Carotid artery stenosis

Congenital Heart Disease

TOF
IUGR
Fetal Doppler assessment
Pulmonary embolism
Renal artery stenosis
Varicose vein
Takayasu arteritis
Coarctation of aorta
Portal vein doppler

CAROTID ARTERY STENOSIS

Extracranial carotid artery stenosis

Usually caused by an atherosclerotic process and is one of the major causes of stroke and transient
ischemic attack (TIA).

Carotid artery stenosis can result in wide-ranging stroke syndromes or TIA symptoms.

Pathology

The plaques formed in the carotid vessels can be divided into four types:

type I: predominantly haemorrhage, lipid, cholesterol and proteinaceous material

type II: dense fibrous connective tissue with >50% volume of haemorrhage, lipid, cholesterol
and proteinaceous material
type III: dense fibrous connective tissue with <50% volume of haemorrhage, lipid, cholesterol
and proteinaceous material
type IV: dense fibrous connective tissue

They can be termed as:

homogenous (type III and IV)

heterogenous (type I and II)

Angiographic calculation of ICA stenosis percentage:

% ICA stenosis = (1 - [narrowest ICA diameter/diameter normal distal cervical ICA]) x 100

The European Carotid Surgery Trial (ECST) also demonstrated benefits for carotid endarterectomy in
patients with symptomatic higher than 80% ICA stenosis.

On grayscale, characterization of plaques can be done:

type I: predominantly hypoechoic with thin echogenic rim

type II: echogenic plaque with >50% hypoechoic areas
type III: echogenic plaque with <50% hypoechoic areas
 type IV: uniformly echogenic plaque

They can be termed as:

homogenous (type III and IV)

heterogenous (type I and II)

Doppler ultrasound has become the first choice for carotid stenosis screening, permitting the evaluation
of both the macroscopic appearance of plaques as well as flow characteristics. Hemodynamically
significant carotid stenosis is usually referred to a further CTA or MRA study.

Society of Radiologists in Ultrasound (SRU) consensus

This consensus developed recommendations for the diagnosis and stratification of ICA stenosis.

Normal

ICA PSV is <125 cm/sec and no plaque or intimal thickening is visible sonographically
additional criteria include ICA/CCA PSV ratio <2.0 and ICA EDV <40 cm/sec

<50% ICA stenosis

ICA PSV is <125 cm/sec and plaque or intimal thickening is visible sonographically
additional criteria include ICA/CCA PSV ratio <2.0 and ICA EDV <40 cm/sec

50-69% ICA stenosis

ICA PSV is 125-230 cm/sec and plaque is visible sonographically

additional criteria include ICA/CCA PSV ratio of 2.0-4.0 and ICA EDV of 40-100 cm/sec

70% ICA stenosis but less than near occlusion

ICA PSV is >230 cm/sec and visible plaque and luminal narrowing are seen at gray-scale and
colour Doppler ultrasound (the higher the Doppler parameters lie above the threshold of 230
cm/sec, the greater the likelihood of severe disease)
additional criteria include ICA/CCA PSV ratio >4 and ICA EDV >100 cm/sec

Near occlusion of the ICA

velocity parameters may not apply, since velocities may be high, low, or undetectable
diagnosis is established primarily by demonstrating a markedly narrowed lumen at colour or
power Doppler ultrasound

Total occlusion of the ICA

no detectable patent lumen at gray-scale US and no flow with spectral, power, and colour
Doppler ultrasound
there may be compensatory increased velocity in the contralateral carotid
Sonographic NASCET Index

This study proposed the incorporation of distal ICA flow velocity information on the conventional carotid
Doppler study improving the diagnostic accuracy of PSV.

<15% stenosis

deceleration spectral broadening with a peak systolic velocity (PSV) <125 cm/s
16-49% stenosis
pansystolic spectral broadening with a PSV <125 cm/s

50-69% stenosis

pansystolic spectral broadening with a PSV of >125 cm/s and

end diastolic velocity (EDV) <110 cm/s or ICA/CCA PSV ratio >2 but <4

70-79% stenosis

pansystolic spectral broadening with PSV >270 cm/s or

EDV >110 cm/s or
ICA/CCA PSV ratio >4

80-99% stenosis:

EDV >140 cm/s

Complete occlusion:

No flow; terminal thump

APPROACH TO CONGENITAL HEART DISEASE CHEST X-RAY

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.

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. Although the vessels are enlarged, are seen more peripherally than
normal and may be tortuous, in contrast to 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.

Size

The aorta may be normal, increased or decreased in size.

An enlarged aortic knob may represent:

1. Post-stenotic dilatation
2. Increased blood flow
patent ductus arteriosus (PDA)
truncus arteriosus
valvular insufficiency
severe tetralogy of Fallot
3. Systemic hypertension

A small aortic knob usually represents reduced blood flow typically due to ASD or VSD. It may also be
primarily hypoplastic in hypoplastic left heart syndrome.

Position

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.

Shape

The most common abnormality of shape is the so-called figure of 3 sign seen in aortic coarctation.

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 secondary 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.

An enlarged pulmonary artery may represent:

1. Post-stenotic dilatation
in pulmonary valve stenosis, the left pulmonary artery preferentially dilates due to the
orientation of the stenotic jet
2. Increased pulmonary blood flow
left-to-right shunts
pulmonary valvular insufficiency
3. 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.

Ribs may demonstrate notching in coarctation of the aorta, or may be only number 11 in patients with
Down syndrome. Down syndrome children may also show hypersegmented sternums.

Cyanotic CHD

Increased pulmonary vascularity

total anomalous pulmonary venous return (TAPVR) (types I and II)

transposition of the great arteries (TGA)
truncus arteriosus (types I, II and III)
large AVSD
single ventricle without pulmonary stenosis

Decreased pulmonary vascularity

Tetralogy of Fallot
 Pentalogy of Cantrell: Omphalocele, Ectopia cordis (abnormal location of heart), Diaphragmatic
defect, Pericardial defect or sternal cleft, Ccardiovascular malformations [ventricular septal
defect (VSD), atrial septal defect (ASD), tetralogy of Fallot, left ventricular diverticulum]
Many other combined and infrequent anomalies such as
double outlet right ventricle (DORV) with pulmonary stenosis
single ventricle with pulmonary stenosis
Ebstein anomaly with atrial septal defect
Uhl anomaly

Acyanotic congenital heart disease

Increased pulmonary vascularity

ventricular septal defect (VSD)

atrial septal defect (ASD)
atrioventricular septal defect (AVSD)
patent ductus arteriosus (PDA)
other less common
aortopulmonary window
ruptured aneurysm of Valsalva
coronary artery fistula
partial anomalous pulmonary venous return (PAPVR)

Normal pulmonary vascularity

small shunts
aortic valve stenosis
aortic coarctation
pulmonary stenosis

TETRALOGY OF FALLOT

Second most common cyanotic congenital heart condition and has been classically characterised by the
combination of ventricular septal defect (VSD), right ventricular outflow tract obstruction (RVOTO),
overriding aorta, and a late right ventricular hypertrophy.

The presentation relies on the degree of right ventricular outflow tract obstruction (RVOTO). Typically
this is significant, resulting in cyanosis evident in the neonatal period, as a consequence of the right to
left shunt across the VSD. In cases where outflow obstruction is minimal, cyanosis may be unapparent
(pink tetralogy) resulting in delayed presentation, even into adulthood, although this is rare.

