This document discusses various congenital anomalies of the mitral valve and mitral apparatus that can be assessed using echocardiography. It begins by describing the normal anatomy of the mitral valve complex. It then discusses specific anomalies in more detail, including isolated cleft mitral valve, double orifice mitral valve, mitral ring, and others. For each anomaly, it provides descriptions of the typical echocardiographic findings and views useful for assessment. The document emphasizes the importance of a thorough echocardiographic examination of the entire mitral valve complex to accurately characterize any congenital anomalies.

1. ECHOCARDIOGRAPHY OF CONGENETAL LV
AND RV INFLOW ANOMALIES
Dr G.RAGHU KISHORE

2. INTRODUCTION
• Congenital anomalies of the mitral valve represent a wide spectrum of lesions that
are often associated with other congenital heart anomalies.
• In a study by Banerjee A, Kohl T, Silverman NH et al (Am J Cardiol 1995;76:1284—
91) congenital malformations of the mitral valve were detected in almost 0.5% of
the 13,400 subjects.
• These lesions can have a variable impact on valve function. When necessary,
surgical repair provides good long-term results.
• Although mitral valve replacement appears to provide acceptable mid- and long-
term results , mitral valve repair is always preferable when possible.

3. • Because suboptimal primary repair is a significant predictor for
reoperation, the successful management of congenital mitral valve
disease is closely dependent on the preoperative assessment of the
anatomical substrate.
• An accurate description of the malformations can be achieved
through echocardiography but requires prior knowledge of these
lesions.
• the mitral valve should be analysed as an entire complex, including
the valvular leaflets, tensor apparatus and papillary muscles.

4. Anatomy of mitral valve
 Mitral valve apparatus :-
 Mitral valve Annulus.
 Mitral leaflets with
commissures.
 Chordae tendinae.
 Papillary muscles.
 Supporting LV Wall.
 Altogether called as mitral valve
complex.
 Resembles the Bishops “mitre” .

5. Mitral valve Annulus
 Annulus :- fibroelastic ring.
Encircles the valve orifice in
cone like manner.
 Annulus is elliptical in shape in
systole & circular in diastole.

6. Normal mitral anatomy. (A) Schematic representation of the saddle-shaped mitral annulus. (B) Anatomical photograph of a
normal mitral complex with its two papillary muscles connected to the leaflets by chordae tendinae. The aortic valve is in direct continuity
with the anterior leaflet of the mitral valve. (C) Photograph of a normal mitral valve seen from the left atrium (as seen by a surgeon). (D)
Both leaflets are divided into three scallops according to the classification by Carpentier et al.. LV: left ventricle; PM: papillary muscle.

7. Mitral leaflets & commissures
 AML :- Anterior mitral leaflet.
 triangular in shape.
 Is in continuity of aortic
annulus.
 Encircles on 1/3rd of annulus,
but covers 2/3rd of valve orifice
area.
 PML :- posterior mitral leaflet.
 Quadrangular in shape.
 Occupies 2/3rd of the annulus,
but covers only 1/3rd of the
valve area.

8. Carpentiers nomenclature
 Anterior leaflet is termed as “A”.
 A1 scallop:- lateral third.
 A2 scallop:- middle third.
 A3 scallop:- medial third.
 Posterior leaflet is termed as “P”.
 P1 scallop:- lateral third.
 P2 scallop:- middle third.
 P3 scallop:- medial third.

9. Chordae tendinae
 These are fine fibrous strings
radiating from the papillary
muscles and attach to
corresponding halves of the
anterior and posterior mitral
leaflets.
 Chordae arising from the APM,
attach to lateral half of
A2,A1,AC,P1,lateral half of P2.
 Chordae arising from PPM,
attach to medial half of A2, A3,
PC, P3, medial half of P2.

10. Papillary Muscles
 Located at the junction of the
apical (lower) third & middle
third of the left ventricle.
 2 in number.
 APM :- antero-lateral wall of LV.
 PPM :- postero-medial wall of
LV.
 APM :- has dual blood supply.
- OM of LCX.
- D1 of LAD.
 PPM:- has single blood supply.
- Last OM/ RCA.

