My presentation

1. Dr.U.K.M.Nazmun Ara
Resident (Phase – B)
Paediatric Cardiology
BSMMU, Dhaka.
14/06/2016

2. Congenital cardiac malformations that lack
two completely well developed ventricles,
and in which functionally there is only a
single ventricular chamber that supports
both pulmonary and systemic circulations

3. Various terms used to describe these hearts
include:
single ventricle,
univentricular heart,
double inlet ventricle,
univentricular atrioventricular connection,
common ventricle,
functionally single ventricle

4. As early as 1699, Chemineau described a
heart composed of two auricles, but only one
ventricle.
In the late 1970s and early 1980s, Anderson
et al. used the term univentricular heart.
Many other authors continued preferred
terms such as double inlet LV and tricuspid
atresia.
In 1984, Anderson et al. introduced the term
“univentricular AV connection” to describe
hearts in which both inlets (whether patent or
not) were committed primarily to one
dominant ventricle.

5. The final concensus of the STS congenital heart surgery
database committee and the European Association for
Cardiovascular Surgery includes single ventricle hearts
are
1. Hearts with double inlet atrioventricular connection(
DILV, DIRV)
2. Hearts with absence of one atrioventricular connection(
Tricuspid atresia, mitral atresia)
3. Hearts with a common atrioventricular valve(
unbalanced complete AV canal defect)
4. Hearts with only one fully well developed ventricle and
heterotaxia syndrome ( single ventricle heterotaxia
syndrome)


6. Despite the HLHS is a common form of
univentricular heart but the current
database proposal includes it in an
separate section.
Some other congenital heart diseases
which is used to treat univentricular heart
are---
Pulmonary atresia with intact IVS, some
complex forms of DORV etc.

7.  The embryology of single ventricle in
humans is still unknown.
Presumably, both ventricular septation and
movement of the common atrioventricular
orifice are disrupted.
It is likely that many genetic alterations can
result in a single ventricle phenotype.

8. Incidence - 54 cases per million live births
by a New England registry in 1980.
Recent estimates are still higher.

9. Autopsy of 60 univentricular hearts excluding
tricuspid and mitral atresia—
Prevalence of common types of single
ventricles

10. Ventricular Morphology
Atrioventricular Connections
Ventricular Arterial Connections

11. A. Double inlet LV with a hypoplastic right
ventricle -
The most common form.
In this anomaly, the rudimentary right
ventricle is located anterior and superior to
the dominant left ventricular chamber and
most commonly connects to the ascending
aorta (ventriculoarterial discordance).

12. 

13. Sidedness - If the hypoplastic right
ventricle lies along the right shoulder of the
heart, the sidedness of the dominant LV
will be normal, and if it is found on the left
shoulder of the heart, then the left
ventricular sidedness will be the mirror
image (ventricular inversion or L-loop
ventricles).

16. In the dominant chamber, direct continuity
between one or both AV valves and the
semilunar valve establishes its morphology
as left ventricular.

17. B. Double inlet right ventricle
It is an exceedingly rare anomaly.
Demonstration of separation of the AV
valves from the semilunar valves by a
collar of myocardium (the conus,
infundibulum, or outflow tract) will establish
the morphology of the dominant chamber
as right ventricular.

18. C. Rarely, the morphology of neither the
dominant chamber nor the hypoplastic
chamber can be determined with certainty.
Such cases are typically categorized as
single functional ventricle of
undifferentiated or indeterminate type.

19. A double inlet AV connection is almost
always associated with a dominant
morphologic LV.
In this situation, both atria connect to the
dominant ventricle by two distinct AV
valves, which are usually mirror-image
morphologic mitral valves.

21. In contrast, in hearts with a common inlet
AV connection, the dominant ventricle is
almost always of right ventricular
morphology.
It should be noted that common inlet right
ventricle occurs much less frequently than
double inlet LV (12% vs. 88%).

22. The third form of
univentricular AV
connection is a
single inlet
ventricle, which
includes
tricuspid atresia
and mitral
atresia.

24. ventriculoarterial relationship is defined by
the ventricle from which most (>50%) of an
overriding semilunar valve originates.

25. Any ventriculoarterial connection can occur -
concordant connection (i.e., aorta arising
from morphologic LV) ,
discordant connections (i.e., aorta arising
off rudimentary outlet chamber with right
ventricular morphology) - univentricular
heart of left ventricular morphology,

26. double outlet from the dominant ventricular
mass (univentricular heart of right
ventricular morphology) or from the
hypoplastic rudimentary outlet chamber ,
single outlet from the dominant ventricular
mass.

27. VSD/the bulboventricular foramen/ the
outlet foramen.
In DILV - muscular in most cases or
sometimes subaortic VSD, when there is
hypoplasia of the infundibulum .
The defect can be restrictive or
unrestrictive at birth but often will become
restrictive over time.

