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

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  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

 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.  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  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  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  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  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  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 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%  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 C: communicating vein, D: aorta E: pulmonary trunk. A: coronary sinus (enlarged) B: aorta, C: inferior vena cava 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  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  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  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  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  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  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  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  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  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  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 A. Injection into the LSVC opacifies CS and shunting of contrast medium into the LA thorough the defect B.MR image in a coronal plane shows complete unroofing of the CS. LSVC connects to the roof of the LA and the CS opening functions as a LA septal defect (Raghib defect)

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  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%)  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  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  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  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  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  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  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  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  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  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  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  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

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

 Coronary Sinus Defect and Unroofed CS  Coronary Sinus Orifice Atresia  Coronary Sinus Aneurysm or Diverticulum  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  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  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  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  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  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

 Interrupted Inferior Vena Cava  Bilateral Inferior Vena Cavae  Inferior Vena Cava drainage to the Left Atrium  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  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  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  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  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  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  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  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

 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  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  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  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  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  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

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  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  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  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  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  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%  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  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  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  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  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