11. (A) Echocardiographic parasternal long-axis view showing a normal mitral complex
(B) Echocardiographic parasternal short-axis view showing the normal position of the papillary muscles
(C) Three-dimensional echocardiography of a normal mitral valve.

12. The major axis (inter
commissural diameter) of the
mitral annulus is found at a
bicommissural 2-chamber TTE
view (when P1-A2-P3 mitral
leaflet scallops are visualized)
or a mid-oesophageal bi
commissural view at 45-60°
during TEE
minor axis (antero-posterior
diameter) of the mitral
annulus can be performed at
end-systole during TTE in the
apical long axis view (3-
chamber view) or its TEE
equivalent, found at mid-
oesophageal level with 135°
tilt of the probe

22. Congenital anomalies of the mitral valve
• Mitral leaflets
Mitral valve prolapse (MVP)
Isolated cleft mitral valve (ICMV)
Double orifice mital valve (DOMV)
Mitral Ring
Congenital MS
Ebstein’s malformation of the mitral valve
• Tensor apparatus
Arcade or hammock valve
Straddling mital valve
• Papillary muscles
Parachute mitral valve

23. Isolated cleft(ICMV)
• Isolated cleft of the anterior mitral valve leaflet is a rare but well-
known finding the origin of which is under debate.
• some authors have considered isolated cleft to be a ‘forme fruste’ of
AVSD whereas others have supposed it to be a distinct morphological
entity.
• The definition of a mitral cleft is a division of one of the leaflets
(usually the anterior leaflet) of the mitral valve.
• This must not be mistaken with the so-called ‘cleft’ in AVSD

24. ICMV
• On transthoracic echocardiography, it looks like a slit-like hole in the anterior mitral
leaflet.
• Chordal attachments may connect the edges of the cleft to the ventricular septum
and subsequently create a subaortic obstruction.
• Rarely, isolated cleft may be seen in the posterior leaflet of the mitral valve.
• Although it may occur at any segment of the posterior leaflet, the predominant
localization of the cleft is within scallop P2.
• Cleft of the posterior mitral leaflet has been reported in association with
counterclockwise malrotation of the papillary muscles that may, again, lead one to
suspect a common embryological origin with AVSD

25. Echocardiographic comparison of isolated anterior mitral cleft and isolated posterior mitral cleft. (A) 2D echocardiographic
apical four-chamber view showing the eccentric mitral regurgitation of an isolated anterior mitral cleft. The regurgitation jet is
passing along the lateral wall of the left atrium. Parasternal short-axis view showing (B) mitral regurgitation in colour Doppler
mode and(C) the cleft, which looks like a slit-like hole, pointing toward the aortic root (white arrow). (D) 2D echocardiographic
apical four-chamber view showing the eccentric mitral regurgitation of an isolated posterior mitral cleft. The regurgitation jet
is passing along the atrial septum. (E and F) Three-dimensional echocardiographic views of the posterior mitral cleft
separating the posterior leaflet into two equal parts

26. 2D Echo - ICMV
• Visualization of the cleft attachments is best from a subxiphoid short-axis
view.
• ICMV with an abnormal conotruncus has more vertically positioned cleft
attachments near the LV outflow.
• Examination of ICMV is focused on determining if outflow tract
obstruction(aortic or pulmonary) by cleft
• best accomplished from modified subxiphoid windows that profile each
respective structure of interest.
• One often needs to rotate the transducer from standard long- and short-axis
views to visualize these pathways better

27. Systolic frame from a modified short-axis view obtained
from a subxiphoid window profiling an isolated cleft of
the mitral valve in a young child. From the subxiphoid
window the chordae of the cleft (white arrow )are
perpendicular to the plane of interrogation, which
provides better visualization compared with the
parasternal short-axis window.
Diastolic frame from a modified view obtained from a
subxiphoid window profiling an isolated cleft of the
mitral valve (ICMV) in an infant with D-transposition of
the great arteries {S,D,D}. Note the vertical orientation
(arrow) of the cleft attachments, typical for ICMV with
transposition or double-outlet right ventricle (see text).
The cleft attaches to the leftward aspect of the left
ventricular outflow.

