Development of great veins and surgical importance

Development of great veins and surgical importance

1. MurtazaKamal
Fellow PediatricCardiology
DOP: 20.02.2018
Development of Great veins &
Surgical Importance

2. Great venous system

3. Azygous venous system

4. EMBRYOLOGY
Heart-First organ to start functioning & the 1st organ to fully
develop in the fetus
First seen as 2 endothelial heart tube
21 days -heart tube forms
23 days-heart beats
Week 4 -cardiac loop forms
Week 7 -heart fully developed

5. EMBRYOLOGY

6. EMBRYOLOGY
Changes in the sinoatrial orifice

7. EMBRYOLOGY
In the fifth week, three pairs of major veins can be
distinguished:
1.The vitelline veins (omphalomesenteric veins)
carrying blood from the yolk sac to the sinus venosus
2.The umbilical veins
originating in the chorionic villi, carrying oxygenated blood to the
embryo
3.The cardinal veins
draining the body of the embryo proper.

8. EMBRYOLOGY
Main components of the venous and arterial systems in a 4-mm
embryo (end of the fourth week).

9. EMBRYOLOGY
Cardinal Veins
The anterior cardinal veins drains the cephalic part of the embryo
The posterior cardinal veins drains the rest of the embryo
The anterior and posterior veins join before entering the sinus
horn and form the short common cardinal veins (ducts of Cuvier)
During the fourth week, the cardinal veins form a symmetrical
system

10. EMBRYOLOGY
Development of veins draining upper part of body
A.Ducts of Cuvier
B.Subclavian veins
C.Transverse anastomosis
E.Superior venacava
F.Right Brachiocephalic vein
G.Left Brachiocephalic vein
H.Internal Jugular vein
External jugular vein arise
as secondary channel

11. EMBRYOLOGY
Development of Inferior venacava
B. Vitelline and umbulical vein
broken in to numerous channel
C. Formation of Hepatocardiac
channel
D. Formation of Common
hepatic vein & Ductus venosus

12. EMBRYOLOGY
Development of Inferior venacava
During the fifth to the seventh week a number of additional
veins are formed:
1.The subcardinal veins, mainly drain the kidneys
2.The sacrocardinal veins, drain the lower extremities
3.The supracardinal veins, drain the body wall by way of the
intercostal veins, taking over the functions of the posterior
cardinal veins

13. EMBRYOLOGY
Development of Inferior venacava
Green-Subcardinal
Red-Supracardinal
Yellow-Subcardinal-
hepatocardinal
anastomosis
Blue-Hepatocardiac
channel
White-Supracardinal-
Subcardinal
anastomosis

14. EMBRYOLOGY
Development of Inferior venacava
The anastomosis between the subcardinal veins forms the
left renal vein
The left subcardinal vein disappears, and only its distal
portion remains as the left gonadal vein
The right subcardinal vein becomes the main drainage
channel and develops into the renal segment of the inferior
vena cava

15. EMBRYOLOGY
Development of Inferior venacava
The anastomosis between the sacrocardinal veins forms the
left common iliac vein
The right sacrocardinal vein becomes the sacrocardinal
segment of the inferior vena cava
When the renal segment of the IVC connects with the
hepatic segment, the IVC (consisting of hepatic, renal, and
sacrocardinal segments) is complete

16. Derivatives of embryonic systemic
veins

17. EMBRYOLOGY
Development of Azygos veins

18. EMBRYOLOGY
.

19. EMBRYOLOGY
Development of Azygos veins
The 4thto 11th right intercostal veins empty into the
right supracardinal vein, which together with a portion
of the posterior cardinal vein forms the azygos vein
On the left the 4th to 8thintercostal veins enter into
the left supracardinal vein, and the left supracardinal
vein, then known as the accessory hemiazygosvein,
empties into the azygos vein

20. Anomalies of the Superior Venae
Cavae

21. Anomalies of the SVC
Bilateral SVC with normal drainage
Bilateral SVC with an Unroofed Coronary Sinus
Absent Right SVC in Visceroatrial Situs Solitus
Left Atrial or Biatrial Drainage of Right SVC
Retroaortic Innominate Vein

22. Bilateral Superior Venae Cavae with Normal
Drainage to the Right Atrium
Result from failure of the left anterior and left common
cardinal veins to involute
The incidence is 0.3%
LSVC drains into RA through CS in 92%
-in to LA by unroofed CS in 8%

