Congenital heart disease chest x-ray (an approach)
With the advent of echocardiography, and cardiac CT and MRI, the role of chest radiographs in evaluating congenital heart disease has been largely been relegated to one of historical and academic interest, although they continue to crop up in radiology exams. In most instances a definite diagnosis cannot be made, however the differential can be narrowed to a few likely diagnoses.
A systematic approach to interpreting paediatric chest radiographs is required. A detailed understanding of the normal contours of the cardiomediastinum on chest radiography is essential if abnormalities are to be detected, as well as knowing the range of normal for pulmonary vasculature marking.
Interpretation is also made significantly easier (and you could argue that it is cheating) if knowledge of whether the child is cyanotic or acyanotic is available.
Step 0: technical assessment
Before you even begin it is essential to assess the technical adequacy of the chest x-ray. The film should not be rotated, over or under exposed and no excessive lordotic or kyphotic angulation should be present. An adequate inspiratory effort must also have been obtained.
Step 1: pulmonary vasculature
Establishing whether pulmonary vasculature is normal, congested (active or passive) or decreased is essential in narrowing the differential.
Normal pulmonary vasculature
Normal vasculature is unhelpful in that it does not narrow the differential. It may represent milder or earlier forms of congenital heart defects, or alternatively represent abnormalities that do not result in altered pulmonary blood flow or pressures, such as simple valvular abnormalities of coarctation of the aorta 1.
Congested pulmonary vasculature
Congested pulmonary vasculature can be active or passive, and represents increased blood flow and increased pulmonary venous pressure respectively.
Active congestion is therefore seen in left to right shunts when right ventricular output is approximately 2.5 times that of the left ventricle 1. Although the vessels are enlarged, are seen more peripherally than normal and may be tortuous, in contrast of passive congestion the margins remain distinct as there is little interstitial oedema.
Passive congestion is due to elevated pulmonary venous pressure and reflects left cardiac dysfunction or obstruction.
Decreased pulmonary vasculature
Oligaemia of the pulmonary vasculature represents decreased blood flow through the pulmonary circulation, usually as a result of right ventricular outflow obstruction with associated right to left shunt.
If the proximal pulmonary arteries are enlarged, with pruning of the peripheral vascular markings, then pulmonary arterial hypertension should be considered.
Step 2: aorta
The aorta may be altered in size, location or shape.
The aorta may be normal, increased of decreased in size.
An enlarged aortic knob may represent:
- post-stenotic dilatation
- increased blood flow
- systemic hypertension
Although most right sided aortic arches are incidental with only ~10% being associated with congenital heart disease, in the setting of mirror image branching anatomy the vast majority do have cardiac anomalies, most frequently tetralogy of Fallot 1-2.
Step 3: pulmonary artery
The pulmonary artery may be normal, increased or decreased in size.
A small or inapparent pulmonary artery can be due to either it being small due to congenital hypoplasia or aplasia, decreased pulmonary flow as a result of pulmonary outflow obstruction as in tetralogy of Fallot, or it being abnormally located as is the case in truncus arteriosus and transposition of the great arteries 1.
An enlarged pulmonary artery may represent:
- post-stenotic dilatation
- in pulmonary valve stenosis, the left pulmonary artery preferentially dilates due to the orientation of the stenotic jet
- increased pulmonary blood flow
- left-to-right shunts
- pulmonary valvular insufficiency
- pulmonary arterial hypertension
- both the right and left pulmonary arteries will enlarge which distinguishes this from pulmonary valve stenosis; there may also be peripheral pulmonary vascular pruning
Step 4: cardiac size and shape
Finally the heart itself may be abnormal in size or demonstrate alterations in shape representing underlying chamber enlargement or anatomic anomalies. It is also important to assess for the correct orientation of the heart by looking for the liver/stomach below the diaphragm and reviewing side markers.
Step 5: spine, rib cage and sternum
The vertebrae should be assessed for congenital anomalies including scoliosis which is present in 6% of patients with a congenital heart defect, but only 0.4% of the normal population 2.
- congenital heart disease
- cyanotic congenital heart disease
- acyanotic congenital heart disease
- plethoric congenital heart disease
- oligaemic congenital heart disease
- frontal projection
- lateral projection
- lateral decubitus
- congenital heart disease
- lines and tubes
- prosthetic heart valve
- chest x-ray in the exam setting
- cardiomediastinal contour
- chest radiograph zones
- normal chest x-ray appearance of the diaphragm
- nipple shadow
lines and stripes
- anterior junction line
- posterior junction line
- right paratracheal stripe
- left paratracheal stripe
- posterior tracheal stripe / tracheo-oesophageal stripe
- posterior wall of bronchus intermedius
- right paraspinal line
- left paraspinal line
- aortic-pulmonary stripe
- aortopulmonary window
- azygo-oesophageal recess
- air bronchogram
- big rib sign
- Chang sign
- coin lesion
- dense hilum sign
- double contour sign
- extrapleural sign
- haystack sign
- hilum overlay sign
- hilum convergence sign
- holly leaf sign
- finger in glove sign
- flat waist sign
- Fleishner sign
- ginkgo leaf sign
- Golden S sign
- incomplete border sign
- juxtaphrenic peak sign
- medial stripe sign
- more black sign
- Naclerio V sign
- Shmoo sign
- silhouette sign
- steeple sign
- spinnaker sign
- third mogul sign
- walking man sign
- water bottle sign
- wave sign
- Westermark sign
Congenital heart disease
There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, 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
- normal relationship between chambers and valves
- atrioventricular valves
- outflow tract
- great vessels
- venous inflow
- anomalous valves
- abnormal relationship of chambers and valves
- atrioventricular abnormality
- great vessel connection abnormality
- conotruncal cardiac anomalies
- pentalogy of Cantrell
- Shone syndrome
- congenital heart disease - chest x-ray approach
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
- 1. The Brant and Helms Solution: Fundamentals of Diagnostic Radiology, Third Edition, Plus Integrated Content Website (Brant, Fundamentals of Diagnostic Radiology). Lippincott Williams & Wilkins. ISBN:0781761352. Read it at Google Books - Find it at Amazon
- 2. Radiological Imaging of the Neonatal Chest (Medical Radiology / Diagnostic Imaging). Springer. ISBN:3540667032. Read it at Google Books - Find it at Amazon