28. ICMV vs AVCD
• Kohl et al. clearly demonstrated that in AVSD, the positions of both
papillary muscles were rotated counterclockwise , whereas in isolated
cleft, the position of the papillary muscles was similar to that in
normal children.
• In AVSD, the posteromedial papillary muscle is more rotated than the
anterolateral one, making it a good marker of this lesion.
• In AVSD, the cleft points towards the ventricular inlet septum,
whereas in isolated cleft, it is usually more directed towards the aortic
root

29. Spatial orientation of the cleft of atrioventricular septal defect and of the isolated cleft. (A) Photograph of an atrioventricular
septal defect. Papillary muscles are horizontalized due to a counterclockwise rotation. Because the common atrioventricular
valve is bridging over the inlet ventricular septal defect, the cleft (white star) is pointing towards the ventricular septum. (B)
Three-dimensional echocardiography of an atrioventricular septal defect showing cleft orientation towards the ventricular
septum (black arrow). (C) Photograph of an isolated cleft of the anterior leaflet of the mitral valve. The cleft (white star) is
pointing towards the left ventricular outflow tract. (D) Three-dimensional echocardiography of an isolated anterior cleft
showing its orientation (white arrow).

30. Subxiphoid short-axis sweep showing isolated cleft of the mitral valve anterior leaflet. Note the
horizontal orientation of the cleft attachments in this patient with normally related great arteries.

31. Parasternal short-axis plane demonstrating cleft attachments to the rightward aspect of the
LVOT - typical for ICMV with normally related great arteries

32. Modified view obtained from a subxiphoid window profiling an isolated cleft of the mitral
valve (ICMV) in an infant with D-transposition of the great arteries {S,D,D}. Note the vertical
orientation of the cleft attachments, typical for ICMV with transposition or double-outlet
right ventricle. The cleft attaches to the leftward aspect of the left ventricular outflow.

33. Double orifice mitral valve(DOMV)
• DOMV is defined as a single fibrous annulus with two orifices opening into the left
ventricle
• occurring in 1% of autopsied cases of CHD and is rarely isolated
• usually an ancillary finding in the setting of a more complex CHDs.
• Usually found in association with AVSD (52%), obstructive left-sided lesions (41%)
and cyanotic heart disease.
• Several cases of DOMV were also reported in association with non-compaction of
the left ventricle

34. DOMV- CLASSIFICATION
Trowitzsch et al. classified DOMV into three types
1) Incomplete bridge type - a small strand of tissue
connecting the anterior and posterior leaflets at the leaflet edge level
2) Complete bridge type - a fibrous bridge divides the
atrioventricular orifice completely from the leaflet edge all the way
through the valve annulus
3) Hole type (eccentric) - a secondary orifice with subvalvular
apparatus occurs in the lateral commissure of the mitral valve.

35. 2D Echo -DOMV
• clinical presentation is variable, mainly depending on the associated
cardiac lesion.
• MR in 43% of cases, MS in 13% and both MR & MS 6.5%,no functional
consequence of DOMV in 37% of cases.
• The two distinct orifices are clearly recognized in parasternal short-
axis view
• DOMV opens as two circles in diastole rather than a single ellipsoid
mitral orifice

36. The key to the echocardiographic diagnosis of DOMV is the visualization of two
anterograde flows through the mitral valve
• Cross-sectional views may be performed from the apex towards the base of the heart,
in order to differentiate the three types of DOMV.
• The orifices of the ‘complete bridge type’ are seen throughout the scan, while in the
‘incomplete bridge type’, the orifices are seen only at the level of the papillary
muscles. In the ‘hole type’, the smaller (accessory) orifice is seen at about the
midleaflet level.
• 3D echocardiography is efficient for accurately depicting DOMV, even in the newborn

37. Double orifice mitral valve. (A) Photograph of a double orifice mitral valve seen by the left atrium (as seen by a
surgeon), with a single fibrous orifice and (B) a double orifice mitral valve associated with partial
atrioventricular septal defect seen by the left ventricle. (C, D) 2D and 3D echocardiographic parasternal short-
axis views showing the two distinct orifices. (E) Apical 4C Doppler colour view showing two typical anterograde
flows (arrows) through the mitral valve.

