Skip to main content

Abstract

Congenital venous anomalies of the thorax may be encountered in patients with documented congenital heart disease, those patients with abnormal echocardiographic findings (dilated right ventricle or pulmonary hypertension, for example), or unexpectedly, in patients having CT scan or magnetic resonance examination of the thorax for other reasons. These anomalies can range from the clinically important (partial anomalous pulmonary venous connection) to the clinically inconsequential (persistent left superior vena cava) and it is essential for the reading physician to be able to identify these anomalies and associated intracardiac or extracardiac defects in order to decide whether further imaging and investigation is warranted.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG. Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg. 2007;84:2020–6.

    Article  PubMed  Google Scholar 

  2. Ammash NM, Seward JB, Warnes CA, Connolly HM, O’Leary PW, Danielson GK. Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography. J Am Coll Cardiol. 1997;29:1351–8.

    Article  PubMed  CAS  Google Scholar 

  3. Attenhofer Jost CH, Connolly HM, Danielson GK, Bailey KR, Schaff HV, Shen W-K, Warnes CA, Seward JB, Puga FJ, Tajik AJ. Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients. Circulation. 2005;112:1953–8.

    Article  PubMed  Google Scholar 

  4. Bartram U, Fischer G, Kramer HH. Congenitally interrupted inferior vena cava without other features of the heterotaxy syndrome: report of five cases and characterization of a rare entity. Pediatr Dev Pathol. 2008;11:266–73.

    Article  PubMed  Google Scholar 

  5. Demos TC, Posniak HV, Pierce KL, Olson MC, Muscato M. Venous anomalies of the thorax. AJR Am J Roentgenol. 2004;182:1139–50.

    PubMed  Google Scholar 

  6. Dillman JR, Yarram SG, Hernandez RJ. Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol. 2009;192:1272–85.

    Article  PubMed  Google Scholar 

  7. Geva T, Van Praagh S. Anomalies of the pulmonary veins. Abnormal systemic venous connections. In: Allen HD, Gutgesell HP, Clark EB, Driscoll DJ, editors. Moss and Adams’ heart disease in infants, children and adolescents. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 761–817.

    Google Scholar 

  8. Ho ML, Bhalla S, Bierhals A, Gutierrez F. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 2009;24:89–95.

    Article  PubMed  Google Scholar 

  9. Kafka H, Mohiaddin RH. Cardiac MRI and pulmonary MR angiography of sinus venosus defect and partial anomalous pulmonary venous connection in cause of right undiagnosed ventricular enlargement. AJR Am J Roentgenol. 2009;192:259–66.

    Article  PubMed  Google Scholar 

  10. Kafka H, Gatzoulis MA, Rubens MB. Superior vena cava obstruction due to markedly enlarged right pulmonary artery in Eisenmenger syndrome. Eur Heart J. 2007;28:1404.

    Article  PubMed  Google Scholar 

  11. Keeble W, Mohiaddin R. Technical failure to perform cardiac resynchronization therapy: use of cardiac magnetic resonance imaging techniques to clarify a left-sided superior vena cava and coronary sinus morphology. Can J Cardiol. 2008;24:589–90.

    Article  PubMed  Google Scholar 

  12. Krishnam MS, Tomasian A, Malik S, Singhal A, Sassani A, Laub G, Finn JP, Ruehm S. Three-dimensional imaging of pulmonary veins by a novel steady-state free-precession magnetic resonance angiography technique without the use of intravenous contrast agent: initial experience. Invest Radiol. 2009;44:447–53.

    Article  PubMed  Google Scholar 

  13. Locca D, Hughes M, Mohiaddin R. Cardiovascular magnetic resonance diagnosis of a previously unreported association: cor triatriatum with right partial anomalous pulmonary venous return to the azygos vein. Int J Cardiol. 2009;135:e80–2.

    Article  PubMed  Google Scholar 

  14. Marom EM, Herndon JE, Kim YH, McAdams HP. Variations in pulmonary venous drainage to the left atrium: implications for radiofrequency ablation. Radiology. 2004;230:824–9.

    Article  PubMed  Google Scholar 

  15. Mozes G, Gloviczki P. Venous embryology and anatomy. In: Bergan JJ, editor. The Vein Book. Oxford: Elsevier; 2007. p. 15–26.

    Chapter  Google Scholar 

  16. Pascoe RD, Oh JK, Warnes CA, Danielson GK, Tajik AJ, Seward JB. Diagnosis of sinus venosus atrial septal defect with transesophageal echocardiography. Circulation. 1996;94:1049–55.

    Article  PubMed  CAS  Google Scholar 

  17. Prasad SK, Soukias N, Hornung T, Khan M, Pennell DJ, Gatzoulis MA, Mohiaddin RH. Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage. Circulation. 2004;109:207–14.

