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Anomalous Systemic and Pulmonary Venous Connections

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MRI of Cardiovascular Malformations

Abstract

The congenital venous anomalies are subdivided into systemic and pulmonary venous anomalies. The different imaging features of the main anomalies of systemic and pulmonary venous connections are described with a special mention on the MR features. The most frequent variant of systemic venous connections is the left superior vena cava, followed in order of frequency by azygos continuation of the inferior vena cava. In total anomalous pulmonary venous connection all pulmonary veins drain directly into the right atrium or via a systemic vein. In partial anomalous pulmonary venous connection or return (PAPVC) some of the pulmonary veins normally drain into the left atrium, while the others drain into the right atrium or one of its tributaries. The different aberrant connections are described (i.e anomalous connection of the right pulmonary vein into the superior vena cava, anomalous connection of the right superior pulmonary vein into the azygos vein, anomalous connection of the right inferior pulmonary vein into the inferior vena cava), as well as the different histological variants. Further rarer anomalies like supernumerary or absent pulmonary veins, pulmonary vein stenosis, Cor triatriatum are also described.

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Notes

  1. 1.

    Especially in the case of right isomerism, in which persistence of right-sided structures explains the presence of two SVC (53%) or even double IVC each connecting with an atrium (see Chap. 3). The presence of two SVC is less frequent (33%) in left isomerism.

  2. 2.

    This criterion is more reliable, hence the value of oblique images to confirm or exclude connection with the coronary sinus – see below.

  3. 3.

    In left isomerism, often associated with polysplenia, azygos continuation of the IVC is present in 84% of cases (see Chap. 3).

  4. 4.

    In left isomerism, the right and left pulmonary veins frequently depend on each of their respective atria (morphologically left), which is logical anatomically, but which creates hemodynamic abnormalities. In right isomerism, there is “always” an anomalous venous connection, as, even if all of the pulmonary veins converge onto the atrium situated to the left, this atrium (like the atrium on the right) has a right-sided morphology.

  5. 5.

    It may have an extrinsic origin (compression) but also an intrinsic origin (stenosis of the venous confluence or drainage into a high-resistance channel i.e., the portal system).

  6. 6.

    The left SVC crosses the left pulmonary artery anteriorly, while the anomalous vertical vein can pass behind the pulmonary artery, between the pulmonary artery and the left main bronchus with a risk of compression (see Figs. 4.4–4.9).

  7. 7.

    The left SVC crosses the left pulmonary artery anteriorly, while the anomalous vertical vein can pass behind the pulmonary artery, between the pulmonary artery and the left main bronchus with a risk of compression (see Figs. 4.4–4.9).

References

  1. Julsrud PR. Magnetic resonance imaging of the pulmonary arteries and veins. Sem US, CT/MR. 1990;11:184–205.

    CAS  Google Scholar 

  2. Masui T, Seelos KC, Masui T, Kersting-Sommerhoff BA, Higgins CB. abnormalities of the pulmonary veins: evluation with MR imaging and comparison with cardiac angiography and echocardiography. Radiology. 1991;181:645–9.

    PubMed  CAS  Google Scholar 

  3. Kastler B, Gangi A, Klinkert A, Livolsi A, Germain P, Dietemann JL. Contribution of MRI in systemic and pulmonary venous anomalies. RSNA,Chicago, Novr 29–Dec 4. Radiology 1992;suppl:353.

    Google Scholar 

  4. Klinkert A, Kastler B, Gangi A, AHRAN JM, Livolsi A, et al. Diagnoctic IRM des anomalies congénitales des veines caves et des veines pulmonaires. Feuillets de Radiologie. 1994;34:319–29.

    Google Scholar 

  5. White CS, Baffa JM, Haney PJ, Pace ME, Campbell AB. MR imaging of congenital anomalies of the thoracic veins. Radiographics. 1997;17(3):595–608.

    PubMed  CAS  Google Scholar 

  6. White CS. MR imaging of thoracic veins. Magn Reson Imaging Clin N Am. 2000;8(1):17–32.

    PubMed  CAS  Google Scholar 

  7. Kastler B, Clair C, Delabrousse E, Klinkert A, Livolsi A, Sarlieve P, Allal R, Vetter D, Germain P, Bernard Y. Retours veineux systémiques et pulmonaires anormaux: aspects IRM et classification Encycl Méd Chir, Radiodiagnostic- Cœur-Poumon, 32-016-A-20, 2002, 15 p.

