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Partial Anomalous Pulmonary and Systemic Venous Connection (PAPVC)

  • Anita SadeghpourEmail author
  • Azin Alizadehasl
Chapter

Abstract

In the normal anatomy four separate pulmonary veins (PVs) directly connect to the left atrium. However, normal variations in the number of PVs are not uncommon. Partial anomalous pulmonary venous connection (PAPVC) is a condition in which a segment or all of one lung drain into a site other than the left atrium. Sinus venosus type defects are correlated with PAPVC classically from the right upper and right middle lobe pulmonary veins to the superior vena cava. PAPVC can be directed to the superior vena cava at the level or above the right pulmonary artery, left vertical vein, and azygos vein and also to the coronary sinus. PAPVC to the inferior vena cava (scimitar syndrome) can be related to right lung hypoplasia and pulmonary sequestration with an abnormal collateral supply [1–4].

Keywords

Partial Anomalies of the Pulmonary Venous Connections (PAPVC) Atrial Septal Defect (ASD) Sinus Venosus Type ASD Right upper pulmonary vein Echocardiography 

Supplementary material

Video 20.1

The first clue in the evaluation of PAPVC in the suprasternal view is a vessel parallel to the aortic arch with red color flow toward the innominate vein. This left vertical vein connect the left pulmonary veins to the innominate vein which finally drain to the SVC (WMV 1,919 kb)

Video 20.2

The first clue in the evaluation of PAPVC in the suprasternal view is a vessel parallel to the aortic arch with red color flow toward the innominate vein. This left vertical vein connect the left pulmonary veins to the innominate vein which finally drain to the SVC (WMV 556 kb)

Video 20.3

The first clue in the evaluation of PAPVC in the suprasternal view is a vessel parallel to the aortic arch with red color flow toward the innominate vein. This left vertical vein connect the left pulmonary veins to the innominate vein which finally drain to the SVC (WMV 556 kb)

Video 20.4

2D and color Doppler imaging study in the subcostal view showing abnormal pulmonary venous drainage to the inferior vena cava (red color flow toward the IVC, opposite direction from the hepatic vein) suggestive for scimitar syndrome (WMV 331 kb)

Video 20.5

2D and color Doppler imaging study in the subcostal view showing abnormal pulmonary venous drainage to the inferior vena cava (red color flow toward the IVC, opposite direction from the hepatic vein) suggestive for scimitar syndrome (WMV 450 kb)

Video 20.6

2D and color Doppler imaging study in the subcostal view showing abnormal pulmonary venous drainage to the inferior vena cava (red color flow toward the IVC, opposite direction from the hepatic vein) suggestive for scimitar syndrome (AVI 2,374 kb)

References

  1. 1.
    Keane JF, Lock JE, Fyler DC, Nadas AS, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders; 2006.Google Scholar
  2. 2.
    Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald’s Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, PA: Saunders Elsevier; 2011.Google Scholar
  3. 3.
    Perloff JK. Clinical. Recognition of congenital heart disease. 4th ed. Philadelphia, PA: WB Sanuders; 1994.Google Scholar
  4. 4.
    Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. J Am Coll Cardiol. 2008;52(23):e143–263.PubMedCrossRefGoogle Scholar
  5. 5.
    Lilje C, Weiss F, Weil J. Detection of partial anomalous pulmonary venous connection by magnetic resonance imaging. Pediatr Cardiol. 2005;26(4):490–1.PubMedCrossRefGoogle Scholar
  6. 6.
    Xue JR, Luo Y, Cheng P, et al. [Diagnosis and treatment of partial anomalous pulmonary venous connection]. Zhonghua Yi Xue Za Zhi. 2008;88(15):1066–8.PubMedGoogle Scholar
  7. 7.
    Julsrud PR, Ehman RL. The “broken ring” signs in magnetic resonance imaging of partial anomalous pulmonary venous connection to the superior vena cava. Mayo Clin Proc. 1985;60(12):874–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Kasahara H, Aeba R, Tanami Y, et al. Multislice computed tomography is useful for evaluating partial anomalous pulmonary venous connection. J Cardiothorac Surg. 2010;5(1):40.PubMedCentralPubMedCrossRefGoogle Scholar
  9. 9.
    Paulista MD, Paulista PH, Guerra AL, et al. Surgical treatment of partial anomalous pulmonary venous connection to the superior vena cava. Rev Bras Cir Cardiovasc. 2009;24(2):133–7.PubMedCrossRefGoogle Scholar
  10. 10.
    Jemielity M, Perek B, Paluszkiewicz L, et al. Results of repair of partial anomalous pulmonary venous connection and sinus venosus atrial septal defect in adults. J Heart Valve Dis. 1998;7(4):410–4.PubMedGoogle Scholar
  11. 11.
    Nakahira A, Yagihara T, Kagisaki K, et al. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. 2006;82(3):978–82.PubMedCrossRefGoogle Scholar
  12. 12.
    Shahriari A, Rodefeld MD, Turrentine MW, et al. Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. Ann Thorac Surg. 2006;81(1):224–9; discussion 229–30.PubMedCrossRefGoogle Scholar
  13. 13.
    Vanderheyden M, Goethals M, Van Hoe L. Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. 2003;89(7):761.PubMedCentralPubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London 2014

Authors and Affiliations

  1. 1.Echocardiography Lab, Department of CardiologyAdult Congenital Heart Disease Clinic, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical ScienceTehranIran
  2. 2.Echocardiography Lab, Department of CardiologyRajaie Cardiovascular Medical and Research Center, Iran University of Medical ScienceTehranIran

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