Tricuspid pulmonary valves

TRICUSPID AND PULMONARY
VALVES DISEASES
DR.RIYADH W. AL ESAWI
DMRD, MSc, PhD
Diagnostic radiology
Assist. Prof. faculty of medicine/kufa university
8-5-2020

Anatomy of right atrium
Size < 45 ml
Pressure 2-5 mmHg
Oxygen sat. 65-75%
Post. wall smooth
Ant. wall trabeculated
Roof right cardiac auricle
Medial wall interatrial septum
Confluences SVC&IVC

Anatomy – The Right Ventricle
 Position: anterior to the left ventricle
 Pressure: 25/0 mmHG
 Size (end diastolic):
 men: 140 +/- 31ml
 women: 110 +/-24ml
 Myocardium: - caudally trabeculated
 RVOT = Right Ventricular Outflow Tract:
 - non trabeculated

Anatomy – The Tricuspid Valve
 Tricuspid valve = right atrioventricular valve
 Three leaflets: - anterior
 - posterior
 - septal
 Three papillary muscels
 Chordae tendineae
 Orifice Area: 5,0 – 5,5 cm²

Tricuspid valve anatomy
 Anterior leaflet is largest, attached to right AV junction.
 The septal leaflet is smallest.
 The septal leaflet attachment is from posterior ventricular wall
across the interventricular septum, its insertion being more
apical relative to the anterior leaflet.
 The posterior leaflet has mural attachment.
 The tendinous chords are attached to the ventricular surface of
the leaflets or the free edges of the leaflets to the papillary
muscle supporting the leaflet. There may be accessory chords
that attach from the septal leaflet to the moderator band or the
right ventricular free wall.

 There are 3 sets of papillary muscles, each set being
composed of up to 3 muscles. The chordae arising from
each set are inserted into 2 adjacent leaflets. Thus, the
anterior set of chordae insert in to half of the anterior and
half of the posterior leaflets, the medial set provides chordae
to anterior and septal leaflets. The third, posterior, set is
more rudimentary and is attached to the diaphragmatic wall
of the right ventricle.

Tricuspid valve disease
 Causes;
 Primary (organic) tricuspid valve disease include rheumatic disease,
endocarditis, prolapse and carcinoid.
 Secondary (functional) TR is caused by abnormalities of the RV
either as a result of infarction, volume or pressure overload
 TV is the most complex of the four cardiac valves.
 The three tricuspid valve leaflets are attached around the tricuspid
annulus.
 Three leaflets are not equally sized.
 The anterior or lateral is larger than septal and posterior leaflets.
 The septal is smaller than other and insert in more apical position.

Tricuspid stenosis
Tricuspid stenosis (TS) is the least common stenotic valve
lesion. In most cases, TS is of rheumatic origin and associated
with some degree of regurgitation.
In the presence of anatomic findings consistent with TS by
2D echo, a mean pressure gradient of 5 mmHg, T½ > 190
ms, inflow time velocity integral > 60 cm, and a valve area
by the continuity equation < 1 cm2 are consistent with
significant stenosis.
Supportive findings are a more than moderately enlarged
right atrium and a dilated inferior vena cava.

CHEST X RAY FINDING IN TS
-Right atrial enlargement.
-SVC enlargement.
-Calcification of TV , rarely seen.
- Features of congestive heart failure may be seen.

Giant right atrium due to rheumatic tricuspid stenosis

Marked Cardiomegally without failure. Prosthetic tricuspid valve
and sternotomy wires noted.

ECHOCARDIOGRAPHY
 If tricuspid velocity is more than 1 m/s, a stenotic valve is
suggested
 If velocity is more than 1.5 m/s , the stenosis is more likely, as
well as if the mean gradient is >2mmHg.
 Small rt.ventricle
 Large right atrium

Tricuspid stenosis
M mode: Stenotic valve had thickened leaflets with restricted
motion at the level of tips and chordae.
2D is more useful
Views are rt.para sternal long axis, short axis
Apical four chambers

Valve morphology
 Anatomical signs of TS are valve thickening and/or
calcification, restricted mobility, diastolic doming, and
right atrial enlargement.

Patient with severe combined rheumatic tricuspid valve disease. (a) Diastolic 2D
image, 4-chamber view, showing doming of the tricuspid valve. (b) Diastolic
colour Doppler image, 4-chamber view, indicating stenosis. (c) Systolic colour
Doppler image, 4-chamber view, showing tricuspid regurgitation. (d) CWD
recording

Tricuspid Stenosis Severity
 Measurement of the valve area (TVA) is difficult in
TS. It cannot be done by 2D echo, 3D echo has not
yet been validated for this purpose. Other Doppler
sonographic methods for calculating TVA also appear
to be less accurate than in MS.

Pressure gradient
 Mean pressure gradient can be measured easily by
Doppler, but has its limitations for quantification of
TS. The tricuspid inflow velocity is best recorded
from either a low parasternal right ventricular inflow
view or from the apical 4-chamber view. As tricuspid
inflow velocities are affected by respiration all
measurements must be averaged throughout the
respiratory cycle or recorded at end-expiratory
apnea.