    Google Scholar 

  8. Fitoz S, Unsal N, Tekin M, Tutar E. Contrast-enhanced MR angiography of thoracic vascular malformations in children. Int J Cardiol. 2007;123(1):3–11.

    Article  PubMed  Google Scholar 

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

    Article  Google Scholar 

  10. Glickstein JS, Haramati LB, Issenberg HJ, et al. MR imaging and CT of vascular anomalies and connections in patients with congenital heart disease: significance in surgical ­planning. RadioGraphics. 2002;22:337–47. discussion, 348–349.

    PubMed  Google Scholar 

  11. Goo HW, Park IS, Ko JK, et al. CT of congenital heart ­disease: normal anatomy and typical pathologic conditions. RadioGraphics. 2003;23(Spec Issue):S147–65.

    Article  PubMed  Google Scholar 

  12. Leschka S, Oechslin E, Husmann L, et al. Pre- and postoperative evaluation of congenital heart disease in children and adults with 64-section CT. Radiographics. 2007;27(3):829–46. Review.

    Article  PubMed  Google Scholar 

  13. Oh KH, Choo KS, Lim SJ, et al. Multidetector CT evaluation of total anomalous pulmonary venous connections: comparison with echocardiography. Pediatr Radiol. 2009;39:950–4.

    Article  PubMed  Google Scholar 

  14. Kawano T, Ishii M, Takagi J, et al. Three-dimensional helical computed tomographic angiography in neonates and infants with complex congenital heart disease. Am Heart J. 2000;139:654–60.

    Article  PubMed  CAS  Google Scholar 

  15. Kim TH, Kim YM, Suh CH, et al. Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR Am J Roentgenol. 2000;175(5):1381–6.

    PubMed  CAS  Google Scholar 

  16. Goo HW, Park IS, Ko JK, et al. CT of congenital heart disease: normal anatomy and typical pathologic conditions. Radiographics. 2003;23 Spec No:S147–65. Review.

    Article  PubMed  Google Scholar 

  17. Hopkins KL, Patrick LE, Simoneaux SF, Bank ER, Parks WJ, Smith SS. Pediatric great vessel anomalies: initial clinical experience with spiral CT angiography. Radiology. 1996;200:811–5.

    PubMed  CAS  Google Scholar 

  18. Hausleiter J, Meyer T, Hadamitzky M, et al. Radiation dose estimates from cardiac multislice computed tomography in daily practice: impact of different scanning protocols on effective dose estimates. Circulation. 2006;113:1305–10.

    Article  PubMed  Google Scholar 

  19. Cha EM, Khouri GH. Persistent left superior vena cava. Radiologic and clinical significance. Radiology. 1972;103:375–81.

    PubMed  CAS  Google Scholar 

  20. Campbell M, Deuchar DC. The left-sided superior vena cava. Br Heart J. 1994;18:423–39.

    Google Scholar 

  21. Webb WR, Gamsu G, Speckman JM, Kaiser JA, Federle MP, Lipton MJ. Computed tomographic demonstration of mediastinal venous anomalies. AJR Am J Roentgenol. 1982;139(1):157–61.

    PubMed  CAS  Google Scholar 

  22. Winter FS. Persistent left superior cava. Survey of world ­literature and report of thirty additional cases. Angiology. 1954;5:90–132.

    Article  PubMed  CAS  Google Scholar 

  23. Dillon EH, Camputaro C. Partial anomalous pulmonary venous drainage of the left upper lobe vs duplication of the superior vena cava: distinction based on CT findings. AJR Am J Roentgenol. 1993;160(2):375–9.

    PubMed  CAS  Google Scholar 

  24. Gonzalez-Juanatey C, Testa A, Vidan J, Izquierdo R, Garcia-Castelo A, Daniel C, et al. Persistent left superior vena cava draining into the coronary sinus: report of 10 cases and literature review. Clin Cardiol. 2004;27(9):515–8.

    Article  PubMed  Google Scholar 

  25. Cormier MG, Yedlicka JW, Gray RJ, Moncada R. Congenital anomalies of the superior vena cava: a CT study. Semin Roentgenol. 1989;24:77–83.

    Article  PubMed  CAS  Google Scholar 

  26. Park HY, Summoror MH, Preues K, et al. Anomalous drainage of the right superior vena cava into the left atrium. J Am Coll Cardiol. 1983;2:358–62.

    Article  PubMed  CAS  Google Scholar 

  27. Peene P, Verschakelen J, Wilms G, Baert AL. Abnormal pulmonary venous return associated with partially persistent left superior vena cava. Röfo. 1991;154:337–9.