    Article  PubMed  Google Scholar 

  18. Rahmani N, White CS. MR imaging of thoracic veins. Magn Reson Imaging Clin N Am. 2008;16:249–62.

    Article  PubMed  Google Scholar 

  19. Valente AM, Sena L, Powell AJ, del Nido PJ, Geva T. Cardiac magnetic resonance imaging evaluation of sinus venosus defects: comparison to surgical findings. Pediatr Cardiol. 2007;28:51–6.

    Article  PubMed  Google Scholar 

  20. White CS, Baffa JM, Haney PJ, Pace ME, Campbell AB. MR imaging of congenital anomalies of thoracic veins. Radiographics. 1997;17:595–608.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Henryk Kafka M.D., FRCPC, FACC .

Editor information

Editors and Affiliations

4.1 Electronic Supplementary Material

Below is the link to the electronic supplementary material.

Movie 4.1

Twenty-year old woman who had repair of a supracardiac TAPVC in infancy and is assessed for increasing dyspnea and Xray evidence of heart failure. (a) Single shot turbo spin echo axial image demonstrating the posterior location of the horizontal pulmonary venous confluence (HVC). (b) Contrast-enhanced Magnetic Resonance Angiography: Maximum intensity projection (mip) coronal image. This demonstrates the connections of the four pulmonary veins into the HVC. (c) Sagittal still image from SSFP cine confirming the superior location of the HVC and stenosis of the orifice between the HVC and the left atrium (LA). Note the hypertrophied right ventricle (RV). (d) Coronal still image from SSFP cine demonstrating the stenotic orifice and the turbulent flow into the LA (arrow) from the HVC. SSFP cines for (cand d) are available as supplemental images (AVI 3339 KB)

Movie 4.2

Twenty-year old woman who had repair of a supracardiac TAPVC in infancy and is assessed for increasing dyspnea and Xray evidence of heart failure. (a) Single shot turbo spin echo axial image demonstrating the posterior location of the horizontal pulmonary venous confluence (HVC). (b) Contrast-enhanced Magnetic Resonance Angiography: Maximum intensity projection (mip) coronal image. This demonstrates the connections of the four pulmonary veins into the HVC. (c) Sagittal still image from SSFP cine confirming the superior location of the HVC and stenosis of the orifice between the HVC and the left atrium (LA). Note the hypertrophied right ventricle (RV). (d) Coronal still image from SSFP cine demonstrating the stenotic orifice and the turbulent flow into the LA (arrow) from the HVC. SSFP cines for (cand d) are available as supplemental images (AVI 3339 KB)

Movie 4.3

Cor triatriatum.Cine MRI images (balanced steady state free precession sequences) acquired in transaxial plane through the left atrium (left) and in the left ventricular outflow tract (right). Arrowindicates cor triatriatum membrane. Note the anomalous right pulmonary venous connection to the azygos vein (asterisk). The movement of the membrane and the flow through the membrane can be better appreciated in the cine loops (Adapted from Locca et al. [13]. With permission from Elsevier13) (AVI 4050 KB)

Movie 4.4

Cor triatriatum.Cine MRI images (balanced steady state free precession sequences) acquired in transaxial plane through the left atrium (left) and in the left ventricular outflow tract (right). Arrowindicates cor triatriatum membrane. Note the anomalous right pulmonary venous connection to the azygos vein (asterisk). The movement of the membrane and the flow through the membrane can be better appreciated in the cine loops (Adapted from Locca et al. [13]. With permission from Elsevier13) (AVI 4505 KB)

Movie 4.5

Pulmonary vein stenosis.(a) Twenty-five year old woman with dyspnea. Still axial image from a SSFP cine demonstrates stenosis (arrow) at the connection of the left lower pulmonary vein to the left atrium. This can be better appreciated on the SSFP cine and corresponding phase contrast flow study (supplemental cine files). (b) Thirty-four year old woman with dyspnea and dilated pulmonary artery on echocardiography. Contrast-enhanced MR angiography coronal image. Arrowspoint to the stenoses of the right lower and right upper pulmonary veins at the left atrium. Aoaorta (AVI 5151 KB)

Movie 4.6

Pulmonary vein stenosis.(a) Twenty-five year old woman with dyspnea. Still axial image from a SSFP cine demonstrates stenosis (arrow) at the connection of the left lower pulmonary vein to the left atrium. This can be better appreciated on the SSFP cine and corresponding phase contrast flow study (supplemental cine files). (b) Thirty-four year old woman with dyspnea and dilated pulmonary artery on echocardiography. Contrast-enhanced MR angiography coronal image. Arrowspoint to the stenoses of the right lower and right upper pulmonary veins at the left atrium. Aoaorta (AVI 6438 KB)

