Abstract
Congenital abnormalities of the major mediastinal venous structures occur in less than 2 % of people who have no other abnormalities (Buirski et al., Clin Radiol 37(2):131–138, 1986; Sungur et al., Heart 93:1292, 2007; Kalke et al., Am J Cardiol 20:91–101, 1967;Snellen and Dekker, Am Heart J 66:184–196, 1963; Pennes and Ellis, Radiology 159:23–24, 1986; Demos et al., Am J Roentgenol 182:1139–1150, 2004). Although many congenital anomalies have been described, three occur with sufficient frequency that they will be encountered in routine clinical practice: azygos or hemiazygos continuation of an interrupted inferior vena cava (IVC), persistence of a left superior vena cava (SVC) with and without a coexisting right SVC, and partial anomalous pulmonary venous return (PAPVR). Although they may occur in adults who have no other abnormalities, these three abnormalities also may be associated with a wide variety of vascular anomalies and cardiac defects (Buirski et al., Clin Radiol 37(2):131–138, 1986; Sungur et al., Heart 93:1292, 2007; Kalke et al., Am J Cardiol 20:91–101, 1967;Snellen and Dekker, Am Heart J 66:184–196, 1963; Pennes and Ellis, Radiology 159:23–24, 1986; Demos et al., Am J Roentgenol 182:1139–1150, 2004). Classifications of systemic and pulmonary venous anomalies are shown in Tables 26.1 and 26.2.
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Buirski G, Jordan SC, Joffe HS, Wilde P. Superior vena caval abnormalities: their occurrence rate, associated cardiac abnormalities and angiographic classification in a paediatric population with congenital heart disease. Clin Radiol. 1986;37(2):131–8.
Sungur M, Ceyhan M, Baysal K. Partial anomalous pulmonary venous connection of left pulmonary veins to innominate vein evaluated by multislice CT. Heart. 2007;93:1292.
Kalke BR, Carlson AG, Ferlic AM, Sellers AD, Lillehei CW. Partial anomalous pulmonary venous connections. Am J Cardiol. 1967;20:91–101.
Snellen HA, Dekker A. Anomalous pulmonary venous drainage in relation to left superior vena cava and coronary sinus. Am Heart J. 1963;66:184–96.
Pennes DR, Ellis JH. Anomalous pulmonary venous drainage of the left upper lobe shown by CT scans. Radiology. 1986;159:23–4.
Demos TC, Posniak HV, Pierce KL, et al. Venous anomalies of the thorax. Am J Roentgenol. 2004;182:1139–50.
Kivistö S, Hänninen H, Holmström M. Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography. J Cardiothorac Surg. 2011;6:126.
Konen E, Raviv-Zilka L, Cohen RA, et al. Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 2003;23:1175–118410.
Uçar T, Fitoz S, Tutar E, et al. Diagnostic tools in the preoperative evaluation of children with anomalous pulmonary venous connections. Int J Cardiovasc Imaging. 2008;24(2):229–35.
White CS, Baffa JM, Haney PJ, et al. Anomalies of pulmonary veins: usefulness of spin-echo and gradient-echo MR images. AJR Am J Roentgenol. 1998;170:1365–8.
Festa P, Ait-Ali L, Cerillo AG, De Marchi D, Murzi B. Magnetic resonance imaging is the diagnostic tool of choice in the preoperative evaluation of patients with partial anomalous pulmonary venous return. Int J Cardiovasc Imaging. 2006;22:685–93.
Valsangiacomo ER, Levasseur S, McCrindle BW, MacDonald C, Smallhorn JF, Yoo SJ. Contrast enhanced MR angiography of pulmonary venous abnormalities in children. Pediatr Radiol. 2003;33:92–8.
Powell AJ, Geva T. Blood flow measurement by magnetic resonance imaging in congenital heart disease. Pediatr Cardiol. 2000;21:47–58.
François CJ, Tuite D, Deshpande V, Jerecic R, Weale P, Carr JC. Pulmonary vein imaging with unenhanced three-dimensional balanced steady-state free precession MR angiography: initial clinical evaluation. Radiology. 2009;250(3):932–9.
Prasad SK, Soukias N, Hornung T, et al. Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage. Circulation. 2004;109(2):207–14.
Greil GF, Powell AJ, Gildein HP, Geva T. Gadolinium-enhanced three-dimensional magnetic resonance angiography of pulmonary and systemic venous anomalies. J Am Coll Cardiol. 2002;39(2):335–41.
Mohrs OK, Petersen SE, Voigtlaender T, et al. Time-resolved contrast-enhanced MR angiography of the thorax in adults with congenital heart disease. AJR Am J Roentgenol. 2006;187(4):1107–14.