    PubMed  CAS  Google Scholar 

  28. Edwards JE, Dushane JW. Thoracic venous anomalies: vascular connnection on the left atrium and the innominatc vein (levoatriocardinal vein) associated with mitral atresia and premature closure of the foramen oval (case 1). Arch Pathol. 1950;49:517–28.

    Google Scholar 

  29. Pastor BH, Blumberg BI. Persistent left superior vena cava demonstrated by angiocardiography. Am Heart J. 1958;55:120–5.

    Article  PubMed  CAS  Google Scholar 

  30. Ellis JH, Denham JS, Bies JR, Olson EW, Cory DA. Magnetic Resonance Imaging of systemic venous anomalies. Computerized Radiol. 1986;10:15–22.

    Article  CAS  Google Scholar 

  31. Huggins TJ, Lesar ML, Friedman AC, Pyatt RS, Thane TT. CT appearance of persistent left superior vena cava. J Comput Assist Tomogr. 1982;6:294–7.

    Article  PubMed  CAS  Google Scholar 

  32. Kellman GM, Alpern MB, Sandler MA, Craig BM. Computed tomography of vena cava anomalies with embryologic correlation. Radiographics. 1988;8:533–56.

    PubMed  CAS  Google Scholar 

  33. Schultz CL, Morrison S, Brian PG. Azygos continuation in the inferior vena cava: demonstration by NMR. J Comput Assist Tomogr. 1984;8:774–6.

    Article  PubMed  CAS  Google Scholar 

  34. Coulomb M, Rose-Pittet L, Dalsoglio S, Lebas JF, Paillasson F, Gros CH, et al. Continuation azygos droite de la veine cave inférieure. A propos de 3 observations avec étude IRM. J Radiol. 1987;68:45–50.

    PubMed  CAS  Google Scholar 

  35. Pernot C, Hoeffel JC, Worms AM, Auguste JP. La continuation azygos de la veine cave inférieure. Ann Radiol. 1978;21:525–30.

    PubMed  CAS  Google Scholar 

  36. Jelinek JS, Stuan PL, Done SL, Ghaed N, Rudd SA. MRI of polysplenia syndrome. Magn Res Imag. 1989;7:681–6.

    Article  CAS  Google Scholar 

  37. Anderson C, Devine WA, Anderson RH, Debich DE, Zuberbuhler JR. Abnormalities of the spleen in relation to congenital malformations of the heart: a survey of necropsy findings in children. Br Heart J. 1990;63:122–8.

    Article  PubMed  CAS  Google Scholar 

  38. Venables AW. Isolated drainage of the inferior vena to the left atrium. Br Heart J. 1963;25:545–8.

    Article  PubMed  CAS  Google Scholar 

  39. Berdon WE, Baker DH. Plain film findings in azygos continuation of the inferior vena cava. AJR Am J Roentgenol. 1968;104:452–7.

    CAS  Google Scholar 

  40. Rey C. Anomalie du retour systémique. Encycl Méd Chir 1990, Radiodiagnostic, 3:32-016-A-15.

    Google Scholar 

  41. Bank ER, Hernandez RJ. CT and MR of congenital heart disease. Radiol Clin North Am. 1988;26:241–62.

    PubMed  CAS  Google Scholar 

  42. Bennet J, Remy J. Le syndrome de Halasz. Ann Radiol. 1975;18:271–6.

    PubMed  CAS  Google Scholar 

  43. Oropeza G, Hernandez FA, Callard GM, Jude JR. Anomalous pulmonary venous drainage of the left upper lobe. Ann Thorac Surg. 1970;9(2):180–5.

    Article  PubMed  CAS  Google Scholar 

  44. Pennes DR, Ellis JH. Anomalous pulmonary venous drainage of the left upper lobe shown by CT scans. Radiology. 1986;159(1):23–4.

    PubMed  CAS  Google Scholar 

  45. Livolsi A, Kastler B, Marcellin L, Casanova R, Bintner M, Haddad J. MR diagnosis of subdiaphragmatic anomalous pulmonary venous drainage in a newborn. J Comput Assist Tomogr. 1991;15:1051–3.

    Article  PubMed  CAS  Google Scholar 

  46. Dupuis C, Kachaner J, Freedom RM, Payot M, Davignon A. Cardiologie pédiatrique. Flammarion 2e édition. 1991.

    Google Scholar 

  47. Haworth SG. Total anomalous plumonary venous return. Prenatal damage to pulmonary vascular bed and extrapulmonary veins. Br Heart J. 1982;48:513–24.