Pathology

Tetralogy of Fallot is classically characterised by four features which are:

1. ventricular septal defect (VSD)

 may be multiple in ~5% of cases
2. right ventricular outflow tract obstruction (RVOTO) due to
infundibular stenosis, or
hypoplastic pulmonary valve annulus, or
bicuspid pulmonary valve, and/or
hypoplasia of pulmonary artery
3. overriding aorta
4. right ventricular hypertrophy: only develops after birth

The right ventricular hypertrophy is a result of the VSD and right ventricular outlet obstruction, both
contributing to elevated resistance to right heart emptying.

Associations

Cardiovascular associations

right-sided aortic arch: seen in ~25% of cases

pulmonary hypoplasia +/- atresia: particularly important in determining treatment, when there
is pulmonary atresia it is sometimes termed pseudotruncus arteriosus
atrial septal defect (ASD) or patent ductus arteriosus (PDA) (termed pentalogy of Fallot)
coronary artery anomalies: seen in 3% of cases
persistent left sided superior vena cava

Extra cardiovascular associations: may be present in ~15% of cases

congenital lobar emphysema

DiGeorge syndrome
fetal rubella syndrome
prune belly syndrome
tracheoesophageal fistula
VACTERL association

Plain radiograph

Chest radiographs may classically show a "boot-shaped" heart with an upturned cardiac apex
due to right ventricular hypertrophy and concave pulmonary arterial segment.
Most infants with TOF, however, may not show this finding.
Pulmonary oligaemia due to decreased pulmonary arterial flow.
Right-sided aortic arch is seen in 25%.

Echocardiography

Echocardiography allows direct visualization of the abnormal anatomy and remains the primary
modality for the diagnosis of TOF.
It has limitations on assessing associated extracardiac anomalies (e.g. peripheral pulmonary
stenosis and atresia).

CT/CTA
 MDCT is useful in demonstrating the complex cardiovascular morphology of TOF, especially the
anatomy of the pulmonary and coronary arteries as well as identification of aortopulmonary
collateral vessels (MAPCAs).
MDCT can be used to evaluate post-surgical changes (e.g. patency of palliative shunts) and
complications.

MRI

MRI has the great advantage of providing both exquisite anatomical details and functional
information without ionizing radiation.
The detailed assessment of the pulmonary artery is of particular importance because repair of
the cardiac defects without addressing pulmonary artery hypoplasia/stenosis has a poor
outcome.

The main pulmonary artery or right pulmonary artery diameter should be compared to that of
the ascending aorta. A ratio of <0.3 usually signifies that primary repair would be unsuccessful,
and a bridging shunt operation may be of benefit 8.
Assessment of coronary artery origin is also essential to surgical planning.

Treatment and prognosis

Approximately 90% of untreated TOF patients succumb by the age of 10 years 6. Over the years many
surgical approaches were performed until the current primary repair was developed. Shunts are
nowadays only performed as a palliative procedure in inoperable cases or to bridge patients until the
repair can be carried out, typically in the setting of pulmonary arterial hypoplasia 8.

Shunt operations included:

Shunts: designed to reduce cyanosis

Pott shunt
Waterston shunt
Blalock-Taussig shunt: still performed in selected cases
Primary repair is now the preferred treatment and is usually performed at the time of diagnosis.

Common post-surgical complications include:

Conduction abnormalities

right bundle branch block (RBBB): 80-90% of cases

bifascicular block: 15% of cases
premature ventricular contractions: ~50% of cases
sustained ventricular tachycardias: ~5% of cases
atrial arrhythmias: common

Valvular dysfunction

tricuspid regurgitation
pulmonary regurgitation
Prognosis is largely dependent on how soon the defect is diagnosed and corrected, with the best
outcome seen in patients repaired before the age of 5. Overall there is a 90-95% survival rate at 10 years
of age; however, residual right ventricular dysfunction is common. Up to 10% of patients require re-
operation within 20 years.

Plain radiograph findings:

ASD

Can be normal in early stages +/- when the ASD is small

Signs of increased pulmonary flow (pulmonary plethora or shunt vascularity)

enlarged pulmonary vessels

upper zone vascular prominence
vessels visible to the periphery of the film
eventual signs of pulmonary arterial hypertension

Chamber enlargement

right atrium
right ventricle
note: left atrium is normal in size unlike VSD or PDA
note: aortic arch is small to normal

VSD

The chest radiograph can be normal with a small VSD.

Larger VSDs may show cardiomegaly (particularly left atrial enlargement although the right and
left ventricle can also be enlarged).
A large VSD may also show features of pulmonary arterial hypertension, pulmonary edema,
pleural effusion and increased pulmonary vascular markings.

PDA

Chest radiographic features may vary depending on whether it is isolated or associated with
other cardiac anomalies and with the direction of shunt flow (right to left or left to right).
Can have cardiomegaly (predominantly left atrial and left ventricular enlargement if not
complicated).
Obscuration of the aortopulmonary window and features of pulmonary oedema may be
evident.

INTRAUTERINE GROWTH RESTRICTION (IUGR)

Estimated fetal weight (EFW) at one point in time during pregnancy being at or below the 10th
percentile for gestational age (Some authors: under the 5th percentile or fall below two standard
deviations)
Some authors consider this definition synonymous with the term small for gestational age (SGA).

An IUGR can be broadly divided into two main types:

Type I: Symmetrical intrauterine growth restriction

Type II: Asymmetrical intrauterine growth restriction
Some authors also enlist a 3rd type termed: femur sparing intrauterine growth restriction.

Causes:

IUGR can result from a vast number of causes:

Maternal conditions

maternal narcotic/alcohol use/smoking

maternal diabetes: when severe maternal diabetes, there can be a paradoxical IUGR as opposed
to fetal macrosomia
maternal malnutrition/starvation
maternal vascular conditions
certain medications

Placental insufficiency: commonest cause overall

abnormal uteroplacental circulation

abnormal fetoplacental circulation

Other placental causes

increased incidence with a single umbilical artery

Fetal conditions

Multifetal pregnancy
Intrauterine infections
chromosomal anomalies
trisomy 13
trisomy 18
triploidy: IUGR is of early onset
Down syndrome: not a dominant feature
chromosome 4p deletion syndrome
Other Syndromic anomalies
Neu-Laxova syndrome
Pena Shokeir syndrome
Seckel syndrome
Smith-Lemli-Opitz syndrome
In utero substance exposure e.g. fetal hydantoin syndrome

Antenatal ultrasound
Sonographic parameters include:

Non-Doppler features

presence of oligohydramnios without ruptured membranes

increased head circumference (HC) to abdominal circumference (AC) ratio (in asymmetrical
type)
decreased total intrauterine volumes
advanced placental grade

Doppler features: will require a chart to calculate absolute values

Umbilical artery Doppler assessment

Increased S/D ratio(s)

Increased resistive index (RI)

Umbilical venous Doppler assessment

presence of pulsatility

Uterine arterial Doppler assessment

presence of notching in mid to late pregnancy

increased S/D ratio(s)

Umbilical arterial (UA) Doppler assessment

Used in surveillance of fetal well-being in the third trimester of pregnancy. Abnormal umbilical
artery Doppler is a marker of uteroplacental insufficiency and consequent intrauterine growth
restriction (IUGR) or suspected pre-eclampsia.
Umbilical artery Doppler assessment has been shown to reduce perinatal mortality and
morbidity in high risk obstetric situations.
As a general rule, a degree of caution should be exercised with the routine use of Doppler in
pregnancy, due to the concerns related to heating/thermal effects from the high intensities of
Doppler ultrasound.

Doppler ultrasound

The Doppler indices measured at the fetal end, the free loop and the placental end of the
umbilical cord are different with the impedance highest at the fetal end.
The changes in the indices are likely to be seen at the fetal end first.
Ideally the measurements should be made in the free cord. However for consistency of
recording in cases being followed up, a fixed site would be more appropriate, i.e. fetal end,
placental end or intra-abdominal portion.

Waveform
 The umbilical arterial waveform usually has a "saw tooth" pattern with flow always in the
forward direction. An abnormal waveform shows absent or reversed diastolic flow.
Before the 15th week, absent diastolic flow may be a normal finding.
The 95% confidence interval limit slowly decreases for both the resistive index (RI) and
pulsatility index (PI) through the course of gestation due to progressive maturation of the
placenta and increase in the number of tertiary stem villi.

Parameters

The commonly used parameters are:

Umbilical arterial S/D ratio (SDR): systolic velocity / diastolic velocity

Pulsatility index (PI) (Gosling index): (PSV - EDV) / TAV
Resistive index (RI) (Pourcelot index): (PSV - EDV) / PSV

PSV: peak systolic velocity, EDV: end diastolic velocity, TAV: time averaged velocity

The Doppler indices have been found to decline gradually with gestational age:

S/D ratio mean value decreases from 3.560 to 2.511

RI mean value decreases from 0.756 to 0.609
PI main value decreases from 1.270 to 0.967

Classification of severity

In growth-retarded fetuses and fetuses developing intrauterine distress, the umbilical artery blood
velocity waveform usually changes in a progressive manner as below

1. Reduction in end diastolic flow: increasing RI values, PI values and S/D ratio
2. Absent end diastolic flow (AEDF): RI = 1
3. Reversal of end diastolic flow (REDF): Reversal of umbilical artery end-diastolic flow (REDF) or
velocity is often an ominous finding if detected after 16 weeks. It is classified as Class III in
severity in abnormal umbilical arterial Dopplers. The feature is seen as a result of a significant
increase in resistance to blood flow within the placenta and often represents a "tip of the
iceberg" where there is a much larger underlying pathology. In a normal situation, umbilical
arterial flow should always be in the forward direction in both systole and diastole. However,
during the first 16 weeks, a reversal in end diastolic flow can be a normal finding due to the low
resistance arcuate arteries and intervillous spaces not yet being formed.

Absent end-diastolic flow (AEDF):

Can be normal in early pregnancy (up to 16 weeks)

In mid to late pregnancy it usually occurs as a result of placental insufficiency
Flow in the umbilical artery(ies) should be in the forward direction in normal circumstances. If
placental resistance increases, the diastolic flow may reduce, later becoming absent and finally
reverses
The umbilical arterial velocity is seen reducing to zero at end diastole
The impedance is found to be highest at the fetal end of the umbilical cord and therefore the
absence of end diastolic flow is seen first in this region
Umbilical venous flow

Physiological situation comprises of a monophasic non-pulsatile flow pattern with a mean velocity of 10-
15 cm/s. The presence of pulsatility implies a pathological state unless in the following situations:

Early in pregnancy: up to ~13 weeks gestation

the presence of pulsatility may be higher in chromosomally abnormal fetuses even in early
pregnancy

When confounded by other movement variables such as

fetal breathing movement

fetal hiccups

Pulsations of the umbilical venous system, especially double pulsations have been associated with
increase in the perinatal mortality when associated with the absent and reversed end-diastolic flow
velocity in the umbilical artery.

Uteroplacental blood flow assessment

Important part of fetal well-being assessment and evaluates Doppler flow in the uterine arteries and
rarely the ovarian arteries.

Pathology

In a non-gravid state and at the very start of pregnancy the flow in the uterine artery is of high pulsatility
with a high systolic flow and low diastolic flow. A physiological early diastolic notch may be present.

Resistance to blood flow gradually drops during gestation as a greater trophoblastic invasion of the
myometrium takes place. An abnormally high resistance can persist in pre-eclampsia and IUGR. If
resistance is low, it has an excellent negative predictive value with a less than 1% chance of developing
either pre-eclampsia or having IUGR . A high resistance often equates to a 70% chance of pre-eclampsia
and 30% chance of IUGR.

Ultrasound

The parameters used in the assessment of uteroplacental blood flow include:

RI = resistive index
PI = pulsatility index
Presence of persistent diastolic notching

Resistive index (RI)

This is calculated by the following equation:

RI = (PSV-EDV) / PSV = (peak systolic velocity - end diastolic velocity) / peak systolic velocity
 normal (low resistance) RI < 0.55
high resistance: bilateral notches RI > 0.55, unilateral notches RI > 0.65

Pulsatility index (PI)

This is calculated by the following equation:

PI = (peak systolic velocity - end diastolic velocity) / time averaged velocity = (PSV - EDV) / TAV

Abnormal patterns include

Persistence of a high resistance flows throughout pregnancy

Persistence of notching throughout pregnancy
Reversal of diastolic flow throughout pregnancy: severe state

Fetal middle cerebral arterial (MCA) Doppler assessment

Important part of assessing fetal cardiovascular distress, fetal anaemia or fetal hypoxia. In the
appropriate situation it is a very useful adjunct to umbilical artery Doppler assessment. It is also used in
the additional work up of:

intra-uterine growth restriction (IUGR)

twin to twin transfusion syndrome (TTTS)
twin anaemia polycythaemia sequence (TAPS)

Doppler ultrasound

For an accurate measurement, the fetal head should be in the transverse plane.

An axial section of the brain, including the thalami and the sphenoid bone wings, should be obtained
and magnified.

The MCA vessels are often found with colour or power Doppler ultrasound overlying the anterior wing
of the sphenoid bone near the base of the skull.

The reading should be obtained close to its origin in the internal carotid artery as the systolic velocity
decreases with distance from the point of origin of this vessel. An angle of insonation of <15 should be
used; typically, an angle that approximates 0 can be achieved by moving the transducer on the
maternal abdomen.

Parameters used include:

Fetal MCA pulsatility index (PI): The fetal MCA PI normally has a high value. The mean value
(normal reference range) slowly decreases through gestation from around 28 weeks onwards. A
low PI reflects redistribution of cardiac output to the brain due to the fetal head sparing theory.
Fetal MCA peak systolic velocity (PSV): the highest velocity should be recorded, reliable between
18-35 weeks, increased PSV can indicate moderate-to-severe anemia in non-hydrops fetuses
 fetal MCA systolic/diastolic (S/D) ratio: Normal fetal MCA S/D ratio should always be higher than
the umbilical arterial S/D ratio, During pregnancy the middle cerebral (and other intracranial)
arteries demonstrate high resistance waveforms, i.e. high systolic velocity and low/absent
diastolic velocity. The fetal MCA S/D ratio value will decrease as the pregnancy progresses.
Cerebroplacental ratio (CPR): ratio of pulsatility index of MCA and umbilical artery

Interpretation

In the normal situation the fetal MCA has a high resistance flow which means there is minimal
antegrade flow in fetal diastole
In pathological states this can turn into a low resistance flow mainly as a result of the fetal head
sparing theory
Paradoxically in some situations such as with severe cerebral oedema, the flow can revert back
to a high resistance pattern when the pathology has not yet resolved - this is a very poor
prognostic sign
Cerebroplacental ratio: >1:1 is normal and <1:1 is abnormal

Fetal head sparing theory

Underpins asymmetrical intra-uterine growth restriction, where the difference between normal
head circumference and decreased abdominal circumference is attributed to the fetus's ability
to preferentially supply the cerebral, coronary, adrenal and splenic circulations.
In a situation of chronic fetal hypoxaemia, the fetus redistributes its cardiac output to maximize
the oxygen supply to brain by vasodilation of the cerebral arteries thereby causing a decrease in
the left ventricular afterload.

FETAL DUCTUS VENOSUS FLOW ASSESSMENT

Can be sonographically assessed in a number of situations in fetal ultrasound:

First-trimester screening for aneuploidic anomalies

Second-trimester scanning when there are concerns regarding
intrauterine growth restriction (IUGR)
fetal cardiac compromise

Of all the pre-cardial veins, the ductus venosus allows the most accurate interpretation of fetal cardiac
function as well as myocardial haemodynamics.

Ultrasound

Technique

the fetus should be as still as possible

the probe is ideally focused so sampling is done where the umbilical vein joins the ductus
venosus
 a right ventral mid-sagittal view of the fetal trunk should be obtained and colour flow mapping
used to demonstrate the umbilical vein, ductus venosus and fetal heart
the probe is ideally angled to allow a mid sagittal plane or a transverse oblique plane through
the fetal abdomen
the image should be magnified enough for the fetal thorax and abdomen to occupy the whole
screen
do not contaminate the ductus venosus flow with the flow from the fetal inferior vena cava, for
this the Doppler sample should be small (0.5-1 mm)
the insonation angle should be 30
the sweep speed should be high (23 cm/s) so that the waveforms are spread allowing better
assessment of the A-wave
On Doppler ultrasound, the flow in the ductus venosus has a characteristic triphasic waveform
where in a normal physiological situation flow should always be in the forward direction 7 (i.e.
towards the fetal heart).

This triphasic waveform comprises of:

S wave: corresponds to fetal ventricular systolic contraction and is the highest peak
D wave: corresponds to fetal early ventricular diastole and is the second highest peak
A wave: corresponds to fetal atrial contraction and is the lowest point in the wave form albeit
still being in the forward direction

Abnormal waveforms include:

Reduced flow in ductus venosus A wave

Absent flow in ductus venosus A wave
Reversal of flow in ductus venosus A wave
Abnormal indices: abnormal pulsatility index (PI), abnormal S wave to A wave ratio (S:A),
abnormal peak velocity index

PULMONARY EMBOLISM (PE)

Rrefers to embolic occlusion of the pulmonary arterial system

The majority of cases result from thrombotic occlusion, and therefore the condition are frequently
termed pulmonary thrombo-embolism which is what this article mainly covers.

Other embolic sources include:

air embolism
carbon dioxide embolism
other gas embolism (e.g. nitrogen, helium)
fat embolism
tumour embolism: comprised of tumour thrombus
 hydatid embolism
talc embolism
iodinated oil embolism
metallic: mercury embolism, barium embolism
amniotic fluid embolism
cement embolism: comprised of polymethyl methacrylate (PMMA)
catheter embolism
septic embolism

Clinical features:

Non-specific
Dyspnoea, chest pain, and haemoptysis have been described as a classic triad in pulmonary
embolism. The ECG may show an S1Q3T3 pattern.
Patients may have deep vein thrombosis (usually from the lower limbs) which are the most
common source of the PE.

Pre-test probability scores are intended to replace empirical assessment of patients with suspected
pulmonary embolism:

Wells criteria
Geneva score
PERC rule

Wells criteria

Risk stratification score and clinical decision rule to estimate the probability for acute pulmonary
embolism (PE) in patients in which history and examination suggests acute PE is a diagnostic possibility.
It provides a pre-test probability which, if deemed unlikely, can then be used in conjunction with a
negative D-dimer to rule out PE avoiding imaging.

Criteria

clinical signs and symptoms of DVT = 3

an alternative diagnosis is less likely than PE = 3
heart rate more than 100 = 1.5
immobilisation for 3 or more consecutive days or surgery in the previous 4 weeks = 1.5
previous objectively diagnosed PE or DVT = 1.5
haemoptysis = 1
malignancy (on treatment, treatment in last 6 months or palliative) = 1

Score interpretation

Can be applied in either three tier or two tier models:

Three tier:
 0-1: low risk
2-6: moderate risk
>6: high risk

Two tier:

4: unlikely
4.5: likely

In the three tier model:

low risk patients: pulmonary embolism rule-out criteria (PERC) can be considered as well as D-
dimer
moderate risk: consider D-dimer or CT pulmonary angiography
high risk: D-dimer not recommended

In the two tier model:

unlikely: consider D-dimer

likely: consider CTPA

Risk factors

Primary hypercoagulable states: protein C deficiency, protein S deficiency, antithrombin III

deficiency, lupus anticoagulant
Recent surgery
Pregnancy
Prolonged bed rest/immobility
Malignancy
Oral contraceptive use
Known or previous DVT
Presence of certain venous aneurysms: e.g. popliteal venous aneurysm

The right ventricular failure due to pressure overload is considered the primary cause of death in severe
PE.

Markers

D-dimer (ELISA) is commonly used as a screening test in patients with a low and moderate probability
clinical assessment, on these patients:

normal D-dimer has almost 100% negative predictive value (virtually excludes PE): no further
testing is required
raised D-dimer is seen with PE but has many other causes and is, therefore, non-specific: it
indicates the need for further testing if pulmonary embolism is suspected

On patients with a high probability clinical assessment, a D-dimer test is not helpful because a negative
D-dimer result does not exclude pulmonary embolism in more than 15%. Patients are treated with
anticoagulants while awaiting the outcome of diagnostic tests.

Plain radiograph
A chest x-ray is neither sensitive nor specific for a pulmonary embolism. It is used to assess for
differential diagnostic possibilities such as pneumonia and pneumothorax rather than for the direct
diagnosis of PE.

Fleischner sign: enlarged pulmonary artery (20%) [Fleischner sign is a prominent central artery
that can be caused either by pulmonary hypertension that develops or by distension of the
vessel by a large pulmonary embolus. It can be seen on chest radiographs and CT pulmonary
angiography.]
Hampton hump: peripheral wedge of airspace opacity and implies lung infarction (20%)
[Hampton hump refers to a dome-shaped, pleural-based opacification in the lung most
commonly due to pulmonary embolism and lung infarction (it can also result from other causes
of pulmonary infarction (e.g. vascular occlusion due to angioinvasive aspergillosis). While a
pulmonary artery embolism is expected to result in a wedge-shaped infarction, the expected
apex of this infarction may be spared because of bronchial arterial circulation in this part,
leading to the characteristic rounded appearance of a Hampton hump.]
Westermark sign: regional oligaemia and highest positive predictive value (10%) [focal
peripheral hyperlucency secondary to oligaemia, central pulmonary vessels may also be dilated]
Pleural effusion (35%)
Knuckle sign: Knuckle sign refers to the abrupt tapering or cutoff of a pulmonary artery
secondary to embolus. It is better visualized on CT pulmonary angiography scan than chest x-
ray. This is an important ancillary finding in pulmonary embolism (PE), and often associated with
the Fleischner sign of dilated central pulmonary arteries.
Palla sign: enlarged right descending pulmonary artery
Chang sign: dilated right descending pulmonary artery with sudden cut-off

CT

Acute pulmonary emboli

CT pulmonary angiography (CTPA) will show filling defects within the pulmonary vasculature
with acute pulmonary emboli. When observed in the axial plane this has been described as the
polo mint sign. The central filling defect from the thrombus is surrounded by a thin rim of
contrast, appearing like the popular sweet, the polo mint.
Emboli may be occlusive or non-occlusive, the latter as seen with a thin stream of contrast
adjacent to the embolus. Typically the embolus makes an acute angle with the vessel, in
contrast to chronic emboli. The affected vessel may also enlarge.
Acute pulmonary thromboemboli can rarely be detected on non-contrast chest CT as
intraluminal hyperdensities.

Chronic pulmonary emboli

In contrast to acute pulmonary embolism, chronic thromboemboli are often complete

occlusions or non-occlusive filling defects in the periphery of the affected vessel which form
obtuse angles with the vessel wall. The thrombus may be calcified.

