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SYSTEMIC VENOUS ANOMALIES
            Dr Ranjith MP
           Senior Resident
       Department of Cardiology
      Government Medical college
              Kozhikode
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
EMBRYOLOGY
EMBRYOLOGY



 Changes in the sinoatrial orifice
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
EMBRYOLOGY




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

                                     Green- Subcardinal
                                     Red- Supracardinal
                                     Yellow- Subcardinal-
                                      hepatocardinal
                                      anastomosis
                                     Blue- Hepatocardiac
                                      channel
                                     White- Supracardinal-
                                      Subcardinal
                                      anastomosis
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
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
EMBRYOLOGY
Development of Azygos veins
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 7th intercostal veins enter into
      the left supracardinal vein, and the left supracardinal
      vein, then known as the hemiazygos vein, empties into
      the azygos vein
EMBRYOLOGY
.
Anomalies of the Superior Venae
             Cavae
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
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%
Bilateral Superior Venae Cavae with Normal
          Drainage to the Right Atrium
superior vena cava A=right B=left   A: coronary sinus (enlarged)
C: communicating vein, D: aorta     B: aorta, C: inferior vena cava
E: pulmonary trunk.                 D: left pulmonary veins
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
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
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
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
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
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
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
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
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
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
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
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
Bilateral SVC with an Unroofed Coronary Sinus
              Diagnostic Features




                                 B.MR image in a coronal plane
  A. Injection into the LSVC     shows complete unroofing of
  opacifies CS and shunting of   the CS. LSVC connects to the
  contrast medium into the LA    roof of the LA and the CS
  thorough the defect            opening functions as a LA
                                 septal defect (Raghib defect)
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
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%)
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
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
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
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
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
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
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
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
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
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
Levoatrialcardinal vein

 First described Edwards and DuShane in 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
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
                                            Levoatriocardinal vein in a
                                            patient with cortriatriatum. It
It complicates the completion of a         drains the proximal chamber of
Fontan-type operation                       the LA to the innominate vein
Levoatrialcardinal vein

                  Multiplanar reformated
                  image showing a
                  levoatriocardinal vein (arrow)
                  connecting an anomalous
                  vein draining into the IVC and
                  a pulmonary vein draining in
                  to LA
Anomalies of the Coronary Sinus
Anomalies of the Coronary Sinus


Coronary Sinus Defect and Unroofed CS

Coronary Sinus Orifice Atresia

Coronary Sinus Aneurysm or Diverticulum
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
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 , Large thebesian vein
      CS septal defect , Connection with the IVC in one case
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
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
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

      The CS was dissected away from the LV and the AV junction
      and that site was cryoablated

      No postoperative recurrences or complications occurred
Anomalies of the Inferior Vena
            Cava
Anomalies of the Inferior Vena Cava


Interrupted Inferior Vena Cava

Bilateral Inferior Vena Cavae

Inferior Vena Cava drainage to the Left Atrium
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
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
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
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
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
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
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
Inferior Vena Cava Drainage to the LA




AP venogram in 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 rt hepatic vein & then enters the rt side of the common atrium. The lt venous
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.
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
Anomalies of the Ductus Venosus
Anomalies of the Ductus Venosus


Anomalous Termination of the Umbilical Veins
 and Absent Ductus Venosus

Postnatal Persistence of the Ductus Venosus

Persistent Valves of the Sinus Venosus
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 rt portal 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
Anomalous Termination of the Umbilical Veins
        and Absent Ductus Venosus


Diagnosis

      Catheterization of the umbilical vein

      Cardiac catheterization plus angiography

      Prenatal echocardiography

      Postnatal echocardiography
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
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
THANK YOU
MCQs
MCQ-1