23. LSVC

24. Bilateral Superior Venae Cavae with Normal
Drainage to the Right Atrium
superior vena cava A=right B=left
C: communicating vein, D: aorta
E: pulmonary trunk.
A: coronary sinus (enlarged)
B: aorta, C: inferior vena cava
D: left pulmonary veins

25. Bilateral Superior Venae Cavae with Normal
Drainage to the Right Atrium
Anatomy
The size of the LSVC varies
left innominate vein may be present in 60%
The LSVC starts at the junction of the left jugular and
left subclavian veins
Joins the CS in the posterior left AV groove

26. Bilateral Superior Venae Cavae with Normal
Drainage to the Right Atrium
Clinical Manifestations
Physiology is usually normal & no clinical
manifestations
Enlargement of the CS may interfere with blood flow
from the LA into the LV
An increase in the magnitude of the Lt to Rt shunt at
the atrial level was found in patients with secundum
ASD persistent LSVC, and dilated coronary sinus

27. Bilateral SVC to Right Atrium
Diagnostic Features
Chest Xray
Shadow along the Lt upper border of the mediastinum
2D echo 100% specificity & 96% sensitivity
A dilated CS is often the first clue to the diagnosis
Imaged from the suprasternal notch or from the high left
parasternal/subclavicular windows
Presence & size of the Lt innominate vein can also be imaged

28. Bilateral Superior Venae Cavae with Normal
Drainage to the Right Atrium
2D echo
There is an inverse relationship between the caliber of
the LSVC and the left innominate vein
May confuse with a TAPVC or PAPVC, left superior
intercostal vein, and a levoatrialcardinal vein
In contrast to a LSVC to an intact coronary sinus,
however, the direction of blood flow in these veins is
expected to be into the left innominate vein

29. Bilateral SVC to Right Atrium
Diagnostic Features
A: Subcostal long-axis
view
B: Parasternal long-
axis view
C: The LSVC is seen
anterior to LPA in the
parasternal short-axis
view
D: The drainage of the
LSVC to the CS and to
RA seen in parasternal
sagittal view

30. Bilateral SVC to Right Atrium
Diagnostic Features
Cardiac catheterization
LSVC can be suspected by the presence of higher-than-
expected CS oxygen saturation
The LSVC can be approached either through the right SVC
(when the innominate vein is present) or through the
coronary sinus
Left innominate vein angiography with balloon occlusion
proximal to the injection site is diagnostic

31. Bilateral Superior Venae Cavae with Normal
Drainage to the Right Atrium
Treatment-No treatment is
necessary for an isolated LSVC to
an intact coronary sinus

32. Bilateral SVC with an Unroofed Coronary Sinus
Anatomy
Common wall between the LA & CS absent
Persistent LSVC drains into the left atrium
In patients with a normal inter atrial septum, the orifice of
the unroofed CS will function as an interatrial
communication
Visceral heterotaxy with asplenia exhibits the highest
incidence of bilateral SVCs with a completely unroofed
coronary sinus

33. Bilateral SVC with an Unroofed Coronary Sinus
Clinical Manifestations
Most patients have a large CS ostium that functions as
an interatrial communication (Raghib syndrome)
Cyanosis and left-to-right shunting
In most patients, the arterial oxygen saturation ranges
between 85% and 95%
They are at risk for complications of right-to-left
shunting, including paradoxical emboli, brain abscess,
strokes, and death

34. Bilateral SVC with an Unroofed Coronary Sinus
Clinical Manifestations
In patients with atretic CS ostium the only clinical
manifestations are cyanosis and its sequelae
When right atrial outflow stenosis or atresia coexists
with a persistent LSVC to an unroofed coronary sinus,
the shunt is exclusively from right to left

35. Bilateral SVC with an Unroofed Coronary Sinus
Diagnostic Features
ECG
Axis of the P wave may be abnormal in patients with
heterotaxy syndrome
Chest Xray
Shadow along the Lt upper border of the mediastinum

36. Bilateral SVC with an Unroofed Coronary Sinus
Diagnostic Features
Echocardiography-the definitive imaging modality
The posterior left AV groove is examined in detail to ascertain the extent of
deficiency of the CS septum
When the CS septum is completely unroofed, the LSVC terminates in the upper
Lt posterior corner of the LA between the LUPV posteriorly and the LA
appendage anteriorly
Color Doppler or contrast injection demonstrates flow from the LSVC into LA
Cardiac catheterization
Step-down in oxygen saturation between PV & LA
LSVC selective angiocardiography

