brief details of av canal defect

1. Presenter : Dr. Sudip Dutta Baruah
SRD, CVTS

2. DEFINITION
Deficiency or absence of septal tissue
immediately above and below the normal level
of the AV valves, including the region normally
occupied by the AV septum, in hearts with two
ventricles
The AV valves are abnormal to a varying
degree

3. HISTORY
Abbott: Recognized OPASD and CAVCD
Rogers and Edwards;1948
Wakai and Edwards in 1956 & 1958 : PAVCD &
CAVCD introduced
Lev: Described the position of the AV node and
bundle of His
Wakai, Edwards and later Bharati & Lev coined
intermediate and transitional AVCD

4. HISTORY
Rastelli; 1966 : Morphology of AV valve
leaflets in common AV orifice
Ugarte; 1976 : Idea of leaflets bridging the
ventricular septum
Baron, Van Mierop and colleagues :
Recognized in late 1960s that the basic defect
is absence of the AV septum
Piccoli and colleagues under the direction of
Anderson emphasized all the variations of the
defect were part of a spectrum

5. HISTORY
Dennis and Varco; 1952 : At the University of
Minnesota Hospital in Minneapolis
Lillehei;1954 : Using cross-circulation and direct
suture of the atrial rim of the septal defect to the
crest of the ventricular septum
Kirklin; 1954 : Repaired PAVCD through the atrial
well of Watkins and Gross
1955 : Repair AVCD by open cardiotomy and pump-oxygenator
Dubost and Blondeau; 1959 : Cleft in “mitral leaflet”
need not be sutured in PAVCD
Maloney & Gerbode; 1962 : Single patch technique

6. HISTORY
McGoon recognized the importance of “taking
from the tricuspid valve” to leave sufficient tissue
from which to create an adequate left AV valve
1968 and 1971: Barratt-Boyes successfully
repaired in severely ill
George Trusler; 1975 : Two patch technique
In 1978, Carpentier emphasized that left AV
valve functions best when repaired as a three-leaflet
valve
Graham Nunn : Modified single patch technique

7. MORPHOLOGY
DEFICIENCY/ABSENCE OF THE AV SEPTUM
OPASD immediately above the AV valves
And
Deficiency (or scooped-out area) in the
inlet (basal) portion of the ventricular
septum immediately below the AV
valves

8. PAVCD
OPASD ( Crescent shaped defect in inferior
portion of atrial septum immediately
adjacent to AV valve)
Cleft in MV leading to varying degree of MR

9. CAVCD
OPASD
Inlet VSD
Common AV valve bridging both sides of
heart creating Superior ( anterior) &
Inferior (posterior ) leaflets
BARE AREA at crest of ventricular septum

10. INTERMEDIATE AVCD
OPASD
Inlet VSD
Two distinct AV valves
Dense chordal attachment to ventricular
septum
NO BARE AREA at crest of ventricular septum

11. RASTELLI CLASSIFICATION
BASED ON
Morphology of Anterior bridging leaflet
Degree of bridging and its chordal
attachment
DONOT RELATE TO ANATOMY OF INFERIOR
BRIDGING LEAFLET

12. RASTELLI CLASSIFICATION
TYPE A : The common superior bridging leaflet is effectively
split into 2 at septum
TYPE B : Rare; involves anamolous papillary muscle
attachment from right side of ventricular septum to the left
side of common superior bridging leaflet
TYPE C : Marked bridiging of ventricular septum by superior
bridging leaflet. The superior leaflet is generally not divided &
floats without chordal attachment to the crest of ventricular
septum
The posterior common leaflet may be divided or
undivided but nearly is always well attached

13. RASTELLI CLASSIFICATION

14. MORPHOLOGY
General Morphologic Characteristics
PAVCD : Normal length of atrial septum, OPASD
result of absence of the relatively small AV septum
plus some deficiency in the inlet portion of the
ventricular
Septal deficiencies may or may not result in
interatrial or interventricular communications,
depending on configuration and attachments of the
AV valves
Basic defect in these malformations is absence of the AV septum,
whether the ventricular septal or atrial septal deficiency or the AV
valve abnormality is the result only of AV septal absence is still
debated