28. 1. Double Inlet Left Ventricle
2. Double-Inlet Right Ventricle
3. Double-Inlet Ventricle of Mixed
Morphology
4. Double-Inlet Ventricle with Indeterminate
or Undifferentiated Morphology
5. Absent AV connection
6. Double-Inlet Ventricle Via a Common AV
Valve

29. DILV is the most common form of single
ventricle (78% of the cases reviewed by
Van Praagh et al.).
Classification based on the great artery
relationships:
I, normally related great arteries;
II, right-anterior aorta;
III, left-anterior aorta; and
IV, left-posterior aorta (inverted).

30. By combining the criteria distinguishing the
types of DILV, three clinically observed
forms occur (A-I, A-II, and A-III).
In each of these clinical forms, other
common associations include subaortic
obstruction, pulmonary outflow tract
obstruction, and conduction abnormalities.

31.  A-I Single Ventricle, “Holmes Heart”
(DILV with Normally Related Great Arteries)
 A-II Single Ventricle
(DILV with Right-Sided Hypoplastic Subaortic
Right Ventricle and discordant VA connection).
 A-III Single Ventricle
(DILV with left-sided subaortic hypoplastic right
ventricle and discordant VA connection)

32. DILV with Normally Related Great Arteries
Relatively rare, observed in only 15% of the
Van Praagh series .

33. DILV with Right-Sided Hypoplastic Subaortic
Right Ventricle.
25% of the cases of single ventricle reviewed
by Van Praagh et al.
A similar anomaly consisting of complete TGA
with severe override and straddling of the
right AV valve into the morphologic LV and
associated hypoplasia of the morphologic
right ventricle.

34. 

35. DILV with left-sided subaortic hypoplastic
right ventricle
38% of the series review by Van Praagh et
al (most common type).
This form of DILV shares anatomic
features similar to AV and VA discordance
(corrected transposition of the great
arteries).

36. 5%, of the series reviewed by Van Praagh et
al.
Hypoplastic rudimentary left ventricular
chamber that is located posteriorly and
slightly to the left of the morphologic right
ventricl usually can be recognized by careful
angiographic or echocardiographic analysis.

37.  5% of the series reported by Van Praagh
et al.
Also called a common ventricle, it was
designated by Van Praagh as the C type of
single ventricle with absence of the
ventricular septum or undivided ventricles
with a rudimentary septum.

38. A primitive form of univentricular AV
connection without a rudimentary chamber.
It shares many of the pathologic features of
both double-inlet right ventricle and double-
inlet of mixed morphology.
No clear-cut differentiation or distinction of
ventricular myocardium can be determined.

39.  Atresia or absence of either Rt or Lt AV
valve.
Either ventricle can be hypoplastic.
VA connection can be concordant or
discordant.

40. Both atria are connected to one ventricle
by a common AV valve.
It is a form of AV septal defect.
33% of Van Praagh et al series of which
40% having asplenia.

41. Normal heart: Circulation consist of
pulmonary and systemic circuit, connected
in series, powerd by a double pump- the
right and left heart.
Unventricular heart: The single ventricle has
to maintain both systemic and pulmonary
circulation-not connected in series but in
parallel.

42. 1.

43. Two main disadvantages in single ventricle
circulation
1. Arterial desaturation
2. Chronic volume overload lead to
a. Dilatation of atrium and ventricle
b. Eccentric hypertrophy
c.Spherical remodeling and reorientation
of wall fibres
d. Annaluar dilatation causing AV regurtation

44. VSD, PDA, ASD/PFO
Transposition of great arteries.
Subpulmonary stenosis (more prevalent)
Aortic arch obst and subaortic stenosis
PLSVC
Interrupted IVC
PAPVC
CoA
Right Aortic arch

45. 70 percent of dominent single left ventricle
died before age 16.
Univentricular heart with right ventricular
morphology—50 percent survival 4 years
after diagnosis.

46. The most common causes of mortality were
arrhythmias, congestive heart failure, and
sudden unexplained death
Despite the overall grim prognosis in
unoperated patients, some adult with DILV,
TGA and well balanced circulations may
survive into their seventh decades.

47. Depends on varying anatomical defects
Basic principles of presentation are
1. Qp ˂ Qs : Severe cyanosis, shock
2. Qp ≈ Qs : Mild cyanosis rather stable
3. Qp »Qs : Heart failure, shock

48. Neonatal Period: Depends on the
associated lessions and degree of outflow
obstruction.
Cyanosis since birth- the most frequent
presentation.
Marked cyanosis, metabolic acidosis- if
subpulmonary stenosis.
CHF and shock- if associated aortic
obstruction.
Cyanosis may not be obvious if increase
PBF.

49. Infancy:
With increased PBF - presents with typical
L-R shunt like repeated CHF and failure to
thrive.
Cyanosis may not be obvious.

50. Older children and adult
In patients with mild to moderate pulmonary
stenosis presents like TOF.
They may be relatively asymptomatic.
In adult they may be presented with
cyanosis, clubbing and retarded growth.

51. 1. An unusual ventricular hypertrophy
pattern with similar QRS complexes across
most or all precordial leads is common (e.g.,
RS, rS, or QR pattern).
2. Abnormal Q waves (representing
abnormalities in septal depolarization)
3. Either first- or second-degree AV block
may be present.
4. Arrhythmias such as SVT or wandering
pacemaker may occur.

