SYSTEMIC VENOUS ANOMALIES

96
SYSTEMIC VENOUS ANOMALIES Dr Ranjith MP Senior Resident Department of Cardiology Government Medical college Kozhikode

description

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 - PowerPoint PPT Presentation

Transcript of SYSTEMIC VENOUS ANOMALIES

Page 1: SYSTEMIC VENOUS ANOMALIES

SYSTEMIC VENOUS ANOMALIES

Dr Ranjith MPSenior Resident

Department of CardiologyGovernment Medical college

Kozhikode

Page 2: SYSTEMIC VENOUS ANOMALIES

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

Page 3: SYSTEMIC VENOUS ANOMALIES

EMBRYOLOGY

Page 4: SYSTEMIC VENOUS ANOMALIES

EMBRYOLOGY

Changes in the sinoatrial orifice

Page 5: SYSTEMIC VENOUS ANOMALIES

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 veinsoriginating in the chorionic villi, carrying oxygenated blood to the embryo

3. The cardinal veinsdraining the body of the embryo proper.

Page 6: SYSTEMIC VENOUS ANOMALIES

EMBRYOLOGY

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

Page 7: SYSTEMIC VENOUS ANOMALIES

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

Page 8: SYSTEMIC VENOUS ANOMALIES

EMBRYOLOGY

Development of veins draining upper part of body

A. Ducts of CuvierB. Subclavian veinsC. Transverse anastomosisE. Superior venacavaF. Right Brachiocephalic veinG. Left Brachiocephalic veinH. Internal Jugular vein External jugular vein arise as secondary channel

Page 9: SYSTEMIC VENOUS ANOMALIES

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

Page 10: SYSTEMIC VENOUS ANOMALIES

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

Page 11: SYSTEMIC VENOUS ANOMALIES

EMBRYOLOGY

Development of Inferior venacava

Green- Subcardinal Red- Supracardinal Yellow- Subcardinal-

hepatocardinal anastomosis

Blue- Hepatocardiac channel

White- Supracardinal-Subcardinal anastomosis

Page 12: SYSTEMIC VENOUS ANOMALIES

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

Page 13: SYSTEMIC VENOUS ANOMALIES

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

Page 14: SYSTEMIC VENOUS ANOMALIES

EMBRYOLOGY

Development of Azygos veins

Page 15: SYSTEMIC VENOUS ANOMALIES

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

Page 16: SYSTEMIC VENOUS ANOMALIES

EMBRYOLOGY

.

Page 17: SYSTEMIC VENOUS ANOMALIES

Anomalies of the Superior Venae Cavae

Page 18: SYSTEMIC VENOUS ANOMALIES

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

Page 19: SYSTEMIC VENOUS ANOMALIES

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%

Page 20: SYSTEMIC VENOUS ANOMALIES

Bilateral Superior Venae Cavae with Normal Drainage to the Right Atrium

superior vena cava A=right B=leftC: communicating vein, D: aortaE: pulmonary trunk.

A: coronary sinus (enlarged) B: aorta, C: inferior vena cava D: left pulmonary veins

Page 21: SYSTEMIC VENOUS ANOMALIES

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

Page 22: SYSTEMIC VENOUS ANOMALIES

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

Page 23: SYSTEMIC VENOUS ANOMALIES

Bilateral SVC to Right AtriumDiagnostic 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

Page 24: SYSTEMIC VENOUS ANOMALIES

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

Page 25: SYSTEMIC VENOUS ANOMALIES

Bilateral SVC to Right AtriumDiagnostic 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

Page 26: SYSTEMIC VENOUS ANOMALIES

Bilateral SVC to Right AtriumDiagnostic 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

Page 27: SYSTEMIC VENOUS ANOMALIES

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

Page 28: SYSTEMIC VENOUS ANOMALIES

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

Page 29: SYSTEMIC VENOUS ANOMALIES

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

Page 30: SYSTEMIC VENOUS ANOMALIES

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

Page 31: SYSTEMIC VENOUS ANOMALIES

Bilateral SVC with an Unroofed Coronary SinusDiagnostic Features

ECGAxis of the P wave may be abnormal in patients with

heterotaxy syndrome

Chest XrayShadow along the Lt upper border of the mediastinum

Page 32: SYSTEMIC VENOUS ANOMALIES

Bilateral SVC with an Unroofed Coronary SinusDiagnostic 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

Page 33: SYSTEMIC VENOUS ANOMALIES

Bilateral SVC with an Unroofed Coronary SinusDiagnostic Features

A. Injection into the LSVC opacifies CS and shunting ofcontrast 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)

Page 34: SYSTEMIC VENOUS ANOMALIES

Bilateral SVC with an Unroofed Coronary SinusTreatment

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

Page 35: SYSTEMIC VENOUS ANOMALIES

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%)

Page 36: SYSTEMIC VENOUS ANOMALIES

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

Page 37: SYSTEMIC VENOUS ANOMALIES

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

Page 38: SYSTEMIC VENOUS ANOMALIES

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

Page 39: SYSTEMIC VENOUS ANOMALIES

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

Page 40: SYSTEMIC VENOUS ANOMALIES

Left Atrial or Biatrial Drainage of Right SVC

Clinical ManifestationsCyanosis 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