38. A parasternal short-axis plane demonstrating a double-orifice mitral valve. Note the equal size of
each orifice and their location above their respective papillary muscle groups.

39. A short-axis plane in a fetus of 31 weeks’ gestation demonstrating a double-
orifice mitral valve

40. Mitral ring
• Also called supravalvar mitral ring or supramitral ring
• One of the components described by Shone et al. in Shone’s syndrome
(association of coarctation of the aorta, subaortic stenosis, PMV and
supramitral ring)
• Exceptionally isolated, this lesion is more often associated with
various other anomalies of the heart mainly VSDs and left-sided
obstructive lesions.

41. Mitral Ring
• Two types
1) The supramitral ring is a fibrous membrane originating just above
the mitral annulus, beneath the orifice of the LAA, within the
muscular atrial vestibule, not adhering to the leaflets and associated
with a normal subvalvular apparatus.
2) The intramitral ring is a thin membrane located within the funnel
created by the leaflets of the mitral valve, closely adherent to the
valve leaflets ,always combined with abnormal subvalvular apparatus.

42. Mitral Ring
• Must be distinguished from cor triatriatum sinister ,a fibromuscular membrane,
clearly separated from the mitral valve (proximal to the left atrial appendage) that
divides the left atrium into two parts.
• The ring can be either complete, circumferential or partial
• It creates a stenosis that is usually progressive with a median age at diagnosis of 36
months in the largest published series
• TTE accurately detects the mitral ring in up to 70% of cases.
• Postoperative outcome is better for supramitral ring, with no need for reoperation
after the ring excision, compared with frequent recurrence (50%) in case of
intramitral ring

43. Mitral ring. (A) Photograph showing a supramitral ring (arrows) seen from the left atrium. The membrane is originating just above the mitral annulus,
beneath the orifice of the left atrial appendage. (B) 2D 0echocardiographic PLAX view showing an intramitral ring (arrows) located within the funnel
created by the mitral leaflets and (C) Doppler colour mode showing blood flow acceleration that begins at the insertion of the membrane. (D)
Transmitral pulsed Doppler acquisition showing mitral stenosis. (E) 3D echocardiographic PLAX view showing the same intramitral ring (arrows). (F)
Three-dimensional view from the left atrium

44. Apical window demonstrating congenital mitral stenosis and a supravalvar mitral ring (SVMR). Note the prominent
papillary muscle that extends further toward the annulus than in a normal apparatus, the accompanying shortened
chordae and hypoplasia of the annulus. The membranous SVMR is seen as a thin projection from the atrial side of
the leaflet, extending from the annulus into the supravalvar flow orifice. Color Doppler in the same imaging plane
demonstrating flow acceleration beginning just prior to the annulus – a characteristic finding in SVMR, which if
present should prompt the imager to undertake an extensive search for an unrecognized SVMR. Note the
additional egress from the inflow directed medially via an abnormal intrachordal space. This type of complex inflow
orifice makes accurate measurement of the flow orifice area particularly challenging

45. Systolic frame from a TEE, mid-
esophageal four-chamber view
Note the enhanced visualization
of the supravalvar mitral
ringprovided by TEE. The
membrane is conspicuous,
extending from both
the lateral aspect of the atrial
side of the leaflet and the medial
aspect (arrow).

46. Congenital Mitral Stenosis
• Congenital mitral stenosis (MS) is defined as an abnormality at any
level of the mitral valve apparatus that results in restriction of
diastolic filling.
• Worldwide, the prevalence of acquired rheumatic mitral stenosis
exceeds that of congenital MS; however, in developed nations
congenital MS is more common.
• congenital MS typically involves disruption of several components of
the valve apparatus, hypoplasia of additional leftsided structures and
other complex cardiac lesions, which makes the determination of
specific etiology equally challenging.

47. • Four common anatomic subtypes:
- typical congenital MS
- parachute MV
- supravalvar mitral ring
- hypoplastic MS.
• By limiting flow to distal structures during embryologic development,
MS may play a role in the hypoplasia or stenosis of downstream
structures
• Typical congenital MS involves thickened and rolled leaflets,
shortened chordae tendineae with absence of the interchordal
spaces, and underdeveloped papillary muscles, which may be closely
spaced.

48. • Typical MS has also been termed “symmetric” , implying equal
distribution of chordae to each papillary muscle and equal papillary
muscle size.
• Asymmetric papillary muscle location, size and distribution of
chordal attachments is common found in approximately 30%nd in
the extreme results in a true parachute MV with all chordae
inserting into a single papillary muscle – typically the anterolateral
papillary muscle is absent
• Congenital MS is strongly associated with obstructive leftsided
lesions including PV stenosis, supravalvar and valvar AS, PMV, SVMR,
subaortic membrane and CoA – the last four lesions together were
originally described by Shone et al.

49. • Congenetal MS is best imaged in the apical and ventricular long- and
short-axis planes.
• The subxiphoid window in infants and younger children typically gives
a higher yield than the parasternal window.
• Slow, targeted sweeps of the mitral apparatus utilizing magnification
mode provide optimal visualization to differentiate subtypes of mitral
stenosis

50. A late-systolic frame in typical congenital
mitral stenosis from an apical 4C- view.
Note the thickened and short chordae
An early diastolic frame – note the rolled and
thickened leaflet tips, the reduced orifice size
and doming of the middle aspect of the
anterior leaflet A

51. Typical congenital mitral stenosis from an apical 4-chamber view. Note the thickened and short
chordae, the rolled and thickened leaflet tips, the reduced orifice size and the diastolic doming
of the middle aspect of the anterior leaflet.

52. Color Doppler of typical congenital mitral stenosis from an apical 4-chamber view. Note the
flow acceleration in the distal aspect of the inflow with the proximal isovelocity surface area
(PISA) aliased lines representing increasing velocity “shells” as the flow approaches the small
orifice

53. A short-axis plane from a subxiphoid window in typical congenital mitral stenosis. Well-spaced papillary
muscle groups are readily appreciated in this short-axis plane – excluding parachute mitral valve
morphology. Note that the orifice appears falsely adequate in the short-axis view, due to the relationship
of the distal aspect of the leaflets to the orifice – the leaflet adjacent to the orifice runs nearly parallel to
the orifice. This aspect of the anatomy is only appreciated in orthogonal views (apical 4-chamber)

54. Ebstein’s malformation of the mitral valve
• Ebstein’s malformation of the left-sided atrioventricular valve has been reported in rare
cases of corrected transposition of the great arteries
• involved valve was obviously of tricuspid morphology.
• The first case of Ebstein’s malformation of a morphological mitral valve was described in
1976 by Ruschhaupt et al.
• The malformation exclusively affects the posterior valve leaflet, which is plastered into
the left ventricle wall, thus displacing the mitral valve orifice downward into the left
ventricle.
• Unlike Ebstein’s malformation of the tricuspid valve, the atrialized inlet portion is usually
not thinned.
• This exceedingly rare anatomical condition causes mitral insufficiency

55. Anomalies of the tensor apparatus
Arcade or hammock valve
• A direct connection of the papillary muscles to the mitral leaflets, either
directly or through the interposition of unusually short chordae.
• Sometimes called hammock valve because it mimics a hammock when the
valve is observed from an atrial aspect (as seen by a surgeon).
• The tendinous cords are thickened and extremely short, reducing the
intercordal spaces and leading to an abnormal excursion of the leaflets that
may cause both stenosis and insufficiency.
• When the space between the abnormal chordae is completely obliterated, a
fibrous (muscular) bridge (band) joins the two papillary muscles.

56. Arcade or hammock valve
• In the most severe form, with no chordae tendinae at all, the papillary muscles are
directly fused with the free edge of the leaflet
• It may be seen in association with PMV.
• Believed to be the result of an arrest in the developmental stage of the mitral valve
before attenuation and lengthening of the collagenized chordae tendinae
• Echocardiographical appearance shows the short chordae and restricted motion of
the leaflets with limited coaptation but also, in Doppler colour mode, multiple jets
through the reduced interchordal spaces

57. Arcade/hammock mitral valve. (A) Photograph showing the typical aspect of a hammock mitral valve seen from the left atrium and (B) the same valve
seen from the left ventricle. (C and D) Postmortem specimens of anomalous mitral arcade characterized by fused interchordal spaces (arrows). (E) 2D-
echocardiographic view showing the obliterated interchordal spaces (arrow). (F) The typical aspect in Doppler colour mode of multiple jets through the
reduced interchordal spaces.

58. Straddling mitral valve
• SMV is defined by an abnormal attachment of the mitral chordae to both ventricles.
• Always associated with a ventricular septal defect
• According to this definition, an AVSD nearly always straddles but the term
‘straddling’ can only be applied to true mitral or tricuspid valves.
• The mitral valve always straddles through a conoventricular (misalignment) type of
VSD.
• SMV is almost always associated with conotruncal anomalies like DORV or TGA
• SMV must be distinguished from the overriding of the mitral valve, which qualifies a
mitral annulus committed to the two ventricular chambers. In that case, the mitral
valve is shared between the ventricles

59. Straddling mitral valve. (A) Photograph of a straddling mitral valve associated with a double outlet right ventricle, seen from
the right ventricle. The mitral valve is attached to the right ventricle by chordae (white arrow) that pass through the ventricular septal
defect. (B) The same mitral valve seen from the left ventricle. (C) Echocardiographic view showing the abnormal attachment (white arrow)
of the mitral valve in the right ventricle.

60. Modified long-axis view obtained from a subxiphoid window profiling major straddling of the
mitral valve in an infant with double-outlet right ventricle (DORV) {S,D,D}. Note the commissure
of the straddling valve with attachments within the infundibulum and the marked degree of
straddling that would complicate biventricular repair

61. A modified short-axis view obtained from a subxiphoid window profiling major straddling
of the mitral valve (SMV) in an infant with TGA {S,D,D} pulmonary stenosis. Note the
inferior positioned tricuspid valve (TV) inflow and the straddling via the anterior (outflow)
aspect of the ventricular septum.

62. Anomalies of the papillary muscles
Parachute mitral valve
• One of the common causes of congenital mitral stenosis, with incidence of 0.17%
• True PMV is characterized by unifocal attachment of the mitral valve chordae to a
single (or fused) papillary muscle.
• This single papillary muscle is usually centrally placed and receives all chordae from
both mitral valve leaflets.
• In PLAMV, chordae are distributed unequally between two identifiable papillary
muscles, with most or all of the chordae converging on a dominant papillary muscle.
• The dominant papillary muscle, classically posteromedial, is of normal size,
whereas the other is elongated and displaced higher in the ventricle with its tip
reaching to the annulus.

63. • In both PMV and PLAMV, the chordae are short and thickened, thus restricting the
motion of the leaflets.
• PMV or PLAMV are commonly seen in association with other obstructive lesions
affecting the left heart or conotruncal anomalies
• Mitral valve should always be carefully inspected in order to diagnose PMV if any
other feature of Shone’s syndrome is present.
• Because opening of the mitral valve is limited, true PMV is highly associated with
mitral stenosis. Mitral regurgitation occurs less commonly but must be equally
carefully followed because of its progressive evolution.

64. • In the parasternal short-axis view, a single papillary muscle is
confirmed at the mid-level of the left ventricle.
• The pathognomonic ‘pear’ shape of the mitral valve is seen in the
four-chamber view, with the left atrium forming the larger base of the
pear and the mitral leaflets the apex.
• In this view, the valve has a typical ‘domed’ appearance in diastole.

65. Parachute mitral valve. (A) Postmortem specimen of a true parachute mitral valve showing a fused papillary muscle (star).All chordae are
inserted into this single papillary muscle. (B) Photograph of a parachute-like asymmetric mitral valve. The posteromedial papillary muscle is
clearly underdeveloped. (C) 2D echocardiographic PLAX view showing a single papillary muscle connected to the leaflets by short and thickened
chordae (arrow). (D) Apical 4C view showing the pathognomonic pearshaped mitral valve. (E and F) Parasternal short-axis views showing the
typical aspects of both the single papillary muscle and the mitral valve.

66. A subxiphoid short-axis view demonstrating a parachute mitral valve. Note the presence of
a hypoplastic anterolateral papillary muscle that receives no chordae, which is the most
common arrangement in the parachute lesion

67. Morphologic anomalies of the tricuspid valve (TV)
• Tricuspid stenosis - hypoplasia and thickening of the valve
leaflets with a reduced valve orifice
• Ebstein anomaly - displacement of part of the origin of the
valve leaflets from the atrioventricular junction into the cavity of the
right ventricle
• Tricuspid dysplasia - malformed but not displaced valve
leaflets
• Double-orifice tricuspid valve - TV exhibiting two valve
orifices

68. Tricuspid stenosis (TS)
• It is a condition producing obstruction to right ventricular filling due to
abnormalities of the TV in respect of its form, annular dimension and/or
function.
• Rarely, the obstruction is found at the subvalvar or supravalvar level.
• The majority of reported cases of isolated TS are sporadic.
• TS has been reported in congenital polyvalvular disease, and is associated
with trisomy 13, 15 and 18.

69. • In the rare form of congenital isolated TS, the reduced valve orifice is
mainly due to thickening of the TV leaflets and abnormal chordal
attachments.
• Congenital TS associated with annular hypoplasia, as found in several
forms of congenital heart disease, is usually characterized by annular
hypoplasia with abnormalities of all parts of the TV including leaflets,
commissures, chordae and papillary muscles.
• Congenital supravalvular TS is caused by a membrane attached either
at the level of the tricuspid annulus or at the midportions of the
leaflets

70. • Isolated TS will cause diastolic obstruction of flow from the RA into
the RV.
• The increase of right atrial and central venous pressure will depend
on right ventricular compliance, the effective size of the TV orifice,
and the possibility of a right-to-left shunt through a PFO or an ASD.
• The pathophysiology of TS associated with other forms of congenital
heart disease, mainly right heart hypoplasia, will depend on the
underlying lesion and the size of an associated atrial septal defect.

71. Characterization of TS
• Thickened, rolled TV leaflets may also dome in diastole, further shortening
chordae with abnormal attachments;
• a stenosing membrane may be detected within the funnel of the TV thus
restricting the opening of the leaflets
• measurement of maximal velocity (Vmax) across TV: normal value <0.8 m/s;
Vmax >1.3 m/s in absence of left-to-right shunt indicates significant TS
• calculation of maximal and mean diastolic transvalvular pressure gradient.
• Assessment of tricuspid regurgitation (TR): hemodynamic assessment of TR
by spectral Doppler and color flow mapping

72. Parasternal long-axis right ventricular inflow demonstrating
tethering of both anterior and septal tricuspid valve leaflets,
annulus dilation with failure of central leaflet coaptation (arrow),
and dilation of the rightatrium (note the position of the interatrial
septum) in a child after dilation of criticalpulmonary stenosis in
infancy.
Modified parasternal short-axis view of tricuspid stenosis
associated with annular hypoplasia (arrow) and a small
ventricular septal defect leak.

73. Subcostal short-axis view
of tricuspid stenosis associated
with annular hypoplasia and
atrial septal defect. Note the
prominent Eustachian valve
(arrow)

74. Tricuspid valve dysplasia
• Tricuspid valve dysplasia is defined as a spectrum of congenital
malformations of valve leaflets, chordae and papillary muscles.
• It frequently leads to tricuspid regurgitation.
• Reported cases of TV dysplasia are sporadic, although association
with Down syndrome is reported
• Depending on its degree, TR associated with TV dysplasia will cause
progressive dilation of the right atrium and right ventricle; it may also
trigger arrhythmias.