37. Bilateral SVC with an Unroofed Coronary Sinus
Diagnostic Features
A. Injection into the LSVC
opacifies CS and shunting of
contrast medium into the LA
thorough the defect
B.MR image in a coronal plane
shows complete unroofing of
the CS. LSVC connects to the
roof of the LA and the CS
opening functions as a LA
septal defect (Raghib defect)

38. Bilateral SVC with an Unroofed Coronary Sinus
Treatment
Repair is done to avoid complications of cyanosis
If the LSVC is relatively small and there is an adequate-
sized left innominate vein, the LSVC can be ligated and
the interatrial communication closed
In the absence of an adequate-sized bridging left
innominate vein, the coronary sinus is reroofed
Baffling the LSVC along the posterior wall of the LA in to RA
ASD device closure of CS defect

39. Absent Right SVC in Visceroatrial Situs
Solitus
0.07% to 0.13% of cardiovascular malformations
Characterized by persistence of the LSVC draining to
the RA via the CS and by left-sided azygos vein
draining into the LSVC
Less constant features
Additional cardiovascular malformations (46%)
Rhythm abnormalities (35%)

40. Absent Right SVC in Visceroatrial Situs
Solitus
Clinical Manifestations
Usually asymptomatic
Rhythm disturbances
Atrioventricular block
sinoatrial node dysfunction
ventricular tachycardia
Left and right bundle-branch block
supraventricular tachycardia
Sudden death

41. Absent Right SVC in Visceroatrial Situs
Solitus
Diagnostic Features
Issues that make diagnosis important are
–Implantation of transvenous pacemaker
–Placement of a pulmonary artery catheter for intraoperative or
postoperative monitoring without the use of fluoroscopy
–Systemic venous cannulation for extracorporeal membrane
oxygenation
–Systemic venous cannulation for cardiopulmonary bypass
–Partial or total cavopulmonary anastomosis
–Orthotopic heart transplantation and endomyocardial biopsies
Diagnosis established by echocardiography, MRI, CT, or angiography

42. Absent Right SVC in Visceroatrial Situs
Solitus
Treatment
No intervention is indicated
when the physiology is normal
Venogram in the innominate vein in a patient
with absence of the right SVC and persistence of
the LSVC, which drains into the RA via the CS

43. Left Atrial or Biatrial Drainage of Right SVC
It represents a sinus venosus defect of the
SVC type in association with atresia of the
right SVC orifice
It results from the deficiency of the common
wall between the SVC & RUPV
This defect unroofs the RUPV & its branches
into the right SVC
The unroofed RUPV then drains into the SVC,
and its LA orifice becomes the interatrial
communication

44. Left Atrial or Biatrial Drainage of Right SVC
Clinical Manifestations
Cyanosis is the dominant clinical feature
symptoms may not develop until late childhood or
adolescence
The risks of Rt to Lt shunt sequelae increase with age
Diagnosis
Demonstration of a common entrance site of Rt SVC
and the RUPV in the roof of the LA by echo or angio

45. Left Atrial or Biatrial Drainage of Right SVC
Treatment
The right SVC flow is surgically diverted into the RA
In the past, this was done by creating an ASD and
redirecting SVC flow into RA and the pulmonary blood
flow into LA
Preferred surgical approach is transection of the right
SVC above the entrance of the RUPV and anastomosis
of the transected caval end to the RA appendage

46. Retroaortic Innominate Vein
First reported in 1888, and 62 cases have been reported
to date
Also known as postaortic innominate vein
Anatomy
Characterized by an abnormal position of the left innominate vein
behind the ascending aorta
Normal course of the left innominate vein is from left to right, anterior
to the aortic arch
In RAIV it is horizontally behind the ascending aorta to reach the SVC
below the insertion of the azygos vein

47. Retroaortic Innominate Vein
Most patients have associated cardiac malformations
Embryology
Results from failure of the high transverse capillary plexus
that forms the left innominate vein to develop
In such circumstance, venous blood returning from the Lt
side of the head and the Lt arm may drain through a lower
venous plexus that communicates between the Lt & Rt
anterior cardinal veins
This lower venous plexus then forms the RAIV

48. Retroaortic Innominate Vein
A: Diagram showing a RAIV associated with a
. right aortic arch in a patient with TOF, RSVC
B: Gadolinium-enhanced MR angiogram showing a
. retroaortic innominate vein

49. Retroaortic Innominate Vein
Clinical Manifestations
Asymptomatic
Surgical importance
Diagnosis
Echo, angio or MRI
Accurate echocardiographic diagnosis is based on tracking
the left innominate vein from its origin through its
retroaortic course to the SVC
Cine MRI and three-dimensional MRA are particularly useful
in depicting the anatomy
Treatment-No treatment is necessary

50. Levoatrialcardinal vein
First described Edwards and DuShanein 1950 as
a vein connecting the rt. SVC and LA
Remnant of an early embryonic venous channel
that connects the splanchnic plexus of the lungs
with the cardinal system
In the mature heart, it connects the LA or a PV
with the Lt innominate or other systemic veins
Typically it is associated with severe LA outlet
obstruction

51. Levoatrialcardinal vein
The diagnosis can be established by
following the anomalous vein from its
origin to its termination in a systemic
vein
Unlike persistent LSVC that courses
anterior to the left pulmonary artery, a
levoatrialcardinal vein typically ascends
posterior to it
It complicates the completion of a
Fontan-type operation
Levoatriocardinal vein in a
patient with cortriatriatum. It
drains the proximal chamber of
the LA to the innominate vein

52. Anomalies of the Coronary Sinus

53. Anomalies of the Coronary Sinus
Coronary Sinus Defect and Unroofed CS
Coronary Sinus Orifice Atresia
Coronary Sinus Aneurysm or Diverticulum

54. Coronary Sinus Defect & Unroofed CS
Unroofed coronary sinus almost always is associated
with a persistent LSVC
A CS defect without an associated LSVC, and the
physiology is the same as in ASD
Diagnosis-Echo, Color Doppler, Contrast echo
Surgery is usually performed for associated
malformations

55. Coronary Sinus Orifice Atresia
Incidence is rare
The CS is usually well formed, the orifice is covered
by a thin membrane like tissue
Alternative exit for coronary venous blood return-
A small LSVC
CS septal defect

56. Coronary Sinus Orifice Atresia
Clinical Manifestations
Myocardial ischemia is unlikely as
long as there is an alternate egress
for the coronary sinus blood
Diagnostic Features
Suspected by the echo demonstration
of a persistent LSVC to an intact CS with retrograde flow
Angiographic demonstration of retrograde flow in the coronary
sinus and a small caliber LSVC is suggestive of the diagnosis

57. Coronary Sinus Aneurysm or Diverticulum
First described in 1983 by Ho et al
It is a pouch with its neck originating in the CS proximal to the
entrance of the middle cardiac vein. The pouch, 2 to 5 cm in
diameter, extends into the LV wall
It may be associated with WPW syndrome (posteroseptal accessory
pathways)
Diagnostic Features
diagnosed by echocardiography
Imaged from the subcostal, apical, and parasternal windows
Seen as an outpouch, typically with a distinct neck, which extends behind the
LV or into the ventricular myocardium

58. Coronary Sinus Aneurysm or Diverticulum
Treatment
Intervention indicated in patients with arrhythmia
Conduction abnormality disappears only after
separation or ablation of the CS diverticulum neck

59. Anomalies of the Inferior Vena
Cava

60. Anomalies of the Inferior Vena Cava
Interrupted Inferior Vena Cava
Bilateral Inferior Vena Cavae
Inferior Vena Cava drainage to the Left Atrium

61. Interrupted Inferior Vena Cava
Anatomy
Absence of the hepatic segment of the IVC with azygos
continuation into the right or left
Rarely the infrahepatic segment of the IVC may continue to
both right and left SVC via bilateral azygos veins
One of the characteristics of the polysplenia syndrome
Also has been reported in patients with normal hearts and
rarely in patients with asplenia

62. Interrupted Inferior Vena Cava
Posterior view of the
heart, lungs, liver, and
kidneys of a 6 1/2-
month-old boy with
visceral heterotaxy and
left-sided polysplenia.
There is interruption of
the right-sided IVC with
bilateral azygos veins
connecting with
bilateral superior venae

63. Interrupted Inferior Vena Cava
Clinical Manifestations
Interrupted IVC with azygos continuation usually does not result in a
physiologic abnormality
Can complicate cardiac catheterization and interventional procedures
Diagnostic Features
Diagnosed readily by echocardiography
Diagnosis is based on imaging of the size, location, and course of the
IVC and the azygos vein

64. Interrupted Inferior Vena Cava
Diagnostic Features
Normally, in the subcostal short-axis view, the renal-to-hepatic
segment of the IVC is seen as an oval blood vessel located anterior
and to the right of the abdominal aorta
In heterotaxy syndrome, the IVC may be juxtaposed to the abdominal
aorta either to the left or to the right of the spine
When the renal-to-hepatic segment of the IVC is absent, no IVC is
seen below the liver
Drainage of the azygos vein to the SVC-can be imaged from the
parasternal and suprasternal windows

65. Interrupted Inferior Vena Cava
three-dimensional MRA is accurate and effective in delineating
normal and abnormal systemic venous anatomy
During cardiac catheterization, venous angiography from the lower
extremity is diagnostic
Treatment
No specific treatment of an interrupted IVC with azygos
continuation is indicated
Inadvertent ligation of the azygos vein can lead to death

66. Bilateral Inferior Venae Cavae
Anatomy
Bilateral suprahepatic IVCs (i.e., a normal IVC and a contralateral
hepatic vein) a frequent finding in cases of visceral heterotaxy with
asplenia
Bilateral suprahepatic IVCs also can occur rarely in patients with
normal visceral situs
The left-sided hepatic vein in those cases drains into a normal
coronary sinus
Do not produce any hemodynamic disturbance

67. Inferior Vena Cava Drainage to the LA
Anatomy
During fetal life, about half of the IVC blood that enters
the RA is directed toward the LA with the help of two
venous valves: the eustachian valve & valve of the
foramen ovale
A left atrial IVC also occur in cases in which all the
systemic and all the PVs drained into a left-sided atrium

68. Inferior Vena Cava Drainage to the LA
AP venogramin a 15-month-old boy with visceral heterotaxy, asplenia. Contrast injected into the
rt. iliac vein filled two venous pathways. The rt-sided pathway represents a rt. IVC, which
receives the rthepatic vein & then enters the rtside of the common atrium. The ltvenous
channel appears to represent a lt. IVC, which connects with the left hepatic vein & then joins the
RIVC and drains into the common atrium via a common orifice.

69. Inferior Vena Cava Drainage to the LA
Clinical Manifestations
Partial or complete drainage of the IVC into the left atrium results
in cyanosis
The clinical manifestations are the result of right-to-left shunting,
including polycythemia, brain abscess, and paradoxical emboli
Treatment
Inferior vena cava blood is surgically redirected into the right
atrium

70. Anomalies of the Ductus Venosus

71. Anomalies of the Ductus Venosus
Anomalous Termination of the Umbilical Veins
and Absent Ductus Venosus
Postnatal Persistence of the Ductus Venosus

72. Anomalous Termination of the Umbilical Veins
and Absent Ductus Venosus
Anatomy
Persisted left umbulical vein terminates directly into the CS , to the CS by way
of the left portal vein or into the iliac vein
Persisted right umbilical vein terminates directly into the RA, into the IVC, into
the rtportal vein & into the right SVC
Clinical Manifestations
Usually do not produce symptoms
Intrauterine obstruction of the umbilical vein flow and postnatal intestinal
obstructions secondary to the anomalous termination of the umbilical veins
have been reported

73. Anomalous Termination of the Umbilical Veins
and Absent Ductus Venosus
Diagnosis
Catheterization of the umbilical vein
Cardiac catheterization plus angiography
Prenatal echocardiography
Postnatal echocardiography

74. Postnatal Persistence of the Ductus
Venosus
Anatomy
Congenital postnatal persistence of the ductus venosus because
the shunt was away from the portal venous septum proximally to
the distal hepatic veins or IVC distally
These intrahepatic portal-systemic shunts are due to abnormal
persistence of elements of the omphalomesenteric system
Clinical Manifestations
Three of the ten cases reported resulted in portal-systemic
encephalopathy

75. Postnatal Persistence of the Ductus
Venosus
Diagnosis
Ultrasound or computed tomography -demonstrates a large tortuous
vessel originating from the portal vein that connected to the hepatic
vein or IVC
Treatment
In the absence of encephalopathy, treatment may not be indicated
If ligation of the ductus venosus is contemplated, one should establish
the integrity of the portal system. If it is not intact, ligation could lead
to mesenteric venous congestion and ultimately bowel ischemia

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