DiagnosisDemonstration of a common entrance site of Rt SVC

and the RUPV in the roof of the LA by echo or angio

Page 41: SYSTEMIC VENOUS ANOMALIES

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

Page 42: SYSTEMIC VENOUS ANOMALIES

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

Page 43: SYSTEMIC VENOUS ANOMALIES

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

Page 44: SYSTEMIC VENOUS ANOMALIES

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

Page 45: SYSTEMIC VENOUS ANOMALIES

Retroaortic Innominate Vein

Clinical ManifestationsAsymptomaticSurgical importance

DiagnosisEcho, 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

Page 46: SYSTEMIC VENOUS ANOMALIES

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

Page 47: SYSTEMIC VENOUS ANOMALIES

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

Page 48: SYSTEMIC VENOUS ANOMALIES

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

Page 49: SYSTEMIC VENOUS ANOMALIES

Anomalies of the Coronary Sinus

Page 50: SYSTEMIC VENOUS ANOMALIES

Anomalies of the Coronary Sinus

Coronary Sinus Defect and Unroofed CS

Coronary Sinus Orifice Atresia

Coronary Sinus Aneurysm or Diverticulum

Page 51: SYSTEMIC VENOUS ANOMALIES

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

Page 52: SYSTEMIC VENOUS ANOMALIES

Coronary Sinus Orifice Atresia

Incidence is rareThe CS is usually well formed, the orifice is covered

by a thin membrane like tissueAlternative exit for coronary venous blood return-

A small LSVC , Large thebesian vein CS septal defect , Connection with the IVC in one case

Page 53: SYSTEMIC VENOUS ANOMALIES

Coronary Sinus Orifice Atresia

Clinical Manifestations

Myocardial ischemia is unlikely aslong as there is an alternate egressfor 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

Page 54: SYSTEMIC VENOUS ANOMALIES

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

Page 55: SYSTEMIC VENOUS ANOMALIES

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

Page 56: SYSTEMIC VENOUS ANOMALIES

Anomalies of the Inferior Vena Cava

Page 57: SYSTEMIC VENOUS ANOMALIES

Anomalies of the Inferior Vena Cava

Interrupted Inferior Vena Cava

Bilateral Inferior Vena Cavae

Inferior Vena Cava drainage to the Left Atrium

Page 58: SYSTEMIC VENOUS ANOMALIES

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

Page 59: SYSTEMIC VENOUS ANOMALIES

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

Page 60: SYSTEMIC VENOUS ANOMALIES

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

Page 61: SYSTEMIC VENOUS ANOMALIES

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

Page 62: SYSTEMIC VENOUS ANOMALIES

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

TreatmentNo specific treatment of an interrupted IVC with azygos

continuation is indicated

Inadvertent ligation of the azygos vein can lead to death

Page 63: SYSTEMIC VENOUS ANOMALIES

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

Page 64: SYSTEMIC VENOUS ANOMALIES

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

Page 65: SYSTEMIC VENOUS ANOMALIES

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.

Page 66: SYSTEMIC VENOUS ANOMALIES

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

Page 67: SYSTEMIC VENOUS ANOMALIES

Anomalies of the Ductus Venosus

Page 68: SYSTEMIC VENOUS ANOMALIES

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

Page 69: SYSTEMIC VENOUS ANOMALIES

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

Page 70: SYSTEMIC VENOUS ANOMALIES

Anomalous Termination of the Umbilical Veins and Absent Ductus Venosus

Diagnosis

Catheterization of the umbilical vein

Cardiac catheterization plus angiography

Prenatal echocardiography

Postnatal echocardiography

Page 71: SYSTEMIC VENOUS ANOMALIES

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

Page 72: SYSTEMIC VENOUS ANOMALIES

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

Page 73: SYSTEMIC VENOUS ANOMALIES

THANK YOU

Page 74: SYSTEMIC VENOUS ANOMALIES
Page 75: SYSTEMIC VENOUS ANOMALIES

MCQs

Page 76: SYSTEMIC VENOUS ANOMALIES

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

Page 77: SYSTEMIC VENOUS ANOMALIES

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

Page 78: SYSTEMIC VENOUS ANOMALIES

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

Page 79: SYSTEMIC VENOUS ANOMALIES

MCQ-4

Inferior venacava formed by all except

A. Supracardinal veins

B. Subcardinal veins

C. vitelline veins

D. Umbulical veins

E. Posterior cardinal veins

Page 80: SYSTEMIC VENOUS ANOMALIES

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

Page 81: SYSTEMIC VENOUS ANOMALIES

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%

Page 82: SYSTEMIC VENOUS ANOMALIES

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

Page 83: SYSTEMIC VENOUS ANOMALIES

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

Page 84: SYSTEMIC VENOUS ANOMALIES

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

Page 85: SYSTEMIC VENOUS ANOMALIES

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

Page 86: SYSTEMIC VENOUS ANOMALIES

Answers

Page 87: SYSTEMIC VENOUS ANOMALIES

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

Page 88: SYSTEMIC VENOUS ANOMALIES

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

Page 89: SYSTEMIC VENOUS ANOMALIES

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

Page 90: SYSTEMIC VENOUS ANOMALIES

MCQ-4

Inferior venacava formed by all except

A. Supracardinal veins

B. Subcardinal veins

C. vitelline veins

D. Umbulical veins

E. Posterior cardinal veins

Page 91: SYSTEMIC VENOUS ANOMALIES

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

Page 92: SYSTEMIC VENOUS ANOMALIES

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%

Page 93: SYSTEMIC VENOUS ANOMALIES

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

Page 94: SYSTEMIC VENOUS ANOMALIES

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

Page 95: SYSTEMIC VENOUS ANOMALIES

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

Page 96: SYSTEMIC VENOUS ANOMALIES

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