52. Abnormal Q waves take one of the following
forms:
Q waves in the right precordial leads,
no Q waves in any precordial leads, or
Q waves in both the right and left
precordial leads.

53. 1. With increased PBF, the heart size
enlarges and the pulmonary vascularity
increases.
2. When PBF is normal or decreased, the
heart size is normal and the pulmonary
vascularity is normal or decreased.
3. A narrow upper mediastinum suggests
TGA.

54.  A single ventricular chamber into which two
AV valves open.
 Morphology of the single ventricle
 Location of the rudimentary outflow chamber
 Size of the BVF and whether there is an
obstruction at the foramen. Obstruction of the
foramen is considered present if the Doppler
gradient is more than 1.5 m/sec or if the area
of the foramen is less than 2 cm2/m2.
Echocardiography

55. Presence or absence of D-TGA or L-TGA,
stenosis of the pulmonary or aortic valve,
and size of the PAs.
Anatomy and position of the mitral and
tricuspid valves - stenosis, regurgitation,
hypoplasia, or straddling.
The size of the ASD.
Associated defects such as COA,
interrupted aortic arch, or PDA.

59. Type Relation of
rudimentary
chamber
to main chamber
Orientation of
trabecular septum
AV valves
LV dominance Anterior with either
D/L loop
Anterior Posterior to
trabecular septum
RV dominance Posterior Posterior Anterior
Primitive No rudimentary
chamber
No trabecular
septum
Echocardiographic diagnosis of
univentricular hearts

60. Schematic
diagram
showing the
checklist
while doing
an
echocardio
gram

61. catheterization is performed only when
certain preoperative information is not
available before the initial stage of surgical
management.
It is, however, routinely indicated before
stages II and III surgical intervention.

62.  Prostaglandin E1
 Restoraration of normal acid base status
 Maintain end organ perfusion & function

63. 1. Initial surgical palliative procedures
a. The first-stage operation
(1) In patients with no PS and large PBF with
resulting CHF and pulmonary edema, PA
banding may be done. PA banding is
performed, only when the BVF is normal or
unobstructed.
(2) In patients with no PS, if the BVF is too
small, the Damus-Kaye-Stansel operation is
performed rather than the PA banding.

64.  (3) If PS or pulmonary atresia is present
(with O2 saturation <85%), a BT shunt is
necessary to improve cyanosis.
Recently, a hybrid procedure consisting of
PDA stenting, bilateral PA banding, and
balloon atrial septostomy (with or without
balloon dilatation), has been used as an
alternative to the BT shunt.

65. (4) If PS is present and the BVF is
obstructive, enlargement of the BVF by a
transaortic approach and without
cardiopulmonary bypass may be
performed.
The surgical mortality rate is about 15%. An
additional BT shunt may be needed to
provide adequate pulmonary blood flow.

66.  b. Surgery for interrupted aortic arch or
coarctation should be performed, if present.
c. After the first-stage operation, the infant
should be watched closely, until the time of
the second-stage palliation, for cyanosis
(with O2 saturation <75%) or signs of CHF
(too large a pulmonary blood flow for which
tightening of the PA band should be
considered).

67. 2. Second-stage surgical palliative
procedures
3. Definitive (Fontan) procedures

68. Surgical approach for single ventricle

69. Damus-Kaye-Stansel anastomosis for
single ventricle and subpulmonary
stenosis.

70. 1. Bidirectional Glenn operation (BDG) -
An end-to-side SVC-to-RPA shunt (
bidirectional superior cavopulmonary
shunt) is performed by 2.5 to 3 months of
age.
2. The hemi-Fontan operation.

72. The hemi-Fontan operation - An incision is
made along the most superior part of the
right atrial appendage and is extended into
the SVC. A connection is made between
this opening and the lower margin of the
central portion of the PA. An intra-atrial
baffle is placed to direct blood to the
pulmonary arteries. The Blalock-Taussig
shunt is taken down and the native
pulmonary valve is oversewn.

75. A modified Fontan operation - is the
definitive procedure for patients with tricuspid
atresia. Directing the entire systemic venous
blood to the pulmonary arteries without an
intervening pumping chamber. The Fontan
operation is usually completed when the child
is around 2 years of age.

76. 1. Following bidirectional Glenn
procedure-An intra-atrial tubular pathway
is created from the orifice of the IVC to the
orifice of the SVC termed cavocaval baffle
or a “lateral tunnel”. The cardiac end of the
SVC is anastomosed to the undersurface
of the RPA to complete the operation . An
extracardiac conduit may be used to
complete the Fontan operation.

77. 2. Following the hemi-Fontan operation -
the intra-atrial patch that was used to direct
SVC blood to the PAs is excised and a
lateral atrial tunnel is constructed directing
flow from the IVC to the previously created
amalgamation of the SVC with the RPA .

79. 1.

80.  1. Close follow-up is necessary for early and
late complications.
2. Some survivors of surgery, if performed
late, remain symptomatic with cyanosis,
dyspnea as a result of ventricular
dysfunction, and arrhythmias. These
symptoms require regular follow-up. Early
surgery tends to reduce unfavorable results.

81. Thank You