Powell AJ, Tsai-Goodman B, Prakash A, Greil GF, Geva T. Comparison between phase-velocity cine magnetic resonance imaging and invasive oximetry for quantification of atrial shunts. Am J Cardiol. 2003;91(12):1523–5.
Debl K, Djavidani B, Buchner S, Heinicke N, et al. Quantification of left-to-right shunting in adult congenital heart disease: phase-contrast cine MRI compared with invasive oximetry. Br J Radiol. 2009;82(977):386–91.
Hillis LD, Firth BG, Winniford MD. Variability of right sided cardiac oxygen saturations in adults with and without left-to-right intracardiac shunting. Am J Cardiol. 1986;58(1):129–32.
Minniti S, Visentini S, Procacci C. Congenital anomalies of the venae cavae: embryological origin, imaging features and report of three new variants. Eur Radiol. 2002;12(8):2040–55.
Kellman GM, Alpern MB, SandIer MA, Craig BM. Computed tomography of vena caval anomalies with embryologic correlation. Radiographics. 1988;8:533–56.
Chen SJ, Liu KL, Chen HY, et al. Anomalous brachiocephalic vein: CT, embryology, and clinical implications. AJR Am J Roentgenol. 2005;184:1235–40.
Steding G, Xu JW, Seidl W, Männer J, Xia H. Developmental aspects of the sinus valves and the sinus venosus septum of the right atrium in human embryos. Anat Embryol (Berl). 1990;181(5):469–75.
Takeda K, Matsumura K, Ito T, Nakagawa T, Yamaguchi N. Anomalous insertion of the superior or the inferior vena cava into the right atrium. Pediatr Cardiol. 1998;19(6):474–6.
Váquez-Pérez J, Frontera-Izquierdo P. Anomalous drainage of the right superior vena cava into the left atrium as an isolated anomaly. Rare case report. Am Heart J. 1979;97(1):89–91.
Moncada R, Demos TC, Marsan RM, Churchill RJ, Reynes C, Love L. CT diagnosis of aneurysms of the thoracic venous system. J Comput Assist Tomogr. 1985;9:305–9.
Kalejs M, Kolitis R, Stradins P, Lacis R. Large thrombosed saccular aneurysm of superior vena cava complicated by pulmonary embolism in a young woman. J Invasive Cardiol. 2010;22(8):E159–61.
Wong AM, Wa YL, Cheung YC, Ng SH, Lee KF. CT features of mediastinal lymphangiohemangioma associated with superior vena cava ectasia: a case report. Acta Radiol. 2000;41:429–31.
Quraishi MB, Mufti O, Wase A. Absent left and right superior vena cava and azygos continuation of inferior vena cava: a rare anomaly of systemic venous return. Eur J Cardiothorac Surg. 2012;41(5):e126.
Webb W, Gamsu G, Speckman J, Kaiser J, Federle M, Lipton M. Computed tomographic demonstration of mediastinal venous anomalies. AJR Am J Roentgenol. 1982;139:157–61.
Mantini E, Grondin CM, Lillehei CW, Edwards JE. Congenital anomalies involving the coronary sinus. Circulation. 1966;33:317–27.
Parikh SR, Prasad K, Iyer RN, Desai N, Mohankrishna L. Prospective angiographic study of the abnormalities of systemic venous connections in congenital and acquired heart disease. Cathet Cardiovasc Diagn. 1996;38(4):379–86.
Sarodia BD, Stoller JK. Persistent left superior vena cava: case report and literature review. Respir Care. 2000;45:411–6.
Rubino M, Van Praagh S, Kadoba K, Pessotto R, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg. 1995;110(3):641–50.
Miraldi F, di Gioia CR, Proietti P, De Santis M, d’Amati G, Gallo P. Cardinal vein isomerism: an embryological hypothesis to explain a persistent left superior vena cava draining into the roof of the left atrium in the absence of coronary sinus and atrial septal defect. Cardiovasc Pathol. 2002;11:149–52.
Raghib G, Ruttenberg HD, Anderson RC, Amplatz K, Adams P, Edwards JE. Termination of left superior vena cava in left atrium, atrial septal defect, and absence of coronary sinus. A developmental complex. Circulation. 1965;31:906–18.
Ezekowitz MD, Alderson PO, Bulkley BH, Dwyer PN, Watkins L, Lappe DL, Greene HL, Becker LC. Isolated drainage of the superior vena cava into the left atrium in a 52-year-old man: a rare congenital malformation in the adult presenting with cyanosis, polycythemia, and an unsuccessful lung scan. Circulation. 1978;58(4):751–6.
Knauth A, McCarthy KP, Webb S, Ho SY, Allwork SP, Cook AC, Anderson RH. Interatrial communication through the mouth of the coronary sinus. Cardiol Young. 2002;12(4):364–72.
Hahm JK, Park YW, Lee JK, Choi JY, Sul JH, Lee SK, et al. Magnetic resonance imaging of unroofed coronary sinus: three cases. Pediatr Cardiol. 2000;21:382–7.