Movie 4.7

PAPVC flow studies. Fifty-seven-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LAleft atrium, RAright atrium, RPAright pulmonary artery, RVright ventricle. (aand b) Oblique axial steady-state free precession cine image (a) shows anomalous connection of right upper pulmonary vein (arrowhead). Sinus venosus defect (arrow) is also evident at this level. Bright white signal in this velocity flow map (b) confirms flow from pulmonary vein into RA. (cand d) Coronal steady-state free precession cine image (c) shows connection of right pulmonary vein to RA–superior vena cava junction (arrowhead). Dark signal in this velocity flow map (d) shows flow (arrowhead) from anomalous pulmonary vein into RA. The cines from which these stills are derived can be viewed as supplemental files (Reprinted with permission from Kafka and Mohiaddin [9]) (AVI 4611 KB)

Movie 4.8

PAPVC flow studies. Fifty-seven-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LAleft atrium, RAright atrium, RPAright pulmonary artery, RVright ventricle. (aand b) Oblique axial steady-state free precession cine image (a) shows anomalous connection of right upper pulmonary vein (arrowhead). Sinus venosus defect (arrow) is also evident at this level. Bright white signal in this velocity flow map (b) confirms flow from pulmonary vein into RA. (cand d) Coronal steady-state free precession cine image (c) shows connection of right pulmonary vein to RA–superior vena cava junction (arrowhead). Dark signal in this velocity flow map (d) shows flow (arrowhead) from anomalous pulmonary vein into RA. The cines from which these stills are derived can be viewed as supplemental files (Reprinted with permission from Kafka and Mohiaddin [9]) (AVI 4611 KB)

Movie 4.9

Sinus venosus defect with PAPVC – velocity flow maps. (aand b) Transverse images in 32-year-old man with sinus venosus defect. Steady-state free precession cine image (a) shows sinus venosus defect (black arrow) between left atrium (LA) and superior vena cava (SVC) (asterisk). Corresponding in-plane velocity flow map (b) shows dark inflow from pulmonary vein (white arrow) into LA crossing sinus venosus defect and entering the SVC. (cand d)Sagittal images in 35-year-old woman with sinus venosus defect. Steady-state free precession cine frame (c) shows superior nature of sinus venosus defect (arrow) between LA and SVC (asterisk). Corresponding in-plane velocity flow map (d) shows dark inflow from LA across sinus venosus defect (arrow) into right atrium (RA). (eand f) Coronal images in 18-year-old woman with sinus venosus defect. FLASH image (e) shows bright flow disturbance in SVC (asterisk) related to flow through sinus venosus defect. Through-plane velocity flow map (f) in same position as (e) shows sinus venosus defect as dark region of flow (arrow) from LA. Aoaorta. (f) Is available to be viewed as a cine in the supplemental files (Reprinted with permission from Kafka and Mohiaddin [9]) (AVI 338 KB)

Movie 4.10

Left superior vena cava. Still image from SSFP cine LVOT view demonstrating dilated coronary sinus (white arrow) posterior to the left atrium (LA). (a) Still image from SSFP cine transverse view. The left superior vena cava (LSVC) is seen anterior to the left pulmonary artery. (b) Still image from SSFP cine sagittal oblique demonstrates the LSVC entering the dilated coronary sinus (white arrow) and flowing into the right atrium (RA). The cine from which this still is derived is available as a supplemental file. Aoascending aorta, DAdescending aorta, RSVCright superior vena cava, PAmain pulmonary artery (AVI 5721 KB)

Movie 4.11

Interrupted IVC with azygos continuation.Adult female presented with PSVT. She was referred for ablation but the right atrium could not be accessed via the femoral vein route. On MR imaging she was found to have an interrupted IVC with azygos continuation. (a) SSFP bright blood sagittal image demonstrating the dilated azygos vein (arrow) and azygos arch connecting to the superior vena cava (*). Note absence of the intrahepatic IVC. (b) SSFP bright blood coronal image demonstrating the double arch appearance with the dilated azygos vein (arrow) on the right of the aorta (Ao). (c) SSFP bright blood axial image at the level of the pulmonary artery (PA) bifurcation demonstrating the dilated azygos vein (arrow) and superior vena cava (*). (d) SSFP bright blood axial image caudal to plane of (c) demonstrating the azygos arch and the aortic arch. SSFPsagittal plane cine is available as supplemental file (AVI 4481 KB)

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer-Verlag London

About this chapter

Cite this chapter

Kafka, H., Mohiaddin, R.H. (2012). Venoatrial Abnormalities. In: Syed, M., Mohiaddin, R. (eds) Magnetic Resonance Imaging of Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-4267-6_4

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-4267-6_4

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-4266-9

  • Online ISBN: 978-1-4471-4267-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics