PULMONARY VEINS: DEVELOPMENT & ANOMALIES

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PULMONARY VEINS: DEVELOPMENT & ANOMALIES : 

PULMONARY VEINS: DEVELOPMENT & ANOMALIES DR. ARINDAM PANDE POST DOCTORAL TRAINEE, CARDIOLOGY, IPGME&R

DEVELOPMENT OF PULMONARY VEINS : 

DEVELOPMENT OF PULMONARY VEINS Pulmonary vascular bed, from- vascular plexus of the foregut (the splanchnic plexus) Common pulmonary vein (a transient anatomic structure), from- --evagination in the sinoatrial region of heart --confluence of vessels from pulmonary plexus --confluence of capillaries that grow into the mesocardium, b/w lung buds and heart Individual major pulmonary veins (4 in number) 3

ANOMALIES OF PULMONARY VEINS : 

ANOMALIES OF PULMONARY VEINS Imperfect development of common pulmonary vein provides the embryologic basis for the most anomalies. 5

EMBRYOLOGIC CLASSIFICATION OF PULMONARY VENOUS ANOMALIES : 

EMBRYOLOGIC CLASSIFICATION OF PULMONARY VENOUS ANOMALIES 6

NORMAL ABSORPTION OF COMMON PULMONARY VEIN WITH PARTIALLY OR TOTALLY ABNORMAL PULMONARY VENOUS DRAINAGE AND NORMAL CONNECTION OF THE PULMONARY VEINS : 

NORMAL ABSORPTION OF COMMON PULMONARY VEIN WITH PARTIALLY OR TOTALLY ABNORMAL PULMONARY VENOUS DRAINAGE AND NORMAL CONNECTION OF THE PULMONARY VEINS SINUS VENOSUS DEFECT MALPOSITION OF SEPTUM PRIMUM COMMON ATRIUM 7

SINUS VENOSUS DEFECT : 

SINUS VENOSUS DEFECT True defect is the deficiency of the common wall b/w right SVC and right upper PV or b/w right atrium and right upper and lower PVs -- unroofing of the PVs Allow atrial level shunt, but not atrial septal defects Always associated with drainage of some/all right pulmonary veins (PVs) into right SVC/right atrium 8

Slide 11: 

—51-year-old man with pulmonary hypertension, right ventricular dilatation, and increase in oxygen saturation between inferior vena cava (IVC) and main pulmonary artery. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

MALPOSITION OF SEPTUM PRIMUM : 

MALPOSITION OF SEPTUM PRIMUM When septum secundum is absent, septum primum may be displaced towards the anatomic left atrium Usually in cases of visceral heterotaxy with polysplenia Incorporation of half/all pulmonary veins into morphologic right atrium 12

Chest 1995;107:1488:1498 : 

Chest 1995;107:1488:1498

ATRESIA OF COMMON PULMONARY VEIN (EARLY) WHILE PULMONARY-TO-SYSTEMIC VENOUS CONNECTIONS ARE STILL PRESENT : 

ATRESIA OF COMMON PULMONARY VEIN (EARLY) WHILE PULMONARY-TO-SYSTEMIC VENOUS CONNECTIONS ARE STILL PRESENT Primitive connections b/w splanchnic plexus and the cardinal or umbilicoviteline venous system persist and enlarge --PAPVC (failure to establish a normal connection between one or more of the PVs with the CPV) --TAPVC (failure to establish a normal connection between the pulmonary venous plexus and the CPV) 15

PAPVC : 

PAPVC Wide anatomic spectrum Left-sided PVs → derivatives of the left cardinal system (CS & LIV) Right-sided PVs → derivatives of the right cardinal system (SVC & IVC) 17

PAPVC : 

PAPVC MAJOR THREE TYPES -- LPV connecting to the LIV with subsequent drainage to the right SVC and to the right atrium (Most common type). -- Scimitar syndrome with connection of the RPV to the IVC (second most common type). This syndrome is often associated with hypoplasia of the right lung and abnormal arterial supply, as well as secondary dextrocardia. --Partial anomalous pulmonary venous connection of the left pulmonary veins to the CS. 18

Slide 24: 

—7-day-old boy with partial anomalous pulmonary venous connection. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

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—7-day-old boy with partial anomalous pulmonary venous connection. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

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—38-year-old woman with anomalous dual pulmonary venous drainage of left lung. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 28: 

—38-year-old woman with anomalous dual pulmonary venous drainage of left lung. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 29: 

—38-year-old woman with anomalous dual pulmonary venous drainage of left lung. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

TAPVC : 

TAPVC Neumerous classifications Drarling et al. Type 1- supra cardiac Type 2- cardiac (to CS) Type 3- infracardiac Type 4- two or more of above Burroughs and Edwards (based on length of anomalous channel, prognostic implications) Long, Intermediate, Short Smith et al. Supracardiac (without pulmonary venous obstruction) Infradiaphragmatic (with pulmonary venous obstruction) 30

Slide 36: 

—3-day-old girl with cardiac total anomalous pulmonary venous connection (type II). Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

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—5-day-old boy with infracardiac total anomalous pulmonary venous connection (type III). Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 38: 

—3-month-old boy with supracardiac total anomalous pulmonary venous connection (type I), asplenia, dextrocardia, right aortic arch with mirror-image branching, and bilateral superior venae cavae. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 39: 

—3-month-old boy with supracardiac total anomalous pulmonary venous connection (type I), asplenia, dextrocardia, right aortic arch with mirror-image branching, and bilateral superior venae cavae. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 40: 

—3-month-old boy with supracardiac total anomalous pulmonary venous connection (type I), asplenia, dextrocardia, right aortic arch with mirror-image branching, and bilateral superior venae cavae. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

ATRESIA OF COMMON PULMONARY VEIN (LATE) WHILE PULMONARY-TO-SYSTEMIC VENOUS CONNECTIONS ARE OBLITERATED : 

ATRESIA OF COMMON PULMONARY VEIN (LATE) WHILE PULMONARY-TO-SYSTEMIC VENOUS CONNECTIONS ARE OBLITERATED Normally, after the common pulmonary vein has established a connection with the left atrium, and the primitive venous connections have regressed, the connection between the common pulmonary vein and the left atrium enlarges. If the normal connection between the common pulmonary vein and the left atrium fails, the pulmonary veins drain into a blind cul-de-sac without a significant alternative egress for pulmonary venous blood (atresia of the common pulmonary vein). 43

STENOSIS OF THE COMMON PULMONARY VEIN: COR TRIATRIATUM : 

STENOSIS OF THE COMMON PULMONARY VEIN: COR TRIATRIATUM The connection between the common pulmonary vein and the left atrium is stenotic, and the common pulmonary vein dilates 45

ANATOMIC CLASSIFICATION OF COR TRIATRIATUM : 

ANATOMIC CLASSIFICATION OF COR TRIATRIATUM 47

Slide 50: 

—24-year-old woman with cor triatriatum. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

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—24-year-old woman with cor triatriatum. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 52: 

—39-year-old woman with recurrent atrial flutter and unsuspected cor triatriatum. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

ABNORMAL ABSORPTION OF COMMON PULMONARY VEIN INTO LEFT ATRIUM : 

ABNORMAL ABSORPTION OF COMMON PULMONARY VEIN INTO LEFT ATRIUM Stenosis of individual pulmonary veins --localized stenosis at its junction with LA --hypoplasia of lumen (intrapulmonary / extrapulmonary) Abnormal number of pulmonary veins 53

Slide 55: 

—2-day-old boy with congenital pulmonary vein atresia, bilateral superior venae cavae, and aberrant right subclavian artery arising from left aortic arch. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 56: 

—2-day-old boy with congenital pulmonary vein atresia, bilateral superior venae cavae, and aberrant right subclavian artery arising from left aortic arch. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 57: 

—2-day-old boy with congenital pulmonary vein atresia, bilateral superior venae cavae, and aberrant right subclavian artery arising from left aortic arch. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 58: 

—2-day-old boy with congenital pulmonary vein atresia, bilateral superior venae cavae, and aberrant right subclavian artery arising from left aortic arch. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 59: 

—5-month-old girl with congenital pulmonary venous stenosis. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

Slide 60: 

—5-month-old girl with congenital pulmonary venous stenosis. Dillman J R et al. AJR 2009;192:1272-1285 ©2009 by American Roentgen Ray Society

THANK YOU : 

THANK YOU 61

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