Jian Z, Li J, Xiao Y. Rare association of tetralogy of Fallot with partially unroofed coronary sinus and PLSVC: case report. Thorac Cardiovasc Surg. 2010;58(2):117–9.
Macartney FJ, Zuberbuhler JR, Anderson RH. Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localization. Br Heart J. 1980;44(6):657–67.
Muster AJ, Naheed ZJ, Backer CL, Mavroudis C. Is surgical ligation of an accessory left superior vena cava always safe? Pediatr Cardiol. 1998;19(4):352–4.
Jha NK, Gogna A, Tan TH, Wong KY, Shankar S. Atresia of coronary sinus ostium with retrograde drainage via persistent left superior vena cava. Ann Thorac Surg. 2003;76:2091–2.
Gerlis LM, Gibbs JL, Williams GJ, Thomas GDH. Coronary sinus orifice atresia and persistent left superior vena cava. Br Heart J. 1984;52:648–53.
Winter FS. Persistent left superior vena cava: survey of world literature and report of thirty additional cases. Angiology. 1954;5:90–132.
Ball Jr JB, Proto AV. The variable appearance of the left superior intercostal vein. Radiology. 1982;144:445–52.
McDonald CJ, Castellino RA, Blank N. The aortic “nipple”. Radiology. 1970;96:533–6.
Gilkeson RC, Nyberg EM, Sachs PB, Wiant AM, Zahka KG, Siwik ES. Systemic to pulmonary venous shunt imaging findings and clinical presentations. J Thorac Imaging. 2008;23:170–7.
Takada Y, Narimatsu NA, Kohno A, et al. Anomalous left brachiocephalic vein: CT findings. J Comput Assist Tomogr. 1992;16:893–6.
Chern MS, Ko JS, Tsai A, Wu MH. Aberrant left brachiocephalic vein: CT imaging findings and embryologic correlation. Eur Radiol. 1999;9:1835–9.
Burkill GJ, Burn PR, Padley SP. Aneurysm of the left brachiocephalic vein: an unusual cause of mediastinal widening. Br J Radiol. 1997;70:837–9.
Dudiak CM, Olson MC, Posniak HV. Abnormalities of the azygos system: CT evaluation. Semin Roentgenol. 1989;24:47–55.
Dudiak CM, Olson MC, Posniak HV. CT evaluation of congenital and acquired abnormalities of the azygos system. Radiographics. 1991;11:233–46.
Jelinek JS, Stuart PL, Done SL, Ghaed N, Rudd SA. MRI of polysplenia syndrome. Magn Reson Imaging. 1989;7:681–6.
Munechika H, Cohan RH, Baker ME, Cooper CJ, Dunnick NR. Hemiazygos continuation of a left inferior vena cava: CT appearance. J Comput Assist Tomogr. 1988;12:328–30.
Mata J, Cáceres J, Alegret X, Coscojuela P, De Marcos JA. Imaging of the azygos lobe: normal anatomy and variations. AJR Am J Roentgenol. 1991;156(5):931–7.
Takasugi JE, Godwin JD. Left azygos lobe. Radiology. 1989;171:133–4.
Hatfield MK, Vyborny CJ, MacMahon H, Chessare JW. Congenital absence of the azygos vein: a cause for aortic nipple enlargement. AJR Am J Roentgenol. 1987;149:272–4.
Anderson RH, Brown NA, Moorman AFM. Development and structures of the venous pole of the heart. Dev Dyn. 2006;235:2–9.
Blom NA, Gittenberger-de Groot AC, Jongeneel TH, DeRuiter MC, Poelmann RE, Ottenkamp J. Normal development of the pulmonary veins in human embryos and formulation of a morphogenetic concept for sinus venosus defects. Am J Cardiol. 2001;87:305–9.
Marom EM, Herndon JE, Kim YH, et al. Variations in pulmonary venous drainage to the left atrium: implications for radiofrequency ablation. Radiology. 2004;230:824–9.
Tsao HM, Wu MH, Yu WC, et al. Role of right middle pulmonary vein in patients with paroxysmal atrial fibrillation. J Cardiovasc Electrophysiol. 2001;12:1353–7.
Choi SI, Seo JB, Choi SH, et al. Variation of the size of pulmonary venous ostia during the cardiac cycle: optimal reconstruction window at ECG-gated multi-detector row CT. Eur Radiol. 2005;15(7):1441–5.
Heyneman LE, Nolan RL, Harrison JK, McAdams HP. Congenital unilateral pulmonary vein atresia: radiologic findings in three adult patients. AJR Am J Roentgenol. 2001;177(3):681–5.
Latson LA, Prieto LR. Congenital and acquired pulmonary vein stenosis. Circulation. 2007;115:103–8.