 In patients with atrial fibrillation, average measurements of five
cardiac cycles should be taken at a mean heart rate of < 100
beats/min. In addition, mean gradients are highly dependent on
heart rate and flow (cardiac output, tricuspid regurgitation).
 Peak inflow velocity through a normal tricuspid valve rarely
exceeds 0.7 m/s.
 In general, the mean pressure gradient is lower in TS than in MS,
usually ranging between 2 and 10 mmHg, and averaging around
5 mmHg.
 Higher gradients may be seen with concomitant regurgitation.

Pressure Half -Time
 The pressure half-time (T1/2) method has been applied in
a manner analogous to MS. Constants of 220 as well as
190 have been proposed for valve area estimation. In
validation studies, TS valve area determined by the T1/2
method appeared less accurate than in MS. However, T1/2
values > 190 ms suggest significant stenosis.

Continuity Equation
The main limitation of the method is to obtain an accurate
measurement of the inflow volume passing through the
tricuspid valve. In the absence of significant tricuspid
regurgitation, the stroke volume obtained from either the left
or right ventricular outflow can be used; an estimated valve
area < 1cm2 is considered indicative of severe TS. However,
as severity of tricuspid regurgitation increases, the valve area
is progressively under-estimated by this method.

Tricuspid stenosis in echocardiogram. 2D and color Doppler images in a
patient with anti-phospholipid antibody syndrome shows thickening of the
valve leaflets (arrow) and continuous-wave Doppler with a mean pressure
gradient of 11 mmHg, which is consistent with severe tricuspid stenosis
Soham et al, Insights Imaging
2016 Oct; 7(5): 649–667

Role of MRI and CT
 Although MRI and CT should be feasible, these imaging
modalities have so far gained no role in the assessment of
TS.

Normal appearance of tricuspid valve. (a) Four-chamber CT image shows the septal (S)
and anterior (A) leaflets. (b) Two-chamber CT image shows septal (S) and posterior (P)
leaflets. (c) Short-axis CT image shows all the leaflets (S, A, P). (d) MRI appearance of
the tricuspid annulus, with the short-axis steady-state free precession (SSFP) image
showing all the leaflets (S, A, P)
2016 Oct; 7(5): 649–667

Tricuspid annulus. (a) Short-axis
CT image of the tricuspid
annulus,which is oval shaped and
non-planar, with the septal leaflet
attached in an oblique plane to the
septum. The posterolateral is the
lowest portion and the anteroseptal
the highest portion of the annulus.
(b) Measurement of the tricuspid
annulus in CT, including
circumference and diameters
2016 Oct; 7(5): 649–667

Tricuspid stenosis in CT and MRI. (a) Four-
chamber reconstructed CT image shows
thickening of the tricuspid leaflets (arrows)
in a patient with carcinoid and tricuspid
stenosis. (b) Four-chamber phase contrast
velocity-encoded image shows a high-
velocity jet extending across the tricuspid
valve, resulting in aliasing (arrow)
2016 Oct; 7(5): 649–667

TRICUSPID REGURGE
 CAUSES
 Functional ;secondary to marked dilatation of tricuspid
annulus due to RVH in the presence of pulmonary
hypertension, mitral valve disease, or replacement, IHD
or DCM.
 Rheumatic heart disease
 Endomyocardial fibrosis and carcinoid syndrome
 Ebsteins anomaly.
 Bacterial endocarditis.

CHEST X RAY IN TR
 Right atrial enlargement
 PA; increased arch of right heart border
 Lateral; increased retrosternal opacity between aortic arch
and outflow tract of RV.
 Other subtle findings
 Right ventricular enlargement.
 Reduced prominence of pulmonary vascularity.
 SVC enlargement.
 IVC enlargement.
 Features of congestive heart failure may be seen.

Echo in Tricuspid regurgitation
 Diagnosis of TR by color Doppler image in right para
sternal view, short axis and 4 chamber views
 TR present in 75% of normal population
 Causes are functional due to annular dilatation as a result
of pulmonary hypertension.
 -rheumatic heart disease
 -Ebstein anomaly

Two-dimensional transthoracic
Echocardiographic appearances of severe
tricuspid regurgitation. In the parasternal
tricuspid inflow view, there is mal-
coaptation of the anterior and posterior
tricuspid valve leaflets ((7A)7A that
gives rise to a severe jet of regurgitation
seen on colour Doppler
imaging imaging7B. 7C shows the
continuous wave Doppler appearance of
severe tricuspid regurgitation (dagger
shape).
Ronak et al, Arq Bras Cardiol. 2014 Sep; 103(3): 251–263

Echocardiography of tricuspid
regurgitation (TR). (a) Apical four-
chamber view with color Doppler
demonstrates a markedly enlarged
RA and a large TR jet (arrow)
coursing along the interatrial
septum. (b) Parasternal short-axis
view with color Doppler
demonstrates elevated RV pressure
via peak TR jet velocity. (c)
Hepatic vein continuous-wave
Doppler with systolic reversal of
flow in the setting of severe TR
Soham et al, Insights Imaging 2016 Oct; 7(5): 649–667

Ebstein anomaly
EA is a myopathy of the RV with abnormalities in both
myocardial structure and function, besides the characteristic
tricuspid valve deformities , in which there is apical
displacement of the septal leaflet and tethering of lateral
leaflet to the ventricular wall, result in atrialization of a
portion of right ventricle.
Embryonic failure of delamination of the septal, inferior and
anterior leaflets of the TV results in the apical displacement
of the tricuspid leaflets to the underlying RV myocardium.