    Article  PubMed  CAS  Google Scholar 

  48. Sano S, Brawn WJ, Mee RBB. Total anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg. 1989;97:886–92.

    PubMed  CAS  Google Scholar 

  49. Ninet J, Gordillo M, Vigneron M, et al. Retour veineux ­pulmonaire anormal total. Résultats de la correction chez 50 nourrissons. Arch Mal Coeur. 1990;83:217–21.

    PubMed  CAS  Google Scholar 

  50. Gathman GE, Nadas AS. Total anomalous pulmonary venous connection: clinical and physiologic observations of 75 pediatric patients. Circulation. 1970;42:143–54.

    Article  PubMed  CAS  Google Scholar 

  51. Paquet M, Gutgesell H. Echocardiographic features of total anomalous pulmonary venous connection. Circulation. 1975;51:599–605.

    Article  PubMed  CAS  Google Scholar 

  52. Rey C, Lablanche IM, Deloche A. Apport de l’échocardio­graphie au diagnostic du retour veineux pulmonaire anormal total infradiaphragmatique. Arch Mal Coeur. 1977;70:997–1001.

    PubMed  CAS  Google Scholar 

  53. Chin AJ, Sanders SP, Sherman F, Lang P, Norwood WI, Castaneda AR. Accuracy of subcostal two-dimensional echocardiography in prospective diagnosis of total anomalous pulmonary venous connection. Am Heart J. 1987;113:1153–9.

    Article  PubMed  CAS  Google Scholar 

  54. Sahn DJ, Allen HD, Lange LW, Goldberg SJ. Cross-sectional echocardiographic diagnosis of the sites of total anomalous pulmonary venous drainage. Circulation. 1979;60:1317–25.

    Article  PubMed  CAS  Google Scholar 

  55. Cooper MJ, Teitel DF, Silverman NH, Enderlein MA. Study of the infradiaphragmatic total anomalous pulmonary venous connection with cross-sectional and pulsed Doppler echocardiography. Circulation. 1984;70:412–6.

    Article  PubMed  CAS  Google Scholar 

  56. Boxer RA, Singh S, Lacorte MA, Goldman M, Stein HL. Cardiac magnetic resonance imaging in congenital heart ­disease. J Pediatr. 1986;109:460–4.

    Article  PubMed  CAS  Google Scholar 

  57. Kastler B, Livolsi A, Germain P, et al. Contribution of MRI in supracardiac total anomalous pulmonary venous drainage. Pediat Radiol. 1992;22:262–3.

    Article  PubMed  CAS  Google Scholar 

  58. Snellen HA, Van Ingen HC, Hoefsmit ECM. Patterns of anomalous pulmonary venous drainage. Circulation. 1968;38:45–63.

    Article  PubMed  CAS  Google Scholar 

  59. Healy JE. Anatomic survey of anomalous pulmonary veins: thier clinical significance. Thorac Surg. 1952;23:443–4.

    Google Scholar 

  60. Kalke BR, Carlson RG, Ferlic RM, Sellers RD, Lillehei CW. Partial anomalous pulmonary venous connections. Am J Cardiol. 1967;20:91–101.

    Article  PubMed  CAS  Google Scholar 

  61. Schatz SL, Ryvicker MJ, Deutsch AM, Cohen HR. Partial anomalous pulmonary venous drainage of the right lower lobe shown by CT scans. Radiology. 1986;159:21–2.

    PubMed  CAS  Google Scholar 

  62. Neill CA, Perenez C, Sabiston DC, Sheldon H. The familial occurence of hypoplastic right long with systemic arterial supply and venous drainage: Scimitar syndrome. Bull John Hopkins. 1960;107:1–21.

    CAS  Google Scholar 

  63. Kuiper-Oosterwal CH, Moulaert A. The scimitar syndrome in infancy and childhood. Eur J Cardiol. 1973;1:55–61.

    PubMed  CAS  Google Scholar 

  64. CIRILLO RL. The scimitar sign. Radiology. 1998;206:623–4.

    PubMed  Google Scholar 

  65. Pennes DR, Ellis JH. Anomalous pulmonary venous drainage of the left upper lobe shown by CT scan. Radiology. 1986;159:23–4.

    PubMed  CAS  Google Scholar 

  66. Julsrud PR, Ehman RL. The “ broken ring ” sign in magnetic resonance imaging of partial anomalous pulmonary venous connection at the superior vena cava. Mayo Clin Proc. 1985;60:874–9.

    Article  PubMed  CAS  Google Scholar 

  67. Vesely TM, Julsrud PR, Brown JJ, Hagler DJ. MR imaging of partial anomalous pulmonary venous connections. J Comput Assist Tomogr. 1991;15:752–6.

    Article  PubMed  CAS  Google Scholar 

  68. Baxter R, McFadden PM, Gradman M, Wright A. Scimitar syndrome: cine magnetic resonance imaging demonstration of anomalous pulmonary venous drainage. Ann Thorac Surg. 1990;50(1):121–3.

    Article  PubMed  CAS  Google Scholar 

  69. Kivelitz DE, Scheer I, Taupitz M. Scimitar syndrome: diagnosis with MR angiography. AJR Am J Roentgenol. 1999;172(6):1700.

    PubMed  CAS  Google Scholar 

  70. Healey JE. Anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Cardiovas Surg. 1952;23:433.

    Google Scholar 

  71. Niwayama G. Cor triatriatum. Am Heart. 1960;J 59:291.

    Article  Google Scholar 

  72. Jegier W, Gibbons JE, Wiglesworth FW. Cor triatriatum: clinical, hemodynamic, and pathologic studies: surgical ­correction in early life. Pediatrics. 1963;31:255.

    PubMed  CAS  Google Scholar 

  73. Edwards JE. Malformations of the coronary vessels: anomalies of the coronary sinus. In: Gould SE, editor. Pathology of the heart. 2nd ed. Springfield: Charles C Thomas; 1960. p. 431.

    Google Scholar 

  74. Loeffler E. Unusual malformation of the left atrium: pulmonary sinus. Arch Pathol. 1949;48:371.

    CAS  Google Scholar 

  75. Grondin C, Leonard AS, Anderson RC, Amplatz KA, Edwards JE, Varco RL. Cor triatriatum: a diagnostic surgical enigma. J Thorac Cardiovasc Surg. 1964;48:527.

    PubMed  CAS  Google Scholar 

  76. Ono Y, Fukuki K, Munakata M, Narita J, Takahata T, Sudo Y, et al. Usefulness of the preoperative MRI for diagnostic and operative method in a case of cor triatriatum. Kyobu Geka. 1996;49(11):921–3.

    PubMed  CAS  Google Scholar 

  77. Horike K, Matsumura C, Egawa Y, Kirino A, Ohshio T, Kawahito T, et al. A case report of cor triatriatum benefit of MRI for preoperative diagnostic and surgical method. Kyobu Geka. 1993;46(12):1063–5.

    PubMed  CAS  Google Scholar 

  78. Sakamoto I, Matsunaga N, Hayashi K, Ogawa Y, Fukui J. Cine-magnetic resonance imaging of cor triatriatum. Chest. 1994;406(5):1586–9.

    Article  Google Scholar 

  79. Kastler BA, Livolsi A, Germain P, et al. MRI in the management of congenital heart disease in newborns and s. Hospimedica. 1991;9:31–41.

    Google Scholar 

  80. Sieverding L, Klose U, Apitz J. Morphological diagnosis of congenital and acquired heart disease by magnetic resonance imaging. Pediat Radiol. 1990;20:311–9.

    Article  PubMed  CAS  Google Scholar 

  81. Didier D, Higgins CB, Fisher MR, Osaki L, Silverman NH, Cheitlin MD. Congenital heart disease: gated MR imaging in 72 patients. Radiology. 1986;158:227–35.

    PubMed  CAS  Google Scholar 

  82. Church WS. congenital malformation of the heart: abnormal septum in left auricle. Trans Pathol Soc (Lond). 1868;19:188.

    Google Scholar 

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Authors and Affiliations

  1. Service de Radiologie A et C, Centre Hospitalier Universitaire Jean Minjoz, bd. Alexander Fleming, 25000, Besançon, France

    Bruno Kastler (Professor of Radiology and Head of Radiology, Associate Professor of Radiology)

  2. Director of Laboratoire I4S (Health, Innovation, Intervention, Imaging, Engineering), Université de Franche-Comté, Besançon, France

    Bruno Kastler (Professor of Radiology and Head of Radiology, Associate Professor of Radiology)

  3. Department of Radiology, Centre Hospitalier Universitaire, Sherbrooke, Québec, Canada

    Bruno Kastler (Professor of Radiology and Head of Radiology, Associate Professor of Radiology)

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Kastler, B. (2011). Anomalous Systemic and Pulmonary Venous Connections. In: MRI of Cardiovascular Malformations. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-30702-0_4

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