Ou P, Marini D, Celermajer DS, et al. Non-invasive assessment of congenital pulmonary vein stenosis in children using cardiac-non-gated CT with 64-slice technology. Eur J Radiol. 2009;70(3):595–9.
Lee HN, Kim YT, Cho SS. Individual pulmonary vein atresia in adults: report of two cases. Korean J Radiol. 2011;12(3):395–9.
Vanherreweghe E, Rigauts H, Bogaerts Y, Meeus L. Pulmonary vein varix: diagnosis with multislice helical CT. Eur Radiol. 2000;10:1315–7.
Senocak F, Ozme S, Bilgic A, et al. Partial anomalous pulmonary venous return. Evaluation of 51 cases. Jpn Heart J. 1994;35:43–50.
Gustafson RA, Warden HE, Murray GF, Hill RC, Rozar GE. Partial anomalous pulmonary venous connection to the right side of the heart. J Thorac Cardiovasc Surg. 1989;98(5 Pt 2):861–8.
Dillman JR, Yarram SG, Hernandez RJ. Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol. 2009;192(5):1272–85.
Haramati LB, Moche IE, Rivera VT, et al. Computed tomography of partial anomalous pulmonary venous connection in adults. J Comput Assist Tomogr. 2003;27:743–9.
Ho ML, Bhalla S, Bierhals A, Gutierrez F. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 2009;24(2):89–95.
Chintu MR, Chinnappa S, Bhandari S. Aberrant positioning of a central venous dialysis catheter to reveal a left-sided partial anomalous pulmonary venous connection. Vasc Health Risk Manag. 2008;4(5):1141–3.
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(1):259–66.
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;160:375–9.
Almeda FQ, Barkatullah S, Nathan S, Kavinsky CJ. Partial anomalous pulmonary venous drainage of the superior left pulmonary vein into the innominate vein resulting in right ventricular failure. Am J Med. 2002;113(2):168–9.
Otsuka M, Itoh A, Haze K. Sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return evaluated by multislice CT. Heart. 2004;90:901.
Toyoshima M, Sato A, Fukumoto Y, et al. Partial anomalous pulmonary venous return showing anomalous venous return to the azygos vein. Intern Med. 1992;31:1112–6.
Ibrahim M, Burwash IG, Morton B, Brais M. Direct drainage of the right pulmonary into the coronary sinus with intact interatrial septum: a case report. Can J Cardiol. 2001;17:807–9.
Davia JE, Cheitlin MD, Bedynek JL. Sinus venosus atrial septal defect: analysis of fifty cases. Am Heart J. 1973;85:177–85.
Dupuis C, Charaf LA, Breviere GM, Abou P, Remy-Jardin M, Helmius G. The “adult” form of the scimitar syndrome. Am J Cardiol. 1992;70:502–7.
Woodring JH, Howard TA, Kanga JF. Congenital pulmonary venolobar syndrome revisited. Radiographics. 1994;14:349–69.
Celik M, Celik T, Iyisoy A, Ayten O. Scimitar syndrome and azygos continuation of the inferior vena cava Diagnosed in an adult-an unusual association. Congenit Heart Dis. 2012;7(5):E85–8. doi:10.1111/j.1747-0803.2012.00650.x.
Lee ML. Isolated and complex scimitar vein anomalies and their differentiation from the meandering right pulmonary vein. Yonsei Med J. 2007;48(6):973–80.
Shinozaki H, Shimizu K, Anno H, et al. Total anomalous pulmonary vein drainage in an adult diagnosed by helical computed tomography. Intern Med. 1997;36(12):912–6.
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;175:1381–6.
Alphonso N, Norgaard MA, Newcomb A, et al. Cor triatriatum: presentation, diagnosis and long-term surgical results. Ann Thorac Surg. 2005;80:1666–71.
Gharagozloo F, Bulkley BH, Hutchins GM. A proposed pathogenesis of cor triatriatum: impingement of the left superior vena cava on the developing left atrium. Am Heart J. 1977;94(5):618–26.
Tanaka F, Itoh M, Esaki H, Isobe J, Inoue R. Asymptomatic cor triatriatum incidentally revealed by computed tomography. Chest. 1991;100(1):272–4.
Saremi F, Gurudevan SV, Narula J, Abolhoda A. Multidetector computed tomography (MDCT) in diagnosis of “cor triatriatum sinister”. J Cardiovasc Comput Tomogr. 2007;1(3):172–4.
Sakamoto I, Matsunaga N, Hayashi K, et al. Cine-magnetic resonance imaging of cor triatriatum. Chest. 1994;106:1586–9.
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Saremi, F. (2014). Congenital Thoracic Venous Anomalies. In: Saremi, F. (eds) Cardiac CT and MR for Adult Congenital Heart Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8875-0_26
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