 Plain Chest Radiograph of a Patient With Ebstein
Anomaly Showing Marked Cardiomegaly
Muhammad et at.JACC: Cardiovascular
Imaging, Volume 12, Issue 4, April 2019

Grades of tricuspid regurgitation (TR). (a) Four-chamber steady-state free precession (SSFP)
shows trace TR, extending just adjacent to the tricuspid valve (arrow). (b) Mild TR, with the jet
extending just beyond the tricuspid valve into the proximal aspect of the right atrium (RA). (c)
Moderate TR, with the jet extending to the middle third of the RA. (d) Severe TR, with the jet
reaching the posterior wall of the RA
2016 Oct; 7(5): 649–667

Quantification of tricuspid
regurgitation (TR). (a) Direct
measurement of TR using a
velocity-encoded phase-
contrast image obtained in the
short-axis plane of the
tricuspid valve. (b) Indirect
measurement of TR using
stroke volume from cine
steady-state free precession
(SSFP) and forward flow
from the pulmonary artery

CMR of a Patient With Ebstein Anomaly The dotted line between the right atrium (RA) and the atrialized right ventricle
(aRV) marks the anatomic atrioventricular grove, and the dashed line between the aRV and the functional right ventricle
(fRV) marks the functional tricuspid valve orifice. (A) Four-chamber view showing apical displacement of the septal
leaflet. (B) Right ventricular inflow-outflow view demonstrating rotation of the functional tricuspid valve orifice toward
the right ventricular outflow tract.
Muhammad et at.JACC: Cardiovascular Imaging, Volume 12, Issue 4, April 2019

Cardiac MRI of a patient with Ebstein’s anomaly showing displacement
of septal leaflet, atrialized right ventricle (ARV) and small functional
right ventricle (fRV); LA, left atrium; LV, left ventricle; and RA, right
atrium.
Sinem et al 2018

Cardiac MRI showing septal bowing in short axis views. (Used with
permission of the Sonomed Imaging Center).
Sinem et al 2018

Quantification of tricuspid regurgitation
(TR). (a) Vena contracta is the diameter
of the smallest regurgitant flow (straight
arrow) before expansion of the jet
(curved arrow) in a four-chamber view.
(b) Effective regurgitant orifice is
measured from a short-axis cine steady-
state free precession (SSFP) image
through the tricuspid valve in systole. (c)
Failure of coaptation of leaflets in four-
chamber cine image (arrowheads) in a
patient with significant TR (arrow). (d)
Jet area (blue) and jet area/right atrial
area are measured in a four-chamber
SSFP or phase-contrast image. TR grade:
mild > 5 cm2, moderate 6–10 cm2, severe
>10 cm2. Ratio of regurgitant area to RA
area: mild > 20 %, moderate 20–34 %,
severe > 35 %. (e) Short-axis steady-
state free precession (SSFP) image
through the ventricles shows a flat
ventricular septum (arrow). (f) CT image
through the liver shows reflux of
contrast through the hepatic veins and
IVC
2016 Oct; 7(5): 649–667

Tricuspid regurgitation (TR). (a)
Four-chamber steady-state free
precession (SSFP) image in a
patient with functional TR shows
dilation of the annulus > 35 mm.
The annulus is also flat and planar.
Dilation occurs along the free wall
of the tricuspid annulus. The septal
wall is intact. (b) Tethering. Four-
chamber SSFP MRI image shows
distance from the annular plane to
coaptation in systole > 8 mm and
area > 1.6 cm2, predicting residual
TR following surgery
2016 Oct; 7(5): 649–667

(A ) Echocardiographic view (four-chamber view, apex up) of a patient with
Ebstein’s anomaly showing a displaced septal leaflet (arrow). (b)The anterior
leaflet is severely tethered and nearly immobile. The functional right ventricle
(RV) is small. aRV indicates atrialized right ventricle; LA, left atrium; LV, left
ventricle; and RA, right atrium.
A B
Sinem et al 2018