15. MORPHOLOGY
General Morphologic Characteristics
Five or more AV valve leaflets of variable
size are usually present
Variability in completeness of commissures
and prominent crenations in the leaflets
CAVCDs most common number of leaflets
was 5
The left superior leaflet (LSL) and left
inferior leaflet (LIL) are variable in size,
connections one to another and degree of
bridging across the crest of the ventricular
septum
There may be one or two AV valve orifices

16. MORPHOLOGY
General Morphologic Characteristics
Absence of usual wedged position of the aortic valve above
the AV valves. Instead, it is elevated and deviated anteriorly
Left ventricular (LV) inflow tract shortened in relation to
outflow portion, and there is related reduction in length of
the diaphragmatic wall of the LV
The LV outflow tract is also narrowed; rarely sufficient to be
of hemodynamic importance
Large variety of major and minor associated cardiac
anomalies
Down syndrome is common, particularly in patients with an
interventricular communication

17. Anomaly No. % of 310
None MORPHOLOGY
237 76
Patent ductus arteriosus 31 10.0
Tetralogy of Fallot 20 6.5
General Morphologic Characteristics
Completely unroofed
coronary sinus with left SVC
9 2.9
Situs ambiguus 7 2.3
DORV without PS 6 1.9
Additional VSDs 5 1.6
DORV + PS 3 1.0
Situs inversus totalis 3 1.0
TAPVC 2 0.6
Left ventricular outflow
2 0.6
obstruction
Transposition of the great
arteries
1 0.3
PS, supravalvar mitral
stenosis, Ebstein
malformation, coarctation,
isolated dextrocardia
1 each 0.3

18. MORPHOLOGY
Without
Interventricul
ar
Communicatio
n
(n = 154)
With
Interventricul
ar
Communicatio
n
(n = 156)
Anomaly No. % of 154 No.
% of
156
(Sizable) ASDs 17 11 32 21
Left SVC without unroofed coronary sinus 10 6 7 4
Partially unroofed coronary sinus 5 3 2 1
Azygos extension of IVC 4 3 3 1
IVC to lower left common atrium 1 1
Bilateral IVCs 1 1
TASVC to common atrium 1 1
Right PVs to RA 1 1
Anomalous origin LAD from RCA (TF) 1 1
Origin stenosis LPA (not TF) 1 1
Wolff-Parkinson-White syndrome 1 1
Spontaneous heart block 1 1
Coronary artery disease requiring CABG 1 1

19. Atrial Septal Deficiency and
Interatrial Communications
Partial Atrioventricular Septal Defect
OPASD : Bounded below by the inferiorly displaced
AV valve leaflets and above by a crescentic ridge of
atrial septum that fuses with the AV valve anulus
only at its extremities
Little atrial septal tissue at the superior border with
valve anulus adjacent to aorta, more tissue is
usually present inferiorly adjacent to the coronary
sinus
ostium primum defect is moderate size

20. MORPHOLOGY
Left ventricular outflow
view. LS and LI leaflets are
firmly attached to crest of
ventricular septum.
Narrowing and elongation
of left ventricular outflow
tract are apparent.

21. MORPHOLOGY
Right atrial view of a
specimen of a partial
atrioventricular (AV) septal
defect. Coronary sinus
ostium (CoS) is seen inferior
and posterior to ostium
primum (P) defect in atrial
septum. Approximate position
of AV node and bundle of His
is shown as a dashed line.
Placement of inferior part of
patch suture line is shown by
the line of x's

22. Atrial Septal Deficiency and
Interatrial Communications
Partial Atrioventricular Septal Defect
OPASD : Fusion of base of the LSL or LIL
to edge of adjacent portion of atrial
septum
Rarely, accessory “parachute” of fibrous
tissue
Under such circumstances, a pressure difference exists
between the two atria

23. Atrial Septal Deficiency and
Interatrial Communications
Common Atrium
Absence of Interatrial Communication
Rarely, AV valve tissue attached completely to edge of
the atrial septum, and no interatrial communication
exists
In this variant, characteristic deficiency of inlet (basal)
portion of ventricular septum is present with a large
interventricular communication beneath the leaflets
The functional left AV valve, consist of portions of the
LSL and LIL on the left side of their attachment to the
atrial septum, tends to be competent

24. Ventricular Septal Deficiency and
InterventricularCommunications
Partial Atrioventricular Septal Defect
Deficiency of inlet portion of ventricular septum
immediately beneath AV valves is constant
Inlet portion of ventricular septum is shortened
usually no interventricular communication

25. MORPHOLOGY
Partial AV septal defect viewed from opened left ventricle. Left superior (LS) and left
inferior (LI) leaflets are completely attached to crest of a deficient ventricular septum (VS).
Area of contact or closure between left superior and left inferior leaflets is indicated by
arrow B, Intermediate type of AV septal defect from left ventricular view. Numerous small
interventricular communications are present between thick, short chordae that tether both
LS and LI leaflets to ventricular crest.

26. Ventricular Septal Deficiency and
InterventricularCommunications
Complete Atrioventricular Septal Defect
LSL and LIL are separate with large interventricular
The communication particularly large beneath the
LSL and smaller beneath the but in 5% larger VSD
beneath LSL and none beneath the LIL
Rarely, there is no VSD beneath the LSL and a large
one beneath the LIL

27. MORPHOLOGY
From left ventricular
aspect. LS and LI
bridging leaflets are
free floating, and
there is a large
interventricular
communication
between them and
the underlying crest
of the ventricular
septum.

28. AV VALVES
Attachments of AV valves to crest of
ventricular septum in PAVCD & chordal
attachments in CAVCD are displaced toward
the apex due to deficiency of inlet (basal)
portion of septum
Alters orientation of the AV orifices relative
to the aortic orifice
provides an important diagnostic imaging criterion

29. AV VALVES
Two Atrioventricular Valve Orifices
The LSL and LIL are joined together to
variable extent anteriorly by leaflet tissue
near the crest of the ventricular septum
Together they resemble AML with a cleft
Connection between the LSL and LIL may be
thin strand of tissue (complete cleft), but
commonly it is 2 to 4 mm

30. AV VALVES
Two Atrioventricular Valve Orifices
Difference between commissure & cleft
supported by chordal apparatus on either side of the
gap and a cleft that is relatively unsupported and
bereft of chordae at its edges
In addition, chordae originating from the central
edges of the LSL and LIL attach to different
papillary muscles, which can cause a distracting
force on the leaflets during closure
( Contrasts with the normal commissure in which the chordae from
adjacent leaflet edges attach to a single papillary muscle,
encouraging coaptation )

31. AV VALVES
Two Atrioventricular Valve Orifices & regurgitation
When LSL and LIL are nearly completely separated,
appreciable gap during systole, producing
regurgitation
Failure of valve coaptation at this site, leaflet tissue
forming the margin usually becomes thickened and
rolled
In other cases, regurgitation appears to be due to
deficiency of leaflet tissue, particularly in the LIL
The mechanism of severe left AV valve regurgitation
is, however, not evident in some cases
The jet of regurgitation usually directed into RA
Rarely, the left AV valve is stenotic, but this usually is
associated with hypoplasia of the LV

32. AV VALVES
Two Atrioventricular Valve Orifices
RAVV also abnormal
It may consist of three leaflets—right superior
leaflet (RSL), right lateral leaflet (RLL), and right
inferior leaflet (RIL)—or of two or four
Leaflet tissue attached directly or by chordae to the
crest or right side of crest of septum
abnormalities of right AV valve, regurgitation is rare
(unless right heart failure develops)

33. AV VALVES
Common Atrioventricular Orifice
Common orifice with large VSD (CAVCD), the LSL
and LIL are separate, and a bare area is exposed on
the crest of the ventricular septum
The LSL may be entirely on the LV side of the
septum or may, to a variable degree, bridge the
septum and extend onto the right ventricular side
This variability formed the basis for the classification by
Rastelli and colleagues into types A, B, and C

34. AV VALVES
Complete AV septal defect viewed from left ventricular aspect.
LS and LI bridging leaflets are free floating, and there is a large
interventricular communication between them and the
underlying crest of the ventricular septum

35. AV VALVES
Common Atrioventricular Orifice
Chordal attachments of the right ventricular extremity of
the LSL vary according to degree of bridging
No bridging :Chordal attachments to ventricular crest
Mild bridging :To the medial papillary muscle in the right
ventricle
Moderate bridging :To accessory (often large) apical papillary
muscle
Marked bridging :To normally positioned (although often bifid)
anterolateral papillary muscle of RV

36. Complete atrioventricular septal defects with
varying degrees of bridging of left
superior (LS) leaflet
A, Nonbridging (bridging grade 0) LS leaflet (Rastelli type
A). This surgical specimen (the patch having been
removed) is viewed from right atrium. Arrow marks mildly
bridging left inferior (LI) leaflet
B, Moderate (grade 2 or 3) bridging of LS leaflet. Chordae
from its right ventricular extremity go to a papillary
muscle in right ventricle. Arrow indicates bridging portion
of LI leaflet. (Rastelli and colleagues termed this type B,
but it is just part of the spectrum of bridging.)
C, Marked (grade 5) bridging of LS leaflet (Rastelli type
C). Arrow marks bridging part of LI leaflet

37. AV VALVES
Common Atrioventricular Orifice
When LSL bridges septum moderately or
markedly and extends into RV, it usually is
free-floating
LSL may occasionally attached by chordae
(tethered)
Length of chordal/ fibrous attachments to
right side or crest varies according to size
of VSD or position of leaflet

38. AV VALVES
Common Atrioventricular Orifice
LIL typically bridges moderately, but varies
Not uncommon for bridging LIL to be attached to
ventricular crest
Chordal attachments of leftward components of common
AV valve in LV are usually relatively normal although
posterior papillary muscle is displaced laterally than normal
and a third papillary muscle may be present
There may be only one papillary muscle, producing a
parachute-type valve that is difficult to repair (13% cases )

39. AV VALVES
Common Atrioventricular Orifice
RV portion of common AV valve has superior, lateral, and inferior
leaflets, but vary considerably in number and size
With bridging; LSL becomes smaller
When leaflets of the common AV valve close appropriately during
ventricular systole, AV valve regurgitation is absent or mild
Kanani and colleagues emphasized on marked valvar
abnormalities, not only of annular component but also of
subvalvar apparatus (with deficiency of chordal arrangement) and
leaflet tissue (which is often deficient in coaptation surface and
pliability following repair).
The mechanism of the regurgitation is often not clearly understood

40. AV VALVES
Unusual Atrioventricular Combinations
Rarely, connected only by a fibrous strand
adherent to the ventricular septal crest forming a
“pseudomitral leaflet,” rather than an “anterior
mitral leaflet with complete cleft
1% cases, the connected LSL and LIL have large
interventricular communications beneath them &
the connection is a thin strand of valve tissue
beneath which there is also a large
interventricular communication but two AV valve
orifices can be said to be present

41. AV VALVES
Accessory Orifice
Double left AV valve orifice present in the
commissure on one side, usually on inferior side of
LLL in 5% of cases
A ring of chordae surrounds the orifice, and small
papillary muscle is usually beneath it
The accessory orifice conceptualized as an
incomplete commissure, and fibrous tissue “bridge”
between accessory and main orifice consists of
valvar tissue and chordae
LLL is often underdeveloped
Danger of producing regurgitation by cutting the bridge &
Accessory orifices predispose patients to stenosis after
repair

42. AV VALVES
Single Papillary Muscle
Single papillary muscle in LV (5% of cases)
commonly in complete type
All chordae inserts into single papillary
muscle, which is situated anteriorly in LV
CAVCD with free-floating and bridging LSL, no LV
inflow obstruction results. Otherwise, or after repair,
the situation is entirely analogous to a true “parachute
mitral valve” and inflow obstruction can complicate
intracardiac repair

43. VENTRICLES
LV outflow tract elongated and narrowed
AVCD with large VSD, the LV may be abnormally
large
Right-dominant type AVCD, LV is severely
hypoplastic
Atrial septum displaced leftward in relation to the
plane of the ventricular septum & overrides LAVV
Associated hypoplasia of LA

44. VENTRICLES
RV no specific anomalies
Enlarged secondary to the left-to-right shunt
LV or RV hypoplastic in 7% of patients
Presence of severe ventricular hypoplasia can increase
risk of surgical correction and may demand a Fontan-type
repair, alone or with a technique for correcting the
hypoplastic left heart physiology

45. VENTRICLES
Left ventricular aspect of a
complete atrioventricular
septal defect with no bridging
of left superior (LS) leaflet and
connection of leaflet to
underlying ventricular septum
by long chordae. Narrowness
of left ventricular outflow tract
is apparent

46. SEPTAL MALALIGMENT
Hypoplastic LV; Ventricular septum is
malaligned and lies more to the side of the
hypoplastic ventricle
Less commonly, atrial septal remnant is
malaligned and is leftward
When severe, both AV valves (or common
AV valve orifice) are accessible only from
right atrium & blood exists from left atrium
only through the ostium primum defect
(double outlet right atrium)

47. Left Ventricular Outflow or Inflow
Obstruction
Rarely in unoperated hearts (1% cases) & apparent as
postoperative complication
CAUSE :
I. Due to extensive area of direct fibrous continuity between
aortic valve and the LSL
II. Short, thick chordae that anchors LSL to crest
III. Anterolateral muscle bundle of LV bulges more into LV outflow
tract
IV. Morphologically discrete subaortic stenosis or excrescences of
AV valve tissue heaped up in LVOT
V. Abnormally positioned papillary muscles
VI. Simple narrowing of AV valve entrance into LV
VII. Related to presence of accessory AV valve orifice on left side,
or from cor triatriatum or supravalvar fibrous ring
VIII. Associated cardiac anomalies more prevalent in Down
syndrome

48. Conduction System
Defect in AV septum displaces coronary sinus ostium
inferiorly (appear to lie in LA)
AV node displaced inferiorly (caudally) and lies in
posterior RA wall between the orifice of the coronary
sinus and ventricular crest (Nodal triangle )
Bundle of His passes forward and superiorly from
node to crest, reaching it where crest fuses
posteriorly with AV valve anulus
Then courses along top of VS beneath bridging
portion of LIL & giving LBB
At midpoint of crest of VS, it becomes RBB which
continues along crest a little farther before it
descends towards muscle of Lancisi and moderator
band

49. CONDUCTION SYSTEM

50. Major Associated Cardiac Anomalies
Patent Ductus Arteriosus 10% cases particularly with an interventricular
communication
Tetralogy of Fallot
I. 5% of patients & 1% of patients TOF has CAVCD
II. LSL bridges markedly and is free-floating over the crest & interventricular
communication beneath it is large and juxtaaortic
III. RVOT typical tetralogy morphology
Double Outlet Right Ventricle
DORV without PS in 2% of cases
I. VSD large and juxtaaortic / noncommitted
II. Rarely, Taussig-Bing type
DORV with PS 1% of cases
I. Frequently have atrial isomerism or situs inversus, common atrium, completely
unroofed coronary sinus with left superior vena cava, azygos extension of the inferior
vena cava, or total anomalous pulmonary venous connection
Transposition of the Great Arteries Rare
Completely Unroofed Coronary Sinus with Left Superior Vena Cava
I. 3% of patients with interventricular communication & 3% without
II. More frequent with CA

51. Minor Associated Cardiac Anomalies
Pulmonary Vascular Disease
I. CAVCD appears early in life and progresses
II. Progress rapidly in patients with complete AV
septal defects
III. More frequent & occur earlier with Down
Down Syndrome
I. Rare in PAVCD but common (75%) with CAVCD
II. Left-sided obstructive lesions 10 times less
common

