Pediatric Radiology

, Volume 44, Issue 9, pp 1158–1168 | Cite as

Imaging in congenital pulmonary vein anomalies: the role of computed tomography

  • Kevin Todd Dyer
  • Anthony Marcus Hlavacek
  • Felix Gabriel Meinel
  • Carlo Nicola De Cecco
  • Andrew Douglas McQuiston
  • Uwe Joseph Schoepf
  • Nicholas Peter Pietris
Pictorial Essay


Pulmonary venous anomalies comprise a wide spectrum of anatomical variations and their clinical presentations may vary from the relatively benign single partial anomalous pulmonary venous connection (PAPVC) to the critical obstructed total anomalous pulmonary venous connection (TAPVC). We briefly review the common anomalies encountered, while highlighting the utility that computed tomographic angiography (CTA) provides for this spectrum of extracardiac vascular malformations and connections. CTA has established itself as an invaluable imaging modality in these patients. A detailed knowledge of the CTA imaging findings in pulmonary venous anomalies is crucial to guide clinical decision-making in these patients.


Pulmonary venous anomalies Computed tomography angiography Total anomalous pulmonary venous connection Partial anomalous pulmonary venous connection Scimitar syndrome 


Conflicts of interest

U. J. Schoepf is a consultant for and receives research support from Bayer, Bracco, GE, Medrad and Siemens. K. Dyer, A. Hlavacek, F. Meinel, C. De Cecco, A. McQuiston and N. Pietris have no disclosures.


  1. 1.
    Geva T, van Praagh S (2008) Anomalies of the pulmonary veins. In: Allen H, Shaddy R, Driscoll D et al (eds) Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult, 7th edn. Lippincott Williams & Wilkins, Philadelphia, p 761Google Scholar
  2. 2.
    Rajiah P, Kanne JP (2010) Computed tomography of pulmonary venous variants and anomalies. J Cardiovasc Comput Tomogr 4:155–163PubMedCrossRefGoogle Scholar
  3. 3.
    Latson LA, Prieto LR (2007) Congenital and acquired pulmonary vein stenosis. Circulation 115:103–108PubMedCrossRefGoogle Scholar
  4. 4.
    Watson TG, Mah E, Joseph Schoepf U et al (2013) Effective radiation dose in computed tomographic angiography of the chest and diagnostic cardiac catheterization in pediatric patients. Pediatr Cardiol 34:518–524PubMedCrossRefGoogle Scholar
  5. 5.
    Nie P, Wang X, Cheng Z et al (2012) Accuracy, image quality and radiation dose comparison of high-pitch spiral and sequential acquisition on 128-slice dual-source CT angiography in children with congenital heart disease. Eur Radiol 22:2057–2066PubMedCrossRefGoogle Scholar
  6. 6.
    Karamlou T, Gurofsky R, Al Sukhni E et al (2007) Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation 115:1591–1598PubMedCrossRefGoogle Scholar
  7. 7.
    Patton WL, Momenah T, Gooding CA et al (1999) The vascular vise causing TAPVR type I to radiographically mimic TAPVR type III. Pediatr Radiol 29:323–326PubMedCrossRefGoogle Scholar
  8. 8.
    Dillman JR, Yarram SG, Hernandez RJ (2009) Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol 192:1272–1285PubMedCrossRefGoogle Scholar
  9. 9.
    Seale AN, Uemura H, Webber SA et al (2010) Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation 122:2718–2726PubMedCrossRefGoogle Scholar
  10. 10.
    Cetin I, Ozkan S, Varan B et al (2009) Anomalous pulmonary venous connection to the azygous vein: surgical approach of a case with 3D computed tomography findings. J Card Surg 24:345–347PubMedCrossRefGoogle Scholar
  11. 11.
    Tajik AJ, Gau GT, Ritter DG et al (1972) Echocardiographic pattern of right ventricular diastolic volume overload in children. Circulation 46:36–43PubMedCrossRefGoogle Scholar
  12. 12.
    Tsitouridis I, Tsinoglou K, Morichovitou A et al (2006) Scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography. Acta Radiol 47:927–932PubMedCrossRefGoogle Scholar
  13. 13.
    Ciliberti P, Taylor AM, Yates R et al (2013) Occlusion of persistent levoatrial cardinal vein without left heart hypoplasia utilizing an Amplatzer device. Eur Heart J Cardiovasc Imaging 14:857PubMedCrossRefGoogle Scholar
  14. 14.
    Butts RJ, Crean AM, Hlavacek AM et al (2011) Veno-venous bridges: the forerunners of the sinus venosus defect. Cardiol Young 21:623–630PubMedCentralPubMedCrossRefGoogle Scholar
  15. 15.
    Seale AN, Webber SA, Uemura H et al (2009) Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study. Heart 95:1944–1949PubMedCrossRefGoogle Scholar
  16. 16.
    Breinholt JP, Hawkins JA, Minich LA et al (1999) Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome. Ann Thorac Surg 68:164–168PubMedCrossRefGoogle Scholar
  17. 17.
    Frazier AA, Burke AP (2012) The imaging of pulmonary hypertension. Semin Ultrasound CT MR 33:535–551PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Kevin Todd Dyer
    • 1
  • Anthony Marcus Hlavacek
    • 1
    • 2
  • Felix Gabriel Meinel
    • 1
    • 3
  • Carlo Nicola De Cecco
    • 1
    • 4
  • Andrew Douglas McQuiston
    • 1
  • Uwe Joseph Schoepf
    • 5
    • 1
    • 2
  • Nicholas Peter Pietris
    • 1
    • 2
  1. 1.Department of Radiology and Radiological ScienceMedical University of South CarolinaCharlestonUSA
  2. 2.Division of Cardiology Department of PediatricsMedical University of South CarolinaCharlestonUSA
  3. 3.Institute for Clinical RadiologyLudwig-Maximilians-University HospitalMunichGermany
  4. 4.Department of Radiological Sciences Oncology and PathologyUniversity of Rome “Sapienza” – Polo PontinoLatinaItaly
  5. 5.Division of Cardiology Department of MedicineMedical University of South CarolinaCharlestonUSA

Personalised recommendations