 False statement about development of heart

A. 21 days - heart tube forms

B. 23 days- heart beats

C. Week 6 - cardiac loop forms

D. Week 7 - heart fully developed
MCQ-2

 False statement about development of Systemic veins

A. Sinus venosus absorbed in to right atrium

B. Left horn of sinus venosus form coronary sinus

C. SVC derived from rt. Posterior cardinal vein and common
   cardinal vein

D. Lt. brachiocephalic vein derived from the part of left anterior
   cardinal vein
MCQ-3

 All are true statements except

A. Subclavian vein derived from intersegmental vein

B. External jugular derived from anterior cardinal vein

C. Lt. Superior intercostal vein formed from lt. anterior and
   posterior cardinal vein

D. Transverse intercardinal anastomosis form part of left
   brachiocephalic vein
MCQ-4

 Inferior venacava formed by all except

A. Supracardinal veins

B. Subcardinal veins

C. vitelline veins

D. Umbulical veins

E. Posterior cardinal veins
MCQ-5

 Bilateral SVC with normal drainage to the RA false statement is

A.   left innominate vein may be present in 60%

B.   The LSVC starts at the junction of the left jugular and left subclavian
     veins

C.   In LSVC to an intact coronary sinus the direction of blood flow is into the
     left innominate vein

D.   A dilated CS is often the first clue to the diagnosis

E.   There is an inverse relationship between the caliber of the LSVC and the
     left innominate vein
MCQ-6

 All are true statements except
A.   Visceral heterotaxy with asplenia exhibits the highest incidence of
     bilateral SVCs with a completely unroofed coronary sinus

B.   Raghib syndrome characterized by cyanosis with right-to-left shunting

C.   In patients with atretic CS ostium & LSVC the only clinical manifestations
     are cyanosis and its sequelae

D.   In bilateral SVC with an Unroofed CS the arterial oxygen saturation
     ranges between 85% and 95%
MCQ-7

False statement about Retroaortic Innominate Vein
A.   Most patients have associated cardiac malformations

B.   Results from failure of the low transverse capillary plexus that
     forms the left innominate vein to develop

C.   Cine MRI and three-dimensional MRA are particularly useful in
     depicting the anatomy

D. Normal course of the left innominate vein is from left to right,
   anterior to the aortic arch
MCQ-8

 False statement about Levoatrialcardinal vein

A.   Remnant of an early embryonic venous channel that connects the
     splanchnic plexus of the lungs with the cardinal system

B.   Typically It is associated with severe LA outlet obstruction

C.   It courses anterior to the left pulmonary artery

D. In the mature heart, it connects the LA or a PV with the Lt
   innominate or other systemic veins
MCQ-9

 False statement regarding Coronary Sinus Defect and
  Unroofed CS

A.   A CS defect without an associated LSVC the physiology is the same
     as in ASD

B.   Step-up in oxygen saturation between PV & LA

C.   Surgery is usually performed for associated malformations

D. Unroofed coronary sinus almost always is associated with a
   persistent LSVC
MCQ-10

 False statement about Interrupted IVC

A.   Interrupted IVC with azygos continuation usually does not result in
     a physiologic abnormality

B.   It is characteristics of the asplenia syndrome

C.   Absence of the hepatic segment of the IVC with azygos
     continuation into the right or left

D. No specific treatment of an interrupted IVC with azygos
   continuation is indicated
Answers
MCQ-1

False statement about development of heart

A. 21 days - heart tube forms

B. 23 days- heart beats

C. Week 6 - cardiac loop forms

D. Week 7 - heart fully developed
MCQ-2

 False statement about development of Systemic veins

A. Sinus venosus absorbed in to right atrium

B. Left horn of sinus venosus form coronary sinus

C. SVC derived from rt. Posterior cardinal vein and common
   cardinal vein

D. Lt. brachiocephalic vein derived from the part of left anterior
   cardinal vein
MCQ-3

 All are true statements except

A. Subclavian vein derived from intersegmental vein

B. External jugular derived from anterior cardinal vein

C. Lt. Superior intercostal vein formed from lt. anterior and
   posterior cardinal vein

D. Transverse intercardinal anastomosis form part of left
   brachiocephalic vein
MCQ-4

 Inferior venacava formed by all except

A. Supracardinal veins

B. Subcardinal veins

C. vitelline veins

D. Umbulical veins

E. Posterior cardinal veins
MCQ-5

 Bilateral SVC with normal drainage to the RA false statement is

A.   left innominate vein may be present in 60%

B.   The LSVC starts at the junction of the left jugular and left subclavian
     veins

C.   In LSVC to an intact coronary sinus the direction of blood flow is into the
     left innominate vein

D.   A dilated CS is often the first clue to the diagnosis

E.   There is an inverse relationship between the caliber of the LSVC and the
     left innominate vein
MCQ-6

 All are true statements except

A.   Visceral heterotaxy with asplenia exhibits the highest incidence of
     bilateral SVCs with a completely unroofed coronary sinus

B.   Raghib syndrome characterized by cyanosis with right-to-left
     shunting

C.   In patients with atretic CS ostium & LSVC the only clinical
     manifestations are cyanosis and its sequelae

D. In bilateral SVC with an Unroofed CS the arterial oxygen saturation
   ranges between 85% and 95%
MCQ-7

False statement about Retroaortic Innominate Vein
A.   Most patients have associated cardiac malformations

B.   Results from failure of the low transverse capillary plexus that
     forms the left innominate vein to develop

C.   Cine MRI and three-dimensional MRA are particularly useful in
     depicting the anatomy

D. Normal course of the left innominate vein is from left to right,
   anterior to the aortic arch
MCQ-8

 False statement about Levoatrialcardinal vein

A.   Remnant of an early embryonic venous channel that connects the
     splanchnic plexus of the lungs with the cardinal system

B.   Typically It is associated with severe LA outlet obstruction

C.   It courses anterior to the left pulmonary artery

D. In the mature heart, it connects the LA or a PV with the Lt
   innominate or other systemic veins
MCQ-9

 False statement regarding Coronary Sinus Defect and
  Unroofed CS

A.   A CS defect without an associated LSVC the physiology is the same
     as in ASD

B.   Step-up in oxygen saturation between PV & LA

C.   Surgery is usually performed for associated malformations

D. Unroofed coronary sinus almost always is associated with a
   persistent LSVC
MCQ-10

 False statement about Interrupted IVC

A.   Interrupted IVC with azygos continuation usually does not result in
     a physiologic abnormality

B.   It is characteristics of the asplenia syndrome

C.   Absence of the hepatic segment of the IVC with azygos
     continuation into the right or left

D. No specific treatment of an interrupted IVC with azygos
   continuation is indicated

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Systemic venous anomalies

  • 1. SYSTEMIC VENOUS ANOMALIES Dr Ranjith MP Senior Resident Department of Cardiology Government Medical college Kozhikode
  • 2. 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
  • 4. EMBRYOLOGY  Changes in the sinoatrial orifice
  • 5. 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
  • 6. EMBRYOLOGY Main components of the venous and arterial systems in a 4-mm embryo (end of the fourth week).
  • 7. 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
  • 8. 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
  • 9. 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
  • 10. 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
  • 11. EMBRYOLOGY Development of Inferior venacava  Green- Subcardinal  Red- Supracardinal  Yellow- Subcardinal- hepatocardinal anastomosis  Blue- Hepatocardiac channel  White- Supracardinal- Subcardinal anastomosis
  • 12. 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
  • 13. 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
  • 15. 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 7th intercostal veins enter into the left supracardinal vein, and the left supracardinal vein, then known as the hemiazygos vein, empties into the azygos vein
  • 17. Anomalies of the Superior Venae Cavae
  • 18. 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
  • 19. 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%
  • 20. Bilateral Superior Venae Cavae with Normal Drainage to the Right Atrium superior vena cava A=right B=left A: coronary sinus (enlarged) C: communicating vein, D: aorta B: aorta, C: inferior vena cava E: pulmonary trunk. D: left pulmonary veins
  • 21. 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
  • 22. 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
  • 23. 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
  • 24. 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
  • 25. 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
  • 26. 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
  • 27. 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
  • 28. 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
  • 29. 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
  • 30. 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
  • 31. 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
  • 32. 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
  • 33. Bilateral SVC with an Unroofed Coronary Sinus Diagnostic Features B.MR image in a coronal plane A. Injection into the LSVC shows complete unroofing of opacifies CS and shunting of the CS. LSVC connects to the contrast medium into the LA roof of the LA and the CS thorough the defect opening functions as a LA septal defect (Raghib defect)
  • 34. 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
  • 35. 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%)
  • 36. 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
  • 37. 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
  • 38. 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
  • 39. 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
  • 40. 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
  • 41. 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
  • 42. 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
  • 43. 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
  • 44. 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
  • 45. 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
  • 46. Levoatrialcardinal vein  First described Edwards and DuShane in 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
  • 47. 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 Levoatriocardinal vein in a patient with cortriatriatum. It It complicates the completion of a drains the proximal chamber of Fontan-type operation the LA to the innominate vein
  • 48. Levoatrialcardinal vein Multiplanar reformated image showing a levoatriocardinal vein (arrow) connecting an anomalous vein draining into the IVC and a pulmonary vein draining in to LA
  • 49. Anomalies of the Coronary Sinus
  • 50. Anomalies of the Coronary Sinus Coronary Sinus Defect and Unroofed CS Coronary Sinus Orifice Atresia Coronary Sinus Aneurysm or Diverticulum
  • 51. 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
  • 52. 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 , Large thebesian vein CS septal defect , Connection with the IVC in one case
  • 53. 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
  • 54. 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
  • 55. 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  The CS was dissected away from the LV and the AV junction and that site was cryoablated  No postoperative recurrences or complications occurred
  • 56. Anomalies of the Inferior Vena Cava
  • 57. Anomalies of the Inferior Vena Cava Interrupted Inferior Vena Cava Bilateral Inferior Vena Cavae Inferior Vena Cava drainage to the Left Atrium
  • 58. 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
  • 59. 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
  • 60. 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
  • 61. 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
  • 62. 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
  • 63. 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
  • 64. 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
  • 65. Inferior Vena Cava Drainage to the LA AP venogram in 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 rt hepatic vein & then enters the rt side of the common atrium. The lt venous 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.
  • 66. 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
  • 67. Anomalies of the Ductus Venosus
  • 68. Anomalies of the Ductus Venosus Anomalous Termination of the Umbilical Veins and Absent Ductus Venosus Postnatal Persistence of the Ductus Venosus Persistent Valves of the Sinus Venosus
  • 69. 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 rt portal 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
  • 70. Anomalous Termination of the Umbilical Veins and Absent Ductus Venosus Diagnosis  Catheterization of the umbilical vein  Cardiac catheterization plus angiography  Prenatal echocardiography  Postnatal echocardiography
  • 71. 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
  • 72. 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
  • 74.
  • 75. MCQs
  • 76. MCQ-1  False statement about development of heart A. 21 days - heart tube forms B. 23 days- heart beats C. Week 6 - cardiac loop forms D. Week 7 - heart fully developed
  • 77. MCQ-2  False statement about development of Systemic veins A. Sinus venosus absorbed in to right atrium B. Left horn of sinus venosus form coronary sinus C. SVC derived from rt. Posterior cardinal vein and common cardinal vein D. Lt. brachiocephalic vein derived from the part of left anterior cardinal vein
  • 78. MCQ-3  All are true statements except A. Subclavian vein derived from intersegmental vein B. External jugular derived from anterior cardinal vein C. Lt. Superior intercostal vein formed from lt. anterior and posterior cardinal vein D. Transverse intercardinal anastomosis form part of left brachiocephalic vein
  • 79. MCQ-4  Inferior venacava formed by all except A. Supracardinal veins B. Subcardinal veins C. vitelline veins D. Umbulical veins E. Posterior cardinal veins
  • 80. MCQ-5  Bilateral SVC with normal drainage to the RA false statement is A. left innominate vein may be present in 60% B. The LSVC starts at the junction of the left jugular and left subclavian veins C. In LSVC to an intact coronary sinus the direction of blood flow is into the left innominate vein D. A dilated CS is often the first clue to the diagnosis E. There is an inverse relationship between the caliber of the LSVC and the left innominate vein
  • 81. MCQ-6  All are true statements except A. Visceral heterotaxy with asplenia exhibits the highest incidence of bilateral SVCs with a completely unroofed coronary sinus B. Raghib syndrome characterized by cyanosis with right-to-left shunting C. In patients with atretic CS ostium & LSVC the only clinical manifestations are cyanosis and its sequelae D. In bilateral SVC with an Unroofed CS the arterial oxygen saturation ranges between 85% and 95%