Features noted with chronic pulmonary emboli include:

 webs or bands, intimal irregularities
abrupt narrowing or complete obstruction of the pulmonary arteries
pouching defects which are defined as chronic thromboembolism organised in a concave
shape that points toward the vessel lumen

Indirect signs include:

mosaic perfusion
vascular calcification
bronchial or systemic collateralisation

Ultrasound

Cardiac echo has an important bedside role in suspected major PE looking for right ventricular
wall hypokinesis (McConnell sign), right ventricular dilatation and pulmonary artery
hypertension.
Lung ultrasound in the diagnosis of PE is controversial with a poor evidence base.

MRI

It is difficult to obtain technically adequate images for pulmonary embolism patients using MRI.
Magnetic resonance pulmonary angiography should be considered only at centres that routinely
perform it well and only for patients for whom standard tests are contraindicated.
Technically-adequate magnetic resonance angiography has a sensitivity of 78% and a specificity
of 99%.

Nuclear medicine

A ventilation/perfusion (V/Q) scan will show ventilation-perfusion mismatches.

A high probability scan is defined as showing two or more unmatched segmental perfusion
defects according to the PIOPED criteria.

Complications

1. Acute emboli: pulseless electrical activity (PEA) in the context of a large obstructing saddle embolus

2. Acute or chronic emboli: right ventricular dysfunction

CT features suggestive of right ventricular dysfunction include:

abnormal position of the interventricular septum

inferior vena caval contrast reflux
RVD (right ventricular diameter): LVD (left ventricular diameter) ratio >1 on reconstructed four
chamber views, RVD: LVD ratio >1 on standard axial views is not considered to be a good
predictor of right ventricular dysfunction

3. Subacute-to-chronic emboli

pulmonary infarction
pulmonary hypertension
pulmonary arterial sclerosis
 4. Chronic emboli

cor pulmonale

Differential diagnosis

Pulmonary artery sarcoma

Pulmonary artery vasculitis e.g. Takayasu arteritis
Misidentification of pulmonary veins for arteries
Arterial bifurcations (a.k.a branch-points) can mimic PE but usually easily identified on
multiplanar assessment
Chronic emboli may be mistaken for acute emboli, or vice-versa

Artefact may cause pseudofilling defects and can be caused by

Breathing motion
Beam hardening
hyperconcentrated contrast in the superior vena cava
medical devices e.g. catheters, orthopaedic prostheses
patient's arms in a down position
Patient movement
Transient contrast bolus interruption- due to Valsalva or patent foramen ovale, where causes
unopacified blood to enter the right ventricle and pulmonary arteries. Scanning in end
expiration can reduce or eliminate this artefact.

AORTIC DISSECTION
Aortic dissection is the most common form of the acute aortic syndromes and a type of arterial
dissection. It occurs when blood enters the medial layer of the aortic wall through a tear or penetrating
ulcer in the intima and tracks along the media, forming a second blood-filled channel within the wall.

Epidemiology
The majority of aortic dissections are seen in elderly hypertensive patients. In a very small minority, an
underlying connective tissue disorder may be present. Other conditions / predisposing factors may also
be encountered, in which case they will be reflected in the demographics. Examples include:

structural aortic abnormalities

o bicuspid aortic valve
o aortic coarctation
o abnormal connective tissues
Marfan syndrome
Ehlers-Danlos syndrome
Turner syndrome
pregnancy
intra-aortic balloon pumps
 Clinical presentation
Aortic dissection is arbitrarily divided into:

1. acute: within 14 days of first symptom onset

2. chronic: after 14 days
Patients are often hypertensive (although they may be normotensive or hypotensive) and present with
anterior or posterior chest pain and a tearing sensation in the chest.

Depending on the extent of dissection and occlusion of aortic branches, end organ ischaemia may also
be present (seen in up to 27% of cases), including:

abdominal organ ischaemia

limb ischaemia
ischaemic or embolic stroke
paraplegia: involvement of the artery of Adamkiewicz
In some cases of aortic rupture, the involvement of coronary arteries may result in collapse and death.
Symptoms of cardiac tamponade (Beck's triad) may also be encountered.

Pathology
The normal lumen lined by intima is called the true lumen and the blood-filled channel in the media is
called the false lumen.

In most cases the vessel wall is abnormal.

hypertension (pathogenesis: medial degeneration)

inherited connective tissue disorders (pathogenesis: medial degeneration)
o Marfan syndrome
o Ehlers-Danlos syndrome
atherosclerosis (pathogenesis: penetrating ulcer)
vasculitis (pathogenesis: inflammation)
pregnancy (pathogenesis: unknown)
iatrogenic: aortic catheterisation, intra-aortic balloon pump

Radiographic features
Imaging is essential in delineating the morphology and extent of the dissection as well as allowing for
classification (which dictates management). Two classification systems are in common usage, both of
which divide dissections according to the involvement of the ascending aorta:

1. Stanford classification
2. DeBakey classification
In recent years, the Stanford classification has gained favour with cardiothoracic surgeons.
Approximately 60% of dissections involve the ascending aorta (Stanford A / DeBakey I and II).

Stanford classification
 Used to separate aortic dissections into those that need surgical repair, and those that usually require
only medical management. The Stanford classification divides dissections by the most proximal
involvement:

type A: A affects ascending aorta and arch

o accounts for ~60% of aortic dissections
o surgical management
o may result in:
coronary artery occlusion
aortic incompetence
rupture into pericardial sac with resulting cardiac tamponade
type B: B begins beyond brachiocephalic vessels
o accounts for ~40% of aortic dissections
o dissection commences distal to the left subclavian artery
o medical management with blood pressure control

DeBakey classification

Used to separate aortic dissections into those that need surgical repair, and those that usually require
only medical management.

Classification
The DeBakey classification divides dissections into:

type I: involves ascending and descending aorta (= Stanford A)

type II: involves ascending aorta only (= Stanford A)
type III: involves descending aorta only, commencing after the origin of the left subclavian artery (=
Stanford B)

Plain radiograph
Chest radiography may be normal or demonstrate a number of suggestive findings, including:

widened mediastinum (more than 8 cm at the level of the aortic knob on portable anteroposterior
chest radiographs)
double aortic contour
irregular aortic contour
inward displacement of atherosclerotic calcification (more than 1 cm from the aortic margin) 9,11

CT / CT angiography
 CT, especially with arterial contrast enhancement (CTA) is the investigation of choice, able not only to
diagnose and classify the dissection but also to evaluate for distal complications. It has reported
sensitivity and specificity of nearly 100% .

Non-contrast CT may demonstrate only subtle findings; however, a high-density mural haematoma is
often visible. Displacement of atherosclerotic calcification into the lumen is also a frequently identified
finding.

Dissections involving the aortic root should ideally be assessed with ECG-gated CTA which nearly totally
eliminates pulsation artefact. Pulsation artefact can mimic dissection, is very common and seen in up to
92% of non-gated CTA studies .

Contrast-enhanced CT (preferably CTA) gives excellent detail. Findings include:

intimal flap
double lumen
dilatation of the aorta
complications (see below)
an atypical variant that may be seen is an aortic intramural haematoma
Mercedes-Benz sign in the case of a "triple-barreled" dissection
windsock sign
An essential part of the assessment of aortic dissection is identifying the true lumen, as the placement
of an endoluminal stent graft in the false lumen can have dire consequences. Distinguishing between
the two is often straighforward, but in some instances, no clear continuation of one lumen with normal
artery can be identified. In such instances, a number of features are helpful 3:

true lumen
o often compressed by the false lumen and the smaller of the two
o outer wall calcifications (helpful in acute dissections)
o origin of coeliac trunk, SMA and right renal artery usually from true lumen
false lumen
o often larger lumen size due to higher false luminal pressures
o beak sign
o cobweb sign (as slender linear areas of low attenuation specific to the false lumen due to residual
ribbons of media that have incompletely sheared away during the dissection process) 3
o often of lower contrast density due to delayed opacification
o may be thrombosed and seen as mural low density only (more common in chronic dissections)
o origin of left renal artery usually from false lumen
o surrounds true lumen in Stanford type A
Chronic dissection flaps are often thicker and straighter than those seen in acute dissections 3.

Transoesophageal echocardiography
 Transoesophageal echocardiography (TOE) has very high sensitivity and specificity for assessment of
acute aortic dissection, but due to limited access and its invasive nature, it has largely been replaced by
CTA (or MRA in some instances) 5.

MRI
Although in general MRA has been reserved for follow-up examinations, rapid non-contrast imaging
techniques (e.g. true FISP) may see MRI having a larger role to play in the acute diagnosis, particularly in
patients with impaired renal function 4. It has similar sensitivity and specificity to CTA and TOE 5 but
suffers from limited availability and the difficulties inherent in performing MRI on acutely unwell
patients.

DSA - angiography
Conventional digital subtraction angiography has historically been the gold standard investigation. CTA
has now replaced it as the first line investigation, not only due to it being non-invasive but also on
account of better delineation of the poorly opacifying false lumen, intramural haematoma and end-
organ ischaemia.

Angiography still is required for endoluminal repair.

Risks of angiography include general risks of angiography plus the risk of catheterising the false lumen
and causing aortic rupture.

Treatment and prognosis

aggressive blood pressure control with beta blockers as they reduce both blood pressure and also
heart rate hence reduce extra pressure on the aortic wall
immediate surgical repair (for type A dissection or complicated type B dissection)

Complications
Complications of all types of aortic dissection include:

dissection and occlusion of branch vessels

o abdominal organ ischaemia
o limb ischaemia
o ischaemic stroke
o paraplegia: involvement of artery of Adamkiewicz
distal thromboembolism
aneurysmal dilatation: this is an indication for endovascular or surgical intervention
6

aortic rupture
A type A dissection may also result in:

coronary artery occlusion

aortic incompetence
rupture into pericardial sac with resulting cardiac tamponade
 Although the combination of blood pressure control and surgical intervention has significantly lowered
in hospital mortality, it remains significant, at 10-35%. Over the 10 years following diagnosis another 15-
30% of patients require surgery for life-threatening complications 5.

Differential diagnosis
The differential on chest x-ray is that of a dilated thoracic aorta.

On CT, a number of entities that can mimic a dissection should be considered :

pseudodissection due to aortic pulsation motion artefact (typically left anterior and right posterior
aspects of the ascending aorta)
pseudodissection due to contrast streaks
mural thrombus
intramural haematoma: really an atypical type of aortic dissection and part of the acute aortic
syndrome
penetrating atherosclerotic ulcer which is part of the acute aortic syndrome
adjacent atelectasis

RENAL ARTERY STENOSIS

Renal artery stenosis (RAS) refers to a narrowing of a renal artery. When the process occurs slowly, it
leads to secondary hypertension. Acute renal artery stenosis does not lead to hypersecretion of renin.

Pathology
When the stenosis occurs slowly, collateral vessels form and supply the kidney. The kidney wrongly
senses the reduced flow as low blood pressure (via juxtaglomerular apparatus) and releases a large
amount of renin, that converts angiotensinogen to angiotensin I. Angiotensin I is then converted to
angiotensin II with the help of angiotensin converting enzyme (ACE) in lungs. Angiotensin II is
responsible for vasoconstriction and release of aldosterone which causes sodium and water retention,
thus resulting in secondary hypertension.

Renal artery stenosis may be caused by several pathological processes:

atherosclerosis ~75% involves proximal renal artery

fibromuscular dysplasia ~20% involves distal renal artery
vasculitides (especially polyarteritis nodosa, Takayasu arteritis, radiation)
neurofibromatosis type 1
abdominal aortic coarctation
aortic dissection
segmental arterial mediolysis
Occurrence is not uncommon following a renal transplant.

Radiographic features
 Ultrasound
Ultrasound although most freely available, cheap and often used first line, is relatively operator
dependent and time consuming.

increased renal arterial resistive index (RI): a cut-off value of 0.7 may be a good approximation in
clinical practice
RI difference between kidneys > 0.05 - 0.07
increased peak systolic velocity (PSV): some advocate 180cm/s
increased renal-interlobar ratio (RIR): some advocate values greater than 5
increased renal aortic ratio (RAR) i.e. PSVrenal/PSVaorta: usually taken as >3.5 although some
advocate >3
turbulent flow in post stenotic area
pulsus parvus et tardus waveform (slow-rising) due to stenosis
intraparenchymal resitive indices > 0.8
intraparenchymal acceleration imte > 0.07 s

Renal arterial resistive index (RI)

Sonographic index to assess for renal arterial disease.

It is measured as

RI = (peak systolic velocity - end diastolic velocity ) / peak systolic velocity

the normal value is 0.60

with 0.70 being around the upper limits of normal

Technique
Measured at arcuate arteries (at the corticomedullary junction) or interlobar arteries (adjacent to
medullary pyramids)

Differential diagnosis

Reasons for elevated values

renal artery stenosis (if measured upstream from the stenosis)
ureteric obstruction
extreme hypotension
very young children
perinephric fluid collection
medical renal disease ref required

Reasons for elevated values in a transplant kidney

acute tubular necrosis (ATN)
 acute or chronic transplant rejection
renal vein thrombosis
drug toxicity
renal artery stenosis (if measured upstream from the stenosis)
ureteric obstruction
perinephric fluid collection
There is thought to be little correlation between the RI and the quantitative extent of renal dysfunction
(measured by serum creatinine values) 2.

Tardus parvus

Refers to a particular pattern of Doppler ultrasound spectral waveform. It commonly occurs

downstream from significant arterial stenosis, and in particular is useful in assessing for renal artery
stenosis.

Radiographic features
tardus: prolonged systolic acceleration (i.e. slow upstroke)
parvus: small systolic amplitude and rounding of systolic peak

CT angiography
The three-dimensional reconstruction of the renal vascular tree provides a reliable method of visualizing
the whole vascular tree. Images are acquired with thin collimation and bolus tracking on the abdominal
aorta. Sensitivity and specificity varying between 90 to 99% have been reported 7. Both the raw data
and 3D reconstructions should be viewed. Additionally, supernumerary arteries may be identified.

MR angiography
Different imaging methods can be used:

time of flight (TOF): whereby the high velocity of the blood jet at the level of stenosis appears as a loss
of signal (black)
phase contrast technique
contrast enhanced MRA: gadolinium is used as a contrast agent
Three-dimensional reconstruction technique offers a sensitivity and specificity values around 90 to
100% 7. In some cases, renal impairment does not permit the use of contrast, in which case TOF imaging
is beneficial.

Nuclear medicine

ACE inhibitor scintigraphy

affected kidney with renovascular hypertension shows impaired function due to ACE inhibition; based
on this principle scintigraphy has been very much useful for diagnosis of renal artery stenosis
 it is performed by IV administration of enalapril maleate after 15 minutes
sequential images and scintigraphic curves are drawn for renal cortex and pelvis; renal uptake is
measured for every 1-2 min interval after giving IV injection
typical isotopes used are Tc-99m MAG3, Tc-99m DTPA or I-123 0-iodohippurate 6
scintigram will be interpreted as either low, intermediate or high probability

VARICOSE VEINS
Varicose veins are dilated tortuous superficially located venous channels that accompany the superficial
veins of the upper or lower limbs.

Epidemiology
Varicose veins are more common in women than men and are more common in the lower limb than in
the upper limb 5. Risk factors include:

pregnancy
older age
female gender
prolonged standing

Pathology
Incompetent saphenofemoral junction, which itself results from saphenofemoral valve insufficiency, is a
well-known cause. It leads to regurgitation of blood during expiration and consequently raises the
venous pressure in the great saphenous and other superficial veins.

Incompetent perforators are another causative factor when destruction of the valves inside the
perforators allow the blood to move from the deep to superficial system (reverse direction) and
consequently increases the superficial venous pressure.

Radiographic features

Plain radiograph
Varicose veins may appear as dilated tortuous subcutaneous opacities.

Ultrasound
Doppler ultrasound can detect the presence and distribution of the subcutaneous varicosities.
Moreover, it is the method of choice to assess the state of the saphenofemoral junction. Reflux of blood
flow at the junction during Valsalva maneuver indicates incompetence. Reflux that lasts for more than 1
second is significant and usually require surgical intervention. Reflux that lasts 0.5-1 second is
insignificant and usually requires conservative management. Incompetent perforators are assessed in
the standing position.

CHRONIC VENOUS INSUFFICIENCY

 Chronic venous insufficiency (CVI) occurs due to inadequate functioning of venous wall and/or valves in
lower limb veins resulting in excessive pooling of blood.

Pathology
The condition results from venous hypertension which in turn is usually caused by reflux in the
superficial venous compartment. Less common causes include:

deep venous compression

post-thrombotic stenosis or occlusion
deep venous reflux
venous hypertension caused by vascular malformations, arteriovenous fistulae, and neuromuscular
disorders (rare)

Radiographic features

Plain radiograph
Findings are non-specific but most commonly are seen in the leg 5,6:

solid undulating periosteal reaction, often symmetrical

dystrophic soft tissue calcification
varicose vein phleboliths
soft tissue swelling from subcutaneous oedema

Ultrasound

Venous Doppler ultrasound

Considered the primary imaging modality of choice. Typically the great saphenous vein and the short
saphenous vein and their primary tributaries are assessed.

The presence of reflux is determined by the direction of flow because any significant flow toward the
feet is suggestive of reflux. The duration of reflux is known as the "reflux time" (replacing the commonly
used "valve closure time").

a reflux time of > 0.5 (or 1.0 according to some publications) second has been used to suggest the
diagnosis the presence of reflux, although a more refined definition with a variable cutoff based on
location has been suggested
the longer the duration of reflux or the greater the reflux time implies more severe disease
Venous duplex imaging may provide information about local valve function to construct an anatomic
map of disease in terms of the systems and levels of involvement.

The presence and location of perforators are also documented. The patient should be able to stand for
this procedure.
 DEEP VEIN THROMBOSIS
The term deep vein thrombosis (DVT) is practically a synonym for those that occur in the lower limbs.
However, it can also be used for those that occur in the upper limbs and neck veins. Other types of
venous thrombosis, such as intra-abdominal and intracranial, are discussed in specific sections.

Epidemiology
1.6 new cases per 1000 per year
2.5-5% of population is affected
>50% have long terms symptoms of post thrombotic syndrome
6% of DVT patients report eventual venous ulcers (0.1% general population)

Risk factors
Age (relative risk increase ~2 per 10 year increase)
Surgery (orthopaedic patients at highest risk: hip 48%, knee 61%)
Trauma
History of venous thromboembolism (2-9% increase)
Primary hypercoagulable states
o Protein A, C, and S deficiency 10x increased risk
o Factor V Leiden (heterozygous 8x increased risk, homozygous 80x)
Oestrogen replacement (2-4x increased risk)
Immobilisation (2x increased risk)
Pregnancy (0.075% of pregnancies)

Radiographic features

Ultrasound
The majority of deep venous thromboses occur in the lower extremities and begin in the soleal veins of
the calf. Doppler compression sonography is the imaging modality of choice. Features include:

Non-compressible venous segment

Increased venous diameter: acute thrombus
Decreased venous diameter: chronic thrombus
Loss of phasic flow on Valsalva manoeuvre
Absent colour flow: if completely occlusive
Increased flow in superficial veins
Lack of flow augmentation of calf squeeze
Anechoic thrombus: acute thrombus
Echogenic thrombus: chronic thrombus

TAKAYASU ARTERITIS
 Takayasu arteritis (TA), also known as idiopathic medial aortopathy or pulseless disease, is a
granulomatous large vessel vasculitis that predominantly affects the aorta and its major branches. It
may also affect the pulmonary arteries. The exact cause is not well known but the pathology is thought
to be similar to giant cell arteritis.

Epidemiology
There is a strong female predominance (F: M ~ 9:1), an increased prevalence in Asian populations, and it
tends to affect younger patients (<50 years of age). The typical age of onset is at around 15-30 years of
age.

Clinical presentation
It induces clinically varied ischaemic symptoms due to stenotic lesions or thrombus formation. The exact
spectrum can be highly variable and dependent on the territory of vascular involvement.

Pathology
There is segmental and patch granulomatous inflammation of the aorta which results in stenosis,
thrombosis and aneurysm formation. Half of the patients present with an initial systemic illness whereas
the other 50% present with late-phase complications.

Two phases of the disease are classically described:

pre-pulseless phase: characterised by non-specific systemic symptoms

pulseless phase: presents with limb ischaemia or renovascular hypertension
The initial systemic illness may include symptoms of malaise, fever, night sweats, weight loss and
arthralgia. There is often anaemia with raised inflammatory markers. This phase gradually resolves with
the initiation of the chronic phase which is characterised by inflammatory and obliterative changes in
the aorta and its branches. There are often reduced or absent peripheral pulses, giving rise to its
alternative name of "pulseless disease".

Classification
It has been classified based on location 3:

type I: classic type involving solely the aortic arch branches: brachiocephalic trunk, carotid and
subclavian arteries
type II:
o IIa: involvement of the aorta solely at its ascending portion and/or at the aortic arch +/- branches of
the aortic arch
o IIb: involvement of the descending thoracic aorta +/- ascending or aortic arch + branches
type III: involvement of the thoracic and abdominal aorta distal to the arch and its major branches, e.g.
descending thoracic aorta + abdominal aorta +/- renal arteries
type IV: sole involvement of the abdominal aorta and/or the renal arteries
type V: generalised involvement of all aortic segments

Radiographic features
 Ultrasound
Findings include 5:

long, smooth, homogeneous and moderately echogenic circumferential thickening of the arterial wall
may be present; on transverse section, this finding is termed as the 'macaroni sign' and is highly
specific for Takayasu arteritis (in contrast, atherosclerotic plaque is non-homogeneous, often calcified
with irregular walls and generally affects a short segment)
vascular occlusion may be seen due to intimal thickening and/or secondary thrombus formation
flow velocities depend on the level of occlusion
aneurysms may be noted
there may be a loss of pulsatility of the vessel

CT/MRI
Findings include 2:

wall thickening: active acute phase

wall enhancement: active acute phase
aortic valve disease: stenosis, regurgitation
occlusion of major aortic branches
aneurysmal dilatation of the aorta or its branches
pseudoaneurysm formation
diffuse narrowing distally (i.e. descending and abdominal aorta): in the late phase
The pulmonary arteries are also commonly involved, with the most common appearance being
peripheral pruning.

Coronary findings
Described features on CTCA include 7:

stenosis of the coronary ostia: ~30%

non-ostial coronary arterial stenoses: ~35%
coronary arterial aneurysms: ~10%

Treatment and prognosis

Treatment is with systemic steroids and judicious use of angioplasty. Corticosteroids can be used for
initial treatment. Other medical options include methotrexate, cyclophosphamide, and cyclosporine.
Percutaneous angioplasty and bypass surgery should only be considered when there is no acute
inflammation.

Prognosis tends to be variable ranging from a rapidly progressive disease in some reaching a quiescent
stage in others.
 COARCTATION OF THE AORTA
Coarctation of the aorta (CoA) refers to a narrowing of the aortic lumen. It can be primarily divided into
two types:

1. infantile (pre-ductal) form: is characterised by diffuse hypoplasia or narrowing of the aorta from just
distal to the brachiocephalic artery to the level of ductus arteriosus, typically with a more discrete area
of constriction just proximal to the ductus but distal to the origin of the left subclavian artery.
Therefore, the blood supply to the descending aorta is via the patent ductus arteriosus.
2. adult (juxta-ductal, post-ductal or middle aortic) form: is characterised by a short segment abrupt
stenosis of the post-ductal aorta. It is due to thickening of the aortic media and typically occurs just
distal to the ligamentum arteriosum (a remnant of the ductus arteriosus).

Epidemiology
Coarctations account for between 5-8% of all congenital heart defects. They are more frequent in males,
M:F ratio of ~2-3:1.

Clinical presentation
Varies accordingly to the degree of stenosis and the associated abnormalities. Patients may be
asymptomatic in a setting of a non-severe stenosis.

Children and adults can present with angina pectoris and leg claudication. On clinical
examination, diminished femoral pulses and differential blood pressure between upper and lower
extremities may be noted.

Pathology

Associations
As is the case with many congenital abnormalities, coarctation of the aorta is associated with other
anomalies.

cardiac: coarctations are frequently associated with other congenital heart defects and conditions
which include
o bicuspid aortic valve: most common associated defect and seen in 75-80%
o ventricular septal defect (VSD)
o cyanotic congenital lesions including
truncus arteriosus
transposition of the great arteries (TGA), especially with a sub-pulmonic VSD and overriding
pulmonary artery (Taussig-Bing)
o mitral valve defects including
hypoplastic mitral valve
parachute mitral valve
abnormal papillary muscles
o patent ductus arteriosus
.there can also be non cardiac associations such as
 intracranial berry aneurysms
o
o spinal scoliosis
recognised syndromic associations include
o cardiac
Shone syndrome
o wider syndromic
PHACE syndrome
Turner syndrome: a coarctation can be seen in 15-20% of those with Turner syndrome

Radiographic features

Plain radiograph
figure of 3 sign: contour abnormality of the aorta
inferior rib notching: Roesler sign
o secondary to dilated intercostal collateral vessels which form as a way to bypass the coarctation and
supply the descending aorta
o the dilated and tortuous vessels erode the inferior margins of the ribs, resulting in notching
o seen only in long standing cases, and therefore not seen in infancy (unusual in patients <5 years of
age)7
o seen in 70% of cases presenting in older children or adults
o if the coarctation is distal to either subclavian artery, then increased flow occurs through the
subclavian artery, forming a collateral pathway via the internal thoracic artery, anterior intercostal
artery, posterior intercostal artery and then into the descending thoracic aorta
o usually the 4 to 8 ribs are involved; occasionally involves the 3 to 9 ribs
th th rd th

o as the 1 and 2 posterior intercostal arteries arise from the costocervical trunk (a branch of
st nd

the subclavian artery) and do not communicate with the aorta, these are not involved in collateral
formation, and the 1st and 2nd ribs do not become notched
o if bilateral rib notching: the coarctation must be distal to the origin of both subclavian arteries, to
enable bilateral collaterals to form
o if unilateral right rib notching
then the coarctation lies distal to the brachiocephalic trunk, but proximal to the origin of the
left subclavian artery
or there may be a right sided aortic arch with abberent left subclavian artery distal to coarctation
collaterals cannot form on the left, as the left subclavian is distal to the coarctation
o if unilateral left rib notching, then this suggests an associated aberrant right subclavian artery arising
after the coarctation. The coarctation is distal to the origin of the left subclavian artery, therefore
collaterals form on the left. Collaterals cannot form on the right, as the aberrant right subclavian
artery arises after the coarctation
may also show evidence of left ventricular hypertrophy

Antenatal ultrasound
 Useful in assessing for infantile coarctations. The suprasternal notch-long axis views are particularly
considered helpful. The fetal right ventricle can be appear enlarged in severe coarctations although this
alone is not a specific feature. Occasionally an aortic arch view may directly show a narrowing.

Angiography: CTA/MRA/DSA
All modalities are capable of delineating the coarctation as well as collateral vessels, most common
collateral pathway being subclavian artery to internal mammary artery to intercostal arteries (resulting
in inferior rib notching) to post-coarctation part of descending thoracic aorta.

Treatment and prognosis

The urgency of treatment depends on the presence of congestive cardiac failure. This is usually the case
in severe coarctations found in infancy. In less severe cases, elective treatment when the child is older
(typically ~2 years of age) is preferred 3.

Treatment can be either primary surgical repair with excision of the coarctation and end-to-end
anastomosis, or balloon angioplasty. Subclavian flap repair is a common surgical technique used, where
the origin and proximal left subclavian artery is excised, opened up and sutured onto the aorta. If the
subclavian is ligated, it is usually anastomosed onto the left common carotid artery.

Complications
neonatal heart failure
subarachnoid haemorrhage from a ruptured berry aneurysm
aortic dissection
infective endocarditis: in the context of an added infective insult
mycotic aneurysm: in the context of an added infective insult

Differential diagnosis
Imaging differential considerations include:

pseudo-coarctation of the aorta: elongation, narrowing or kinking with no pressure gradient or

collateral formation, no rib notching
chronic large vessel arteritis, e.g. chronic phase of Takayasu arteritis

PORTAL VEIN DOPPLER

Normally, the portal veins and hepatitic arteries flflow in the same directition, toward the liver. This
direction is called hepatopetal flflow (-petal = toward). The normal portal venous waveform is above the
baseline (hepatopetal) and gently undulating.

A pulsatile portal venous waveform is abnormal. The differential diagnosis for a pulsatile portal venous
waveform includes tricuspid regurgitation and right-sided CHF.

Portal pressure is defined as a direct portal venous pressure of >5 mm Hg, although the portal venous
pressure is not measured directly.
Ultimately, when portal venous pressure is higher than forward pressure, the portal venous flow will
reverse, which is diagnostic for portal hypertension. Reversal of portal venous flow is called hepatofugal
flow (-fugal = away, same Latin root as fugitive).

In addition to flow reversal, there are several secondary findings of portal hypertension:

Low portal venous velocity (<16 cm/sec).

Dilated portal vein (13 mm is the maximal normal diameter in quiet respiration).
Splenomegaly.
Varices.
Portosystemic shunts are often present, most commonly gastro-esophageal, paraumbilical, or
splenorenal. Note that an isolated portosystemic shunt may not be caused by portal
hypertension. For
instance, isolated obstruction of the splenic vein from pancreatitis or neoplasm may lead to a
shunt.
A recanalized umbilical vein is a portosystemic shunt that is diagnostic of portal hypertension.

Transjugular intrahepatic portosystemic shunt (TIPS)

Portal hypertension (and reversal of portal flow) can be treated with a transjugular intrahepatic
portosystemic shunt (TIPS), which connects a branch of the portal vein to a systemic hepatic
vein.
Ultrasound is used for surveillance of TIPS patency, starting with a post-procedure baseline.
Routine follow-up is performed according to the following schedule: In 1 month, every 3 months
for the first year, and then every 6 to 12 months.
Flow in a patent TIPS will be towards the hepatic veins, and flow in the portal veins will be
towards the TIPS. Therefore, flow in the main portal vein will be hepatopetal and flow in the
right and left portal veins will be hepatofugal (highlighted below with yellow circles).