notching of the terminal upstroke of the R wave (crochetage sign) correlates with size of
ASD and implies a greater degree of shunting 11
left axis deviation
o characteristic of ostium primum defects and anatomical distortion of the left
bundle branch fascicles
o associated first degree AV block
right axis deviation
o suggests ostium secundum defect
low atrial ectopic rhythms
o negative P wave polarity in lead II
o found in sinus venosus ASDs
Pathology
Classification
There are four major types of ASD 4, distinguished according to their location within the septum:
secundum ASD
o 60-90% of all ASDs
o usually an isolated abnormality
primum ASD
o 5-20%
o associated with cleft anterior mitral valve leaflet (partial atrioventricular septal
defect)
sinus venosus
o 5%
o associated with anomalous right pulmonary venous return to the superior vena
cava or right atrium
coronary sinus type ASD ("unroofed coronary sinus")
o <1%
o see: unroofed coronary sinus
Associations
ASDs usually tend to be isolated anomalies, of which its associations include:
Down syndrome (ostium primum defect)
Holt-Oram syndrome
Ellis-van Creveld syndrome
mitral valve prolapse
Lutembacher syndrome
anomalous pulmonary venous return (especially with sinus venosus defects)
o total anomalous pulmonary venous return (TAPVR)
o partial anomalous pulmonary venous return (PAPVR)
A patent foramen ovale (PFO) is a form of atrial septal defect.
Radiographic features
Plain radiograph
can be normal in early stages when the ASD is small
signs of increased pulmonary flow (pulmonary plethora or shunt vascularity)
o enlarged pulmonary vessels
o upper zone vascular prominence
o vessels visible to the periphery of the film
o eventual signs of pulmonary arterial hypertension
chamber enlargement
o right atrium
o right ventricle
o note: left atrium is normal in size unlike VSD or PDA
o note: aortic arch is small to normal
Treatment and prognosis
ASDs do not cause any impairment in cardiac function in utero and even most neonates are
asymptomatic. The defect can be closed surgically or percutaneously (e.g. atrial septal occlusion
device). However, careful evaluation has to be made to ensure lack of development of elevated
right heart pressures or a right to left shunt before any intervention.
Complications
In approximately 10% of untreated patients, pulmonary hypertension develops. In this situation,
flow through the shunt eventually reverses and becomes right to left. The patient then becomes
cyanotic. This is known as the Eisenmenger syndrome.
Other complications include:
paradoxical emboli
cardiac conduction defects, e.g. atrial fibrillation, flutter.
References
Related Radiopaedia articles
Congenital heart disease
There is more than one way to present the variety of congenital heart diseases. Whichever way
they are categorized, it is helpful to have a working understanding of normal and fetal
circulation, as well as an understanding of the segmental approach to imaging in congenital heart
disease.
congenital heart disease
o subtypes
cyanotic congenital heart disease
acyanotic congenital heart disease
plethoric congenital heart disease
oligaemic congenital heart disease
o normal relationship between chambers and valves
shunting
ASD
patent foramen ovale (PFO)
VSD
Gerbode defect
AVSD
aortopulmonary septal defect
PDA
stenosis
atrioventricular valves
mitral atresia
congenital mitral stenosis
tricuspid atresia
outflow tract
aortic atresia
left ventricular outflow tract obstruction
pulmonary atresia
congenital aortic stenosis
congenital pulmonary stenosis
cor triatriatum
great vessels
coarctation of the aorta
interrupted aortic arch
venous inflow
partial anomalous pulmonary venous return
total anomalous pulmonary venous return
hypoplasia
hypoplastic left heart syndrome
hypoplastic right heart syndrome
anomalous valves
Ebstein anomaly
o abnormal relationship of chambers and valves
atrioventricular abnormality
when associated with a univentricular heart
double inlet right ventricle
double inlet left ventricle
atrioventricular discordance
great vessel connection abnormality
conotruncal cardiac anomalies
transposition of the great arteries
D-loop transposition of the great arteries
L-loop transposition of the great arteries
tetralogy of Fallot
truncus arteriosus
double outlet left ventricle
double outlet right ventricle
Taussig-Bing anomaly
pentalogy of Cantrell
Shone syndrome
o congenital heart disease - chest x-ray approach
o surgical repairs (mnemonic)
arterial switch procedure
Blalock-Taussig shunt
double switch procedure
Fontan procedure
Glenn procedure
Mustard repair
Norwood procedure
Pott shunt
pulmonary artery banding
Rastelli procedure
Sano shunt
Senning repair
total repair of tetralogy of Fallot (TOF)
unifocalisation procedure
Waterston shunt