52. Clinical Features and Diagnostic Criteria

53. PATHOPHYSIOLOGY
 Left-to-right shunting present unless severe pulmonary
vascular disease
 No interventricular communication
 Shunt at atrial level and usually large
 Large shunt with mild LAVVR, hemodynamics of ASD
 Important LAVVR, left-to-right shunt much larger; in
fact, regurgitation jet usually goes directly from LV to RA
 Left & right ventricular stroke volume increased, and
marked cardiomegaly and heart failure in early life

54. PATHOPHYSIOLOGY
 Large interventricular communication
 Large left-to-right shunt
 RV & PA pressures approach/ equal systemic
pressures
 Pulmonary vascular resistance rises rapidly
importantly elevated after age 6 to 12 months
 AVVR adds to ventricular volume overload
 Overload usually seems to enlarge RV more

55. PATHOPHYSIOLOGY
AVVR
10% to 15% PAVCD have important regurgitation
Moderate regurgitation in 20% CAVCD
Severe regurgitation in 15% CAVCD
More common in older patients
Site of regurgitation : Gap between the LSL and LIL
near leaflet hinge or base
Regurgitant flow directly to RA
If interatrial communication smaller or regurgitation
is sited elsewhere, regurgitation enter left atrium
Precise mechanism of AV valve regurgitation is often
unclear

56. Symptoms & Physical Findings
PAVCD With Mild AVVR
I. First decade of life
II. Clinical presentation of OSASD + apical systolic murmur + left
axis deviation
PAVCD With Moderate 2 Severe AVVR
I. Symptoms earlier
II. Progressive severe heart failure requiring treatment in infancy
III.Tachypnea and hepatomegaly
IV. Usual signs of ASD + loud apical pansystolic murmur + palpable LV
apex

57. Symptoms & Physical Findings
CAVCD
I. Presentation in the first year of life
II. Progressive severe heart failure
III. Tachypnea, poor peripheral perfusion, and failure to
thrive
IV. severe hypertensive pulmonary vascular disease and
Eisenmenger complex
V. cardiomegaly with increased ventricular activity
VI. Fixed & wide split second heart sound with
accentuation of P2 by elevated pulmonary artery
pressure
VII. Systolic murmur over left precordium from the shunt
at ventricular level and with increased intensity nearer
apex when there is important AV valve regurgitation
VIII. Mid-diastolic flow murmur widely heard both over the
lower left precordium and at the apex

58. Chest Radiograph
Without VSD or LAVVR : Same as in other large
ASDs
Moderate or severe LAVVR :
I. Marked cardiomegaly with evidence of LV, right
ventricular, and right atrial enlargement +
pulmonary plethora
II. LAE not apparent unless ostium primum defect is
restrictive
CAVCD :
I. Cardiomegaly + pulmonary plethora in
presentation with heart failure
II. Increased pulmonary vascular resistance with heart
less enlarged + central pulmonary arteries large +
clear lung fields

59. ECG
Biventricular hypertrophy
Prolong PR interval
Left axis deviation

60. ECHOCARDIOGRAPHY
2D-echo with Doppler color flow imaging provide full
information for CAVCD
Four-chamber view
I. AV orifice easily seen
II. elongated outflow septum and unwedged position of
aortic valve identifiable
III. Chordal attachment and degree of leaflet bridging
can be assessed
Subcostal view : Degree of balance
Color Doppler : Degree of AV valve regurgitation is
assessed with.

61. E: CAVCD as viewed from the apex (apical
four-chamber view). Arrows point to the
common AV valve. There are both primum
and secundum atrial septal
defects. F: PAVCD(apical four-chamber view).
Thin arrows point to common AV valve. Thick
arrow points to tissue occluding the inlet
ventricular septal defect right AV valve pouch
formation C: Parasternal long axis view
showing a “gooseneck” appearance of LVOT
caused by displacement of left-sided portion
of a common AV valve (arrow).

62. Cardiac Catheterization and
Cineangiogram
required only when major cardiac anomalies coexist and when operability is
questioned because of evidence of pulmonary vascular disease
 Direction & magnitude of shunting; pulmonary and systemic
pressures, resistances, and flows; and right and left ventricular
pressures can be measured
 Angiocardiographic features :
I. Absence of AV septum and deficiency of inlet portion of VS
II. Elongation of LVOT
III. Elevation and anterior displacement of aortic valve vis-à-vis the AV
valves
IV. Anomalous relationship of anterior components of LAVV to aorta
V. Anomalous LAVV’s relationship to aorta results in change in direction
of left AV valve movement
VI. Interatrial and interventricular shunting demonstrated, as can
presence of one or two AV valve orifices
VII. High-quality studies shows leaflets of LAVV visualized in motion to
delineate the degree, location, and mechanism of valvar regurgitation

63. Complete atrioventricular (AV) septal defect shown by left
ventriculogram in four-chamber view. Anulus of valve is seen as a
negative shadow formed by accumulation of contrast medium
between the leaflets and ventricular free wall (arrowheads)

64. Natural History
Depends On Morphologic And Functional Details Of Their Malformations
14% children of mothers with AVCD have congenital heart disease; half have
tetralogy of Fallot, and half have AV septal defects. This prevalence is much
higher than the 2% to 4% among children of parents with other types of
congenital heart disease
PAVCD + Mild AVVR + no major associated cardiac anomaly
I. Similar to OSASD
II. Pul vascular disease in small number of patients in their
20s, 30s, and 40s
III. Symptomatic deterioration in adult life coincides with
development of atrial fibrillation

65. Natural History
PAVCD + Mod- Sev AVVR
I. 20% severely symptomatic in infancy, and
without surgical treatment die in the first
decade
CAVCD
I. Ideal database for delineation of natural
history does not exist
II. Severe pulmonary vascular disease :Apparent
at 7 to 12 months of age in 30% & 90% of
patients by age 3 to 5 years

66. Indication For Operation
Presence indicate operation
PAVCD :
optimal age for operation is 1 to 2 years, assuming
no AVVR
If AVVR or heart failure, earlier operation
CAVCD :
early in the first year of life
general condition is good, repair can be delayed
until about age 3 to 6 months

67. Technique of operations
PRINCIPLES OF OPERATION
1. Closing interatrial communication
2. Closing interventricular communication
3. Avoiding damage to AV node and bundle
of His
4. Maintaining or creating two competent,
nonstenotic AV valves

68. Repair of CAVCD : Two Patch
technique

69. Repair of CAVCD : Single Patch Technique
Differs from the two-patch technique in the following
ways:
I. Patch is always pericardium
II. Tailoring the waist of the patch (at the
level of the AV valves) is critical
III. (3) Both LSL and RSL and LIL and RIL
are sutured to the patch

70. Repair of CAVCD : Single Patch Technique

71. Repair of CAVCD : Modified Single Patch
Technique
1997, Wilcox : Direct suturing of AV valves to the ventricular
with a small ventricular component
Advantages
I. Simplicity by avoiding a separate patch for
VSD closure
II. No division of valve leaflets or chordae
III. Reduced operative time

72. Repair of CAVCD : Modified Single Patch
Technique

73. Repair of PAVCD

74. Repair of CAVCD/PAVCD with mod- sev
LAVVR
Primary determinant of LAVVR when
preoperative AV valve regurgitation is moderate
or less
Accurate sizing of ventricular patch to
produce an anuloplasty effect (width
somewhat less than combined width of
LSL and LIL)
Avoiding elevation by patch of LSL and
LIL above their pre-repair level

75. Repair of CAVCD/PAVCD with mod- sev
LAVVR
If patch and its placement are considered optimal, the basic options
depend on observations about location and mechanism of
regurgitation by filling the LV with saline:
I. Leakage between LSL and LIL, “cleft” is partially or
completely closed with interrupted 5-0 polypropylene or
braided sutures, which may be reinforced
II. Leakage central or at commissure between LLL and LSL or
between LLL and LIL, anuloplasty sutures (with or without
pledgets) are placed
III. Marked central leakage and an enlarged orifice, a more
extensive anuloplasty of the LLL and its commissures using a
polytetrafluoroethylene band
IV. Leakage through accessory clefts, they may be partially
closed
V. If repair is completed and leakage is trivial by saline injection,
remaining orifice is sized with Hegar dilators (z value of −2 or
greater adequate to avoid valve stenosis )