An echocardiogram (four-chamber view, apex up)showing the displacement of septal leaflet(in
the right hand panel), anterior leaflet fenestrations, and tricuspid regurgitations (in the left hand
panel). The hinge point of the normal septal tricuspid leaflet is positioned slightly toward the
cardiac apex relative to the septal hinge point of the anterior mitral leaflet. This displacement is
exaggerated in hearts with Ebstein’s malformation, as shown in the image (outlined by the
arrow, in the right hand panel) This can be quantitated by the displacement index, dividing the
distance between the valvar insertions divided by the body surface area. A value of greater than
8 mm/m2 is diagnostic of Ebstein’s malformation. It should be noted that the valvar leaflets are
also abnormal in Ebstein’s malformation. LA, left atrium; LV, left ventricle; RA, right atrium;
RV, right ventricle.
Sinem et al 2018

Tricuspid Atresia
 Tricuspid atresia is the third most common congenital cyanotic
heart disease, accounting for 2 % of all congenital cardiac
disorders .
 This is caused by embryological fusion of the dorsal and
ventral endocardial cushions too far to the right lateral position.
There is resultant obstruction to outflow from the RA to the
RV; hence, both atrial septal defect (ASD) and ventricular
septal defect (VSD) or a patent ductus arteriosus (PDA) are
necessary for survival. There are three subtypes: I, normal great
arterial arrangement (70 %; II, D-transposition of the great
arteries

X-rays from a female with tricuspid atresia, normally related great arteries, a nonrestrictive
ventricular septal defect, and a large ostium secundum atrial septal defect. A, At age 11 years,
pulmonary vascular resistance was below systemic, pulmonary vascularity was increased, the
left ventricle (LV) was enlarged, and the pulmonary trunk (PT) and right atrium (RA) were
dilated. B, At age 19 years, the pulmonary vascular resistance was supra systemic, pulmonary
vascularity was normal, and the pulmonary trunk and right atrium remained dilated, but the left
ventricle was no longer enlarged. Overlying breast tissue accounts for prominent lower lung
field radio densities.

X-rays from an 18-year-old man with tricuspid atresia, complete transposition of the great
arteries, a nonrestrictive ventricular septal defect, and supra systemic pulmonary vascular
resistance. A, Pulmonary vascularity is diminished, and the dilated hypertensive posterior
pulmonary trunk is border-forming (PT). The right cardiac silhouette is hump-shaped
because a prominent superior border is caused by an enlarged right atrium (RA) and a
receding inferior border, which is caused by a hypoplastic right ventricle. B, Left anterior
oblique projection highlights the hump-shaped right superior border

Tricuspid atresia. (a) Four-chamber
steady-state free precession (SSFP) image
shows absent tricuspid valve, which has
been replaced by a fatty wedge of tissue in
the right AV groove (arrow), a hypoplastic
RV and dilated right atrium (RA). (b)
Tricuspid atresia in a patient with dextro-
transposition of the great arteries. The RV
is hypoplastic. Note that this has been
treated with a Fontan shunt (arrow)
2016 Oct; 7(5): 649–667

Transthoracic Echocardiography of a Patient With Tricuspid Valve Dysplasia
(A) Apical 4-chamber view shows abnormal tricuspid valve with a coaptation gap (yellow
arrowhead), without apical displacement of the septal leaflet. (B) Parasternal long-axis view in 2
dimensions and color shows no displacement of inferior leaflet but restricted opening in diastole.
RA = right atrium; RV = right ventricle
Muhammad et at.JACC: Cardiovascular Imaging,
Volume 12, Issue 4, April 2019

Other causes
pace maker and catheter
ischemic heart disease
endocarditis, carcinoid syndrome and Amyloidosis
TV prolapse occur due to myxomatous valve syndrome
commonly seen with mitral valve prolapse.

 Indirect sign of TR are dilatation of IVC and/or loss of
respiratory variation seen by M mode.

Gerbode defect
 A Gerbode defect is a ventriculoatrial defect, with
communication between the LV and the RA. In
the congenital type, there is a defect in the AV component of the
membranous septum, resulting in direct shunting from the LV to
the RA, above the hinge points of the tricuspid valve leaflets.
 In the indirect type, the shunting happens through a peri-
membranous VSD into the RV below the level of the tricuspid
valve, after which the atrial shunting happens through a cleft in
the septal leaflet of the tricuspid valve . The acquired type of
Gerbode defect is caused by trauma, iatrogenic surgery or
infective endocarditis. Gerbode defects are treated with surgical
closure using a patch.

Gerbode defect. Four-chamber
steady-state free precession
(SSFP) image shows a defect
and a shunt extending from
the left ventricle (LV) to the
right atrium (RA) through a
Gerbode defect.

Carcinoid tumor
 A carcinoid tumour is a slow-growing neuroendocrine cell-derived
neoplasm. The appendix and terminal ileum are the most common
locations of primary carcinoid tumours, whereas the liver is the
most frequent site of metastasis 50 %.
 The tricuspid valve is the most common cardiac valve that is
involved by metastatic carcinoid tumours.
 Carcinoid syndrome occurs when vasoactive products (5-
hydroxytryptamine, serotonin, histamine, tachykinins,
prostaglandins) released by a metastatic liver carcinoid overwhelm
the normal function of the liver to inactivate these products.
Cardiac lesions are seen in 50–60 % of patients with carcinoid
syndrome, typically 18–24 months after diagnosis .

Carcinoid. (a) Axial CT of the
abdomen shows a metastatic
carcinoid tumour (arrow) in the
liver. (b) Four-chamber steady-state
free precession (SSFP) image
shows a thickened tricuspid leaflet
(straight arrow) with moderate
tricuspid regurgitation (curved
arrow). Mitral regurgitation is also
visible.

Grading of TR
parameter mild Moderate Severe
Tricuspid valve normal Normal or
abnormal
Abnormal
leaflet, poor
coaptation
RV,RA,,IVC
size
normal Normal or
dilated
Usually dilated
Color neck mm Usually none <7 >7
Jet area cm2 <5 5-10 >10
Jet/RA ratio <20% 20-34% >35%
CW envelope incomplete Moderate
intensity
Dense may be
triangular

Three examples of various degrees of TR, moderate (left), severe
(middle), and massive (right) are provided. The RJA increases with the
severity of TR. The peak velocity of TR (CW Doppler) allows the
estimation of pulmonary pressure except in case of massive TR,
as the Bernouilli equation is not applicable

Quantitative assessment of TR severity using the VCW and the PISA method. Stepwise
analysis of TR. (a) Apical 4-chamber view (AP 4-CV). (b) Colour flow display. (c)
Zoom of the selected zone to obtain the vena contracta and the three components of
TR jet. (d) Downward shift of zero baseline to obtain an hemispheric PISA

Sub-costal echocardiogram recorded in a patient with severe TR. (a–c) The colour
Doppler confirms retrograde flow into the vena cava and hepatic vein in systole
consistent with TR (red). (d) A spectral Doppler recording from a hepatic vein, also
confirming the systolic retrograde flow

Anatomy – The Pulmonary Valve
Tricuspid valve = right semilunar valve
- Located between RVOT and Pulmonary
artery
- Three cusps: - anterior
 - right
 - left
- Orifice Area: 2,5 – 3,0 cm²

(a) Axial CT image of the heart,
shows the pulmonary valve sinuses
posterior (P), right anterolateral
(Ra), and left anterolateral (La) in
relation to the heart. (b) Short-axis
CT image shows the pulmonary
sinuses anterior (A), left posterior
(Lp), and right posterior (Rp) in
relation to the heart. Depending on
their relation to the aorta, the
sinuses are left-facing, right-facing,
or nonfacing. The same rules may
be applied to the aortic valve. (c)
Photograph of a cadaveric heart
shows the pulmonary valve sinuses.
Saremi et al , 2014

PULMONARY VALVE STENOSIS
Pulmonary stenosis is almost always congenital of origin.
Stenosis below (proximal to) the pulmonary valve may
result from both congenital and acquired causes. Stenosis
of the pulmonary artery distal to the valve may occur in
the main pulmonary trunk, or more distally in the branch
vessels.
The most common type of congenital pulmonary stenosis is
a dome-shaped pulmonary valve, which accounts for
40%–60% of pulmonary valve stenosis. It is characterized
by a mobile valve with two to four raphes and incomplete
separation of valve cusps, a result of commissural fusion,
which creates a funnel shape with a small circular orifice.

PV stenosis
 Domed – 42%
 Tricuspid - 6%
 Bicuspid - 10%
 Unicommissural - 16%
 Hypoplastic annulus - 6%
 Dysplastic-20%
 Echo PS_PAIVS_PR SCAI 2013

Valve Anatomy
Evaluation of anatomy is important in defining the
location of stenosis. The valve itself may be dome-
shaped , dysplastic, or, in rare cases, calcified. A post-
stenotic dilatation is common in dome-shaped valves.

PV stenosis
Prevalence = 8-12% of all CHD
Familial recurrence rate = 2 - 4% in 1st degree relatives
Noonan’s syndrome
–50% of patients have form with PS
– autosomal dominant transmission
– 25% offspring affected

Pulmonic Stenosis
 Without VSD = 8% of all CHD
 Mostly asymptomatic
 When symptomatic
 Cyanosis and heart failure
 Cor pulmonale
 Loud systolic ejection murmur

Types of PS
 Subvalvular
 Valvular
 Supravalvular

VALVULAR PS
 Classic pulmonic stenosis (95%)
 Congenital in origin
 Associated with metastatic carcinoid syndrome
 Tricuspid valve dz as well
 Associated with Noonan Syndrome
 ASD
 Hypertrophic cardiomyopathy

VALVULAR PS
 Presents in childhood
 Pulmonic click
 Dome-shaped pulmonic valve in systole
 RX: Balloon valvulo-plasty

Xray finding
 Enlarged main pulmonary artery .
 Enlarged left pulmonary artery , due to jet effect.
 Normal to decreased peripheral pulmonary vasculature.
 Rare calcification of pulmonary valve in older adults.

Normal heart size
Enlarged main p a
Enlarged left pa
Pulmonary stenosis

Subvalvular PS
 Infundibular pulmonic stenosis
 Typically in Tetralogy of Fallot
 50% of pts with TOF also have bicuspid pulmonic valves
 50% of patients with TOF also have valvular pulmonic
stenosis
 Subinfundibular pulmonic stenosis associated with VSD
(85%)

Concave pulmonary artery
segment
TOF with subvalvular stenosis

TRILOGY OF FALLOT
 Severe pulmonary valve stenosis
 RV hypertrophy
 ASD with R L shunt

SUPRAVALVULAR PS
 May be either tubular hypoplasia or localized with poststenotic
dilatation

Supravalvular PS associated CV
anomalies
 Valvular pulmonary stenosis
 Supravalvular aortic stenosis
 VSD, PDA
 Systemic arterial stenosis

Supravalvular stenosis associated
syndrome
 Williams Syndrome
 􀂄Pulmonic Stenosis
 􀂄Supravalvular AS
 􀂄Peculiar facies
 Post-rubella syndrome
 Carcinoid syndrome with liver mets
 Ehlers-Danlos syndrome

PV Stenosis; Enlarged main pulmonary and left
pulmonary artery and normal right pulmonary
artery, dilated rt.ventricle..

Role of MRI and CT
Of limited role.
MRI is, however, definitely helpful in patients with poor image
quality and for detailed anatomic evaluation (subvalvular–
valvular– supra-valvular stenosis–pulmonary arteries).

Pulmonary Valve Stenosis – Natural History
 PS may progress rapidly during infancy
 Mild PS rarely progresses after 2 y.o.
 Valve thickness/mobility does not correlate with
progression.

(a) Photograph shows the dome-shaped pulmonary valve from the arterial aspect. The
fused commissures (yellow arrows) pull the sinutubular junction toward the central circular
orifice (red arrow). (b, c) Dysplastic pulmonary valve stenosis. Sagittal (b) and axial (c) CT
angiograms of the valve show club-shaped myxomatous thickening (red arrows) limited to
the free margin of the leaflets. The proximal part of the leaflets appears intact. The sinuses
of Valsalva are well developed, and the sinutubular junction (green arrows in b) is
constricted. MPA = main pulmonary artery, RVOT = right ventricular outflow tract.
Saremi et al , 2014

Pulmonary stenosis in a 19-year-old man with LEOPARD syndrome. Volume-rendered (a) and
sagittal (b) CT images of the RVOT show mild thickening of the valve leaflets, narrowing at
the sinutubular junction (red arrow), dilated coronary arteries (green arrow in b),
poststenotic dilatation of the pulmonary arteries, and a dilated right ventricle (RV). LPA =
left pulmonary artery.
Saremi et al , 2014

Severe congenital pulmonic stenosis. SSFP) in a sagittal
view through the right ventricular outflow tract,
demonstrating mobile leaflets but fused tips of the pulmonic
valve (arrow).

Contrast-enhanced MR angiography of a patient
with severe supra-valvular pulmonic stenosis, after
surgery for complex congenital heart disease.

Bi- and quadricuspid pulmonary valves. (a, b) Trans axial (a) and long-axis (b) MR images show a
stenotic bicuspid pulmonary valve (arrow in a), flow jet (arrows in b), and post stenotic dilatation of
the left pulmonary artery (LPA).
(c, d) Axial (c) and anterior volume-rendered (d) CT images show a quadricuspid valve with pulmonary
stenosis. One extra rudimentary cusp is seen between the posterior and left anterior cusps (arrow in c), the
main pulmonary artery (MPA) appears aneurysmal, and the right ventricle (RV) is hypertrophied
Saremi et al , 2014

Color Doppler flow
shows Post-stenotic
dilatation
– Eccentric Flow
May extend into
LPA
Not usual with
dysplastic PV

Patient with severe pulmonic stenosis. (a)Parasternal short-axis view, 2D image
showing doming of the pulmonic valve. (b) Parasternal short-axis view, colour
Doppler image, showing flow acceleration and the stenotic jet. (c) CWD recording of
pulmonic stenosis.
(d) CWD recording of the corresponding tricuspid regurgitation

Pulmonary Valve Stenosis - Progression
Echo PS_PAIVS_PR SCAI 2013

Stenosis severity
Pressure Gradient
Quantitation of pulmonary stenosis severity is primarily
based on the trans-pulmonary pressure gradient, using the
simplified Bernoulli equation, DP = 4v².
-parasternal short-axis view.
-Sub-costal window.
-A modified apical 5-chamber view.

Pulmonary valve stenosis severity
 Severity Peak Doppler (mmHg)
 Trivial <25
 Mild 25-40
 Moderate 40-60
 Severe >60

 Muscular infundibular obstruction is frequently characterized
 by a late peaking systolic jet that appears “dagger
 shaped,” reflecting the dynamic nature of the obstruction;
 this pattern can be useful in separating the dynamic muscular
 obstruction from a fixed valvular obstruction, where the peak
 velocity is generated early in systole.
 Severe stenosis is defined by a peak jet velocity of > 4 m/s
 (peak gradient > 64 mmHg), moderate stenosis is defined by
 a peak jet velocity of 3–4 m/s (peak gradient 36–64 mmHg),
 and mild stenosis is defined by a peak jet velocity of < 3 m/s
 (peak gradient < 36 mmHg).

Other Indices of Severity
The continuity equation and the PISA method, are not well
validated. Pulmonic valve area by planimetry is not possible, as
the required imaging plane is generally not available.
A useful index of severity is the right ventricular systolic
pressure from the tricuspid regurgitant velocity and the addition
of an estimate of right atrial pressure.
The pulmonary artery systolic pressure should equal RV systolic
pressure minus pulmonary valve pressure gradient.

Right Ventricular Systolic Pressure
 the trans-tricuspid systolic gradient estimated from the peak
tricuspid velocity.
 The formula is: The right atrial pressure may be estimated by
examination of the patients neck veins. Using this method, mean
jugular venous pressure (JVP) in cmH20 is first estimated by
inspection of the jugular venous pulse with the patient at 45
degrees.
 Right atrial pressure (RAP) is estimated by adding 5 cm to the
venous pressure measurement (to approximate the distance
between the right atrium and the clavicle) and then converted to
mmHg by dividing by 1.3. This is then added to RVSP=
(JVP+5/1.3)+(peak systolic velocity2x4)

Pulmonary hypertension
 Theoretically, calculation of mean PAP from PA systolic
pressure is possible;
mean PAP = 0.61 x PA systolic pressure+ 2 mmHg.
PA systolic pressure = tricuspid regurgitation pressure gradient
+estimated right atrial pressure .

1- Pulmonary artery systolic pressure by TR peak
velocity
 Continuous wave (CW) Doppler of the tricuspid
regurgitation (TR) trace is used to measure the difference
in pressures between the right ventricle and right atrium.
The simplified Bernoulli equation (P = 4[TRmax]2) is
used to calculate this pressure difference using peak TR
velocity. This method correlates well with PASP on right
heart catheterisation .
 A peak TR velocity value of ≤ 2.8 m/s is considered
normal.

 Method: A coaxial TR jet is identified in parasternal long axis (RV
inflow), parasternal short axis, or apical 4-chamber view with the
help of colour Doppler. CW Doppler is used with a sweep speed of
100 mm/s to achieve a satisfactory envelope .
 The peak velocity of the envelope is then measured (TRmax). A
value of ≤ 2.8 m/s suggests low probability, a value of 2.9–3.4 m/s
indicates intermediate probability, and a value > 3.4 m/s suggests a
high probability for pulmonary hypertension . Traditionally, right
atrial pressure (RAP) is assumed by the size and distensibility of
inferior vena cava (IVC) during inspiration at rest and during forced
inhalation, and this value is added to the peak TR velocity .
However, recent ESC guidelines suggest just using the TRmax
without additional RAP, as IVC assessment is error prone . Mean
PAP can be approximated from the systolic PAP (SPAP) using the
following formula: mPAP = 0.61*SPAP + 2 mmHg

TR Vmax method for measuring PASP.

pulmonary hypertension
Peak pressure gradient of tricuspid regurgitation = 4x
(tricuspid regurgitation velocity)2. This equation
allows for estimation of PA systolic
pressure taking into account right atrial pressure:
PA systolic pressure = tricuspid regurgitation pressure
gradient +estimated right atrial pressure

2-Mean pulmonary artery pressure from peak PR
Doppler signal
Method
A pulmonary regurgitation (PR) signal is obtained in the
parasternal short axis view using colour Doppler. CW Doppler
at a sweep speed of 100 mm/s is used to measure the peak PR
velocity .
Peak pressure difference (measured by the Bernoulli equation)
is then added to the RAP. This method has been validated
against gold standard catheter-measurements.
Mean PAP can be approximated from the peak PR Doppler
signal using the following formula: mPAP = 4(PRpeak
velocity)2 + RAP.

Pulmonary regurgitation method for measuring mean and diastolic
pulmonary artery pressure.
Sashith et al,Int J Cardiol Heart Vasc2016 Sep; 12: 45–51

3-Pulmonary artery diastolic pressure measured by PR-
end velocity
 A PR signal is obtained as above. End PR velocity is measured
in multiple (non-continuous) traces and averaged. Pulmonary
artery diastolic pressure (PADP) is calculated from the
following equation: 4(PR-end velocity)2 + RAP. Mean
pulmonary artery pressure can be calculated from systolic (by
TRmax method) and diastolic (by PR-end velocity method)
pulmonary artery pressures: mPAP = 2/3rd of PADP + 1/3rd of
PASP.

4-Mean pulmonary artery pressure from right
ventricular outflow tract (RVOT) acceleration
time
 Pulse wave of RVOT normally produces a dome shape,
but in patients with pulmonary hypertension, there is rapid
rise to peak, resulting in shorter acceleration time .
 A mid-systolic notching could also indicate pulmonary
hypertension .

 Right ventricular outflow tract (RVOT) acceleration time is
measured from the beginning of the flow to the peak flow
velocity.
 It is important that the marker is placed at the peak first and then
tracked back to the onset of flow, as the aim is to measure time
taken to peak velocity and not the propagation. A value of
> 130 ms is normal, while < 100 ms is highly suggestive of
pulmonary hypertension .
 Mean pulmonary pressure is calculated by the formula:
mPAP = 90 − (0.62*ATRVOT).
 For example, if the ATRVOT is 80 ms, the mPAP = 90 −(0.62*80),
that is 40.4 mmHg (normal < 25 mmHg). On the other hand, if
the ATRVOT is 137 ms , then the calculated mPAP is
90 −(0.62*137) = 50.6 mmHg.

RVOT acceleration time method for assessing pulmonary pressure.
A—Pulmonary acceleration time measurement.
B—Rapid rise and mid-systolic notching suggesting elevated pulmonary pressure.

5-Mean pulmonary artery pressure from TR velocity-
time integral
 In this fairly new technique, CW Doppler of the TR jet is
traced and the mean pressure difference is measured from the
velocity-time integral (VTI) .
 RAP is then added to calculate the mPAP. Mean PAP
measured by this method correlates closely with catheter-
measured mPAP .
 The mPAP from TR VTI can be calculated using the following
formula: mPAP = meanΔP + RAP.

Tricuspid regurgitation velocity-time integral method for measuring mPAP.

6-RV free wall strain Sm, SmVTI
 Method
 Tissue Doppler imaging (TDI) is used on the RV free
wall in the apical 4-chamber view, and tricuspid
annular systolic myocardial (Sm) velocity is recorded.
The maximal Sm velocity and the SmVTI are then
measured . Sm velocity < 12 cm/s and SmVTI < 2.5 are
highly suggestive of elevated PASP

RV tissue Doppler method for assessing pulmonary pressure.

7-Right ventricular isovolumic relaxation time
(rIVRT)
 TDI is deployed at the lateral tricuspid annulus with a
sweep speed of 100 mm/s. Pulse wave (PW) Doppler with a
6 mm sample window is obtained. Right ventricular
isovolemic relaxation time (rIVRT) is measured from the
offset of the S′ wave to the onset of the E’ wave.
 rIVRT of > 75 ms reliably predicts pulmonary hypertension
while an rIVRT of < 40 ms has a high negative predictive
value for pulmonary hypertension.

Right ventricular isovolemic relaxation time measurement.

Severe PS suggested by
 Maximum trans-pulmonary velocity (Vmax) is >4m/s
 Mean pressure difference >50 mmHg.
 Mean pressure difference <50 mmHg with right ventricular
dysfunction.

Pulmonary valve regurge
 Causes- conditions that dilated pulmonary valve ring e.g
pulmonary hypertension
 Surgical correction of pulmonary stenosis.
 Chest x ray findings
 Sign of pulmonary regurge on chest X ray are subtle but
include
 Right ventricular enlargement.
 Prominent pulmonary trunk.
 Features of tricuspid regurge may be present.
 Features of congestive heart failure may be present.

Pulmonary valve aneurysm of the sinus of Valsalva and paravalvular fistula. CT images
obtained with volume rendering (a) and at the level of the RVOT (b) clearly show an
aneurysm (green arrows) in the medial aspect of the pulmonary valve (PV) straddling
the valve leaflet (yellow arrow in b). Two orifices (red arrows in b) are seen (one above the
valve and the other below) and are the cause of a paravalvular fistula and regurgitation.
These findings are indicative of pulmonary valve aneurysm of the sinus of Valsalva and
paravalvular fistula, sequelae of Behçet arteritis, which is possibly a result of aseptic
endocarditis.

Pulmonary regurge
• Assessment from
multiple views
• Width of PR jet
• Color flow for extent of
diastolic flow reversal

Bi dimensional trans thoracic echocardiographic appearances of
severe pulmonary regurgitation. In diastole, the colour Doppler jet is
seen to occupy the entirety of the right ventricular outflow tract (8a).
On continuous wave Doppler imaging, the pressure half-time is <
100 ms in keeping with severe regurgitation (8b).
Ronak et al, Arq Bras Cardiol. 2014 Sep; 103(3): 251–263

Geva Journal of Cardiovascular Magnetic Resonance 2011 13:9
CMR – RV Dilatation

CMR – PR Fraction
Geva Journal of Cardiovascular Magnetic Resonance 2011 13:9

Real Time 3D Echo RV Volume
• Limitations of 3DE RV Vol
– Visualization of endocardium
– Field of view
– Volumes/sec
Echo PS_PAIVS_PR SCAI 2013

Severe regurgitation suggested by
 Wide color jet >7.5 mm or filling the right ventricular
outflow tract
 Diastolic flow reversal visible in the distal main
pulmonary artery
 A steep dense signal (pressure half-time <200 ms)
 Active dilated right ventricle.

Tricuspid pulmonary valves

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