  • 82. MCQ-7 False statement about Retroaortic Innominate Vein A. Most patients have associated cardiac malformations B. Results from failure of the low transverse capillary plexus that forms the left innominate vein to develop C. Cine MRI and three-dimensional MRA are particularly useful in depicting the anatomy D. Normal course of the left innominate vein is from left to right, anterior to the aortic arch
  • 83. MCQ-8  False statement about Levoatrialcardinal vein A. Remnant of an early embryonic venous channel that connects the splanchnic plexus of the lungs with the cardinal system B. Typically It is associated with severe LA outlet obstruction C. It courses anterior to the left pulmonary artery D. In the mature heart, it connects the LA or a PV with the Lt innominate or other systemic veins
  • 84. MCQ-9  False statement regarding Coronary Sinus Defect and Unroofed CS A. A CS defect without an associated LSVC the physiology is the same as in ASD B. Step-up in oxygen saturation between PV & LA C. Surgery is usually performed for associated malformations D. Unroofed coronary sinus almost always is associated with a persistent LSVC
  • 85. MCQ-10  False statement about Interrupted IVC A. Interrupted IVC with azygos continuation usually does not result in a physiologic abnormality B. It is characteristics of the asplenia syndrome C. Absence of the hepatic segment of the IVC with azygos continuation into the right or left D. No specific treatment of an interrupted IVC with azygos continuation is indicated
  • 87. MCQ-1 False statement about development of heart A. 21 days - heart tube forms B. 23 days- heart beats C. Week 6 - cardiac loop forms D. Week 7 - heart fully developed
  • 88. MCQ-2  False statement about development of Systemic veins A. Sinus venosus absorbed in to right atrium B. Left horn of sinus venosus form coronary sinus C. SVC derived from rt. Posterior cardinal vein and common cardinal vein D. Lt. brachiocephalic vein derived from the part of left anterior cardinal vein
  • 89. MCQ-3  All are true statements except A. Subclavian vein derived from intersegmental vein B. External jugular derived from anterior cardinal vein C. Lt. Superior intercostal vein formed from lt. anterior and posterior cardinal vein D. Transverse intercardinal anastomosis form part of left brachiocephalic vein
  • 90. MCQ-4  Inferior venacava formed by all except A. Supracardinal veins B. Subcardinal veins C. vitelline veins D. Umbulical veins E. Posterior cardinal veins
  • 91. MCQ-5  Bilateral SVC with normal drainage to the RA false statement is A. left innominate vein may be present in 60% B. The LSVC starts at the junction of the left jugular and left subclavian veins C. In LSVC to an intact coronary sinus the direction of blood flow is into the left innominate vein D. A dilated CS is often the first clue to the diagnosis E. There is an inverse relationship between the caliber of the LSVC and the left innominate vein
  • 92. MCQ-6  All are true statements except A. Visceral heterotaxy with asplenia exhibits the highest incidence of bilateral SVCs with a completely unroofed coronary sinus B. Raghib syndrome characterized by cyanosis with right-to-left shunting C. In patients with atretic CS ostium & LSVC the only clinical manifestations are cyanosis and its sequelae D. In bilateral SVC with an Unroofed CS the arterial oxygen saturation ranges between 85% and 95%
  • 93. MCQ-7 False statement about Retroaortic Innominate Vein A. Most patients have associated cardiac malformations B. Results from failure of the low transverse capillary plexus that forms the left innominate vein to develop C. Cine MRI and three-dimensional MRA are particularly useful in depicting the anatomy D. Normal course of the left innominate vein is from left to right, anterior to the aortic arch
  • 94. MCQ-8  False statement about Levoatrialcardinal vein A. Remnant of an early embryonic venous channel that connects the splanchnic plexus of the lungs with the cardinal system B. Typically It is associated with severe LA outlet obstruction C. It courses anterior to the left pulmonary artery D. In the mature heart, it connects the LA or a PV with the Lt innominate or other systemic veins
  • 95. MCQ-9  False statement regarding Coronary Sinus Defect and Unroofed CS A. A CS defect without an associated LSVC the physiology is the same as in ASD B. Step-up in oxygen saturation between PV & LA C. Surgery is usually performed for associated malformations D. Unroofed coronary sinus almost always is associated with a persistent LSVC
  • 96. MCQ-10  False statement about Interrupted IVC A. Interrupted IVC with azygos continuation usually does not result in a physiologic abnormality B. It is characteristics of the asplenia syndrome C. Absence of the hepatic segment of the IVC with azygos continuation into the right or left D. No specific treatment of an interrupted IVC with azygos continuation is indicated