Atrial septal defect

David Carroll ◉ and A.Prof 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