75. • The spectrum of TV dysplasia ranges from minimal changes with mildly dysplastic,
thickened leaflets but normal chordae and papillary muscles, through short chordae
and underdeveloped papillary muscles, to severe changes including agenesis of
entire leaflets and subvalvar structures.
• In contrast to Ebstein anomaly there is no displacement of both the septal and mural
leaflets. This is important for discriminating the extreme variant of TV dysplasia –
unguarded orifice TV, exhibiting only rudimentary valve tissue at the atrioventriclar
junction – from Ebstein anomaly.
• In tricuspid dysplasia, and its extreme variant, the leaflet will be absent, not
displaced .
• In the rare case of unguarded orifice, ruling out displacement of the posterior
leaflet will confirm the diagnosis.

76. Apical 4-chamber view of
color flow mapping of
tricuspid regurgitation due
to tricuspid dysplasia.

77. Apical 4-chamber view demonstrating tricuspid valve dysplasia. Note the normal insertion of
the septal tricuspid leaflet at the cardiac crux and the lack of central valve leaflet coaptation.

78. Double-orifice tricuspid valve (DOTV)
• Double-orifice tricuspid valve (DOTV) is defined as an anomalous tricuspid
valve exhibiting two orifices. It is also termed “duplication of the TV’’
• Is an extremely rare anomaly and is reported to be associated with
atrioventricular septal defect, tetralogy of Fallot and several TV anomalies
such as Ebstein anomaly, tricuspid dysplasia, TV prolapse and straddling
TV
• The etiology of DOTV is not known.
• DOTV is considered as benign, and its pathophysiology is determined by
associated lesions.

79. • In DOTV, 2D echocardiography can demonstrate duplication of the
ostium on parasternal short-axis, apical 4- chamber and parasternal
long-axis views through the right ventricular inflow tract.
• Imaging of the two orifices can best be achieved from subcostal short-
axis and en face views.

80. 2D echo with subcostal
short axis view (enface view)
showing double orifice of
right sided component of
the common AV valve

81. Anomalies of tricuspid valve alignment
• The tricuspid valve is termed as straddling when its chordae tendineae and
papillary muscles are attached on both sides of the ventricular septum so
that the right atrium may empty into both ventricles.
• It is termed as overriding when its annulus is connected to both ventricles.
• The degree of overriding will determine whether this connection is truly
biventricular (override <50%), with two ventricles of comparable size
present, or whether this connection is univentricular, with one dominant
and one rudimentary ventricle (override >50%)
• Straddling and overriding may coexist

82. • The exact incidence of straddling/overriding TV per se is not known.
• The condition of a straddling TV is of importance in univentricular
hearts, but crucial in decision-making for biventricular repair as it may
be present in 3% of lesions as VSD, tetralogy of Fallot, DORV , and
transposition of the great arteries.
• Although certainly underdiagnosed in univentricular hearts, straddling
TV is a rare condition, found in only 0.71% of autopsies in the Cardiac
Registry of the Children’s Hospital in Boston

83. • In straddling TV in biventricular hearts, there is marked malalignment
between the ventricular and the atrial septum.
• The right ventricular inflow tract is usually smaller than the left ventricular
inflow tract.
• The nonstraddling part of the TV opens into the right ventricle, whereas the
straddling part opens into the left ventricle.
• In order to avoid surgically induced heart block, it is important to know that
the position of the penetrating atrioventricular bundle reflects the degree of
malalignment
• The great arteries were either normally related (42%, complicated in the
majority by TOF ) or abnormally related (58%, TGA, DORV, or doubleoutlet
left ventricle)

84. Apical 4c view of a straddling and overriding
tricuspid valve (TV), mild hypoplasia of right
ventricular inflowtract, and inlet VSD.
Apical 4C view of a perimembranous/inlet
VSD, malalignment of IAS and IVS , over
riding tricuspid valve with high offsetting
(arrow) and smallish RV inflow.

85. THANK YOU