76. Repair of CAVCD/PAVCD with mod- sev
LAVVR
Persistent moderate to severe residual regurgitation
(particularly in the reoperative setting)

77. Replacement of LAVV
Severe left AV valve regurgitation cannot be repaired, or when it persists or develops
postoperatively
 Replacement may accentuate or produce subaortic stenosis --
attaching rectangular piece of polyester to anulus of LAVV in
subaortic area - prosthesis sutured to artificial mitral-aortic
anulus and to natural anulus for the rest of its circumference
 Minimize damage to AV node and bundle, care taken anteriorly and
inferiorly to sew only to fringe of left AV valve that has been
preserved
 Prosthesis attached in supraanular position using atrial wall to base
the sewing ring
When possible, valve replacement should be deferred until
childhood

78. Postoperative Care
I. TEE or TTE
II. LAP more than 6 mmHg higher than RAP raises
possibility of severe LAVVR or stenosis( can result
simply from small size and low compliance of LV )
III. prophylaxis is taken against pulmonary
hypertensive crises
IV. important residual LAVR suspected—repeat
echocardiographic study in ICU
V. If results inconclusive, left ventriculographic
VI. If severe regurgitation is demonstrated,
reoperation is indicated. Likewise, if the patient's
condition is unsatisfactory and a large residual
left-to-right shunt is present, reoperation is
indicated
LAV repair failure predisposes to death within first year after operation,
consideration should be given to early reoperation

79. Survival
Early (Hospital) Death
I. Hospital mortality after repair of PAVCD 1%
II. Complete AVCD with balanced ventricles
undergoing repair in first 3 to 6 months is 3%
III. Overall operative mortality 14% and did not
differ between Down and non-Down
IV. CAVCD with major cardiac anomalies such as
tetralogy of Fallot, hospital mortality remains
generally low
Late outcomes
Generally good,15-year survival of 80% to 90%

80. Incremental risk factor for premature deaths
Incremental risk factor for pHrazeamrda Pthuasree deaths
Risk Factor
Early and
Decreasing
Con
stan
t
Demographic Variables
Prematurity •
Younger age •
Clinical Variables
Higher NYHA class • •
Greater severity of preoperative AV valve
•a •
regurgitation
Morphologic Variables
Single papillary muscle •
Accessory valve orifice •
Major associated cardiac anomalies •
Severe LV hypoplasia • •

81. Incremental risk factor for premature
deaths
Hazard Phase
Risk Factor
Early and
Decreasing
Cons
tant
Surgical Variables
Earlier date of operation •
Interaction with age •
Postoperative Variables
Severe postoperative left AV valve regurgitation • •
Absence of sinus rhythm •
Higher left atrial, right atrial, or pulmonary artery pressure •
Reoperation Variables
For pacemaker •
For VSD •
For left AV valve regurgitation •

82. Results
Heart Block and Other Arrhythmias
Surgically induced permanent complete
heart block 1%
First-degree AV block, present in about
30% preoperatively & 50% after repair
Right bundle branch block is common
after repair

83. Results
Functional Status
88% of surviving patients in NYHA
functional class I, and 11% were in
class II

84. Results
AV Valve Function
30% to 40% have no LAVVR
PAVCD 10% severe LAVVR
Worse postoperative LAVVR common in children repaired
after 4 years
10% to 20% of infants and older patients have
important regurgitation late postoperatively
Repair of uncomplicated partial and complete AV septal
defects uncommonly results in stenosis of the LAVV

85. Results
LVOTO
5% cases
LVOTO associated with LAVVR; its relief
usually permits regression of regurgitation
Simple resection rarely suffices
Extensive transaortic myectomy or modified
Konno operation, without aortic valve
replacement indicated

86. Results
Residual PAH
Timely operation usually younger than
age 6 months

87. THANK
YOU