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Part of the book series: Medical Radiology ((Med Radiol Diagn Imaging))

Abstract

Anomalous pulmonary venous returns (APVRs) are congenital malformations in which a portion or the entirety of the pulmonary veins drains into the right atrium or one of its tributaries, resulting in left-to-right (L-to-R) shunt. Partial APVR (PAPVR) is found in 0.4–0.7% of routine necropsy and in 10–15% of patients with atrial septal defect. PAPVRs occur on the right side twice as often as on the left. PAPVRs can be classified in four groups based on their drainage: into supracardiac veins (SVC, azygos vein, vertical vein, left SVC, etc.), into right atrium or coronary sinus, into infradiaphragmatic veins (IVC, portal vein, etc.), and as mixed patterns. PAPVRs may be completely asymptomatic and incidentally discovered, but some will present with large L-to-R shunt requiring surgery. MDCT is currently considered as a first-line technique in the work-up of PAPVRs, as it evidences the anomalous vein and its drainage and shows associated tracheobronchial, lung or vascular abnormalities.

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References

  • Agarwal PP, Seely JM and Matzinger FR (2004) MDCT of anomalous unilateral single pulmonary vein. AJR Am J Roentgenol 183:1241–1243

    PubMed  Google Scholar 

  • Aldridge HE, Wigle ED (1965) Partial anomalous pulmonary venous drainage with intact interatrial septum associated with congenital mitral stenosis. Circulation 31:579–584

    CAS  PubMed  Google Scholar 

  • Alpert JS, Dexter L, Vieweg WV, Haynes FW, Dalen JE (1977) Anomalous pulmonary venous return with intact atrial septum: diagnosis and pathophysiology. Circulation 56:870–875

    CAS  PubMed  Google Scholar 

  • Ammash NM, Seward JB, Warnes CA, Connolly HM, O’Leary PW, Danielson GK (1997) Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography. J Am Coll Cardiol 29:1351–1358

    Article  CAS  PubMed  Google Scholar 

  • Babb JD, McGlynn TJ, Pierce WS, Kirkman PM (1981) Isolated partial anomalous venous connection: a congenital defect with late and serious complications. Ann Thorac Surg 31:540–541

    CAS  PubMed  Google Scholar 

  • Becu LM, Tauxe WN, Dushane JW et al. (1955) Anomalous connection of pulmonary veins with normal pulmonary venous drainage; report of case associated with pulmonary venous stenosis and cor triatriatum. AMA Arch Pathol 59:463–470

    CAS  PubMed  Google Scholar 

  • Benfield JR, Gots RE and Mills D (1971) Anomalous single left pulmonary vein mimicking a parenchymal nodule. Chest 59:101–103

    Article  CAS  PubMed  Google Scholar 

  • Black MD, Shamji FM, Goldstein W et al. (1992) Pulmonary resection and contralateral anomalous venous drainage: a lethal combination. Ann Thorac Surg 53:689–691

    CAS  PubMed  Google Scholar 

  • Blake HA, Hall RJ and Manion WC (1965) Anomalous pulmonary venous return. Circulation 32:406–414

    CAS  PubMed  Google Scholar 

  • Bliss DF 2nd, Hutchins GM (1995) The dorsal mesocardium and development of the pulmonary veins in human embryos. Am J Cardiovasc Pathol 5:55–67

    PubMed  Google Scholar 

  • Brody H (1942) Drainage of the pulmonary veins into the right side of the heart. Arch Pathol Lab Med 33:221–240

    Google Scholar 

  • Brown JW, Ruzmetov M, Minnich DJ et al. (2003) Surgical management of scimitar syndrome: an alternative approach. J Thorac Cardiovasc Surg 125:238–245

    Article  PubMed  Google Scholar 

  • Budorick NE, McDonald V, Flisak ME et al. (1989) The pulmonary veins. Semin Roentgenol 24:127–140

    Article  CAS  PubMed  Google Scholar 

  • Chanoine JP, Viart P, Perlmutter N et al. (1988) Unusual ventilation-perfusion mismatch in partial anomalous venous return. Pediatr Radiol 18:497–498

    Article  CAS  PubMed  Google Scholar 

  • Darling RC, Rothney WB and Craig JM (1957) Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies. Lab Invest 6:44–64

    PubMed  Google Scholar 

  • D’Cruz IA, Arcilla RA (1964) Anomalous venous drainage of the left lung into the inferior vena cava. A case report. Am Heart J 67:539–544

    Article  Google Scholar 

  • Dupuis C, Charaf LA, Breviere GM et al. (1992) The “adult” form of the scimitar syndrome. Am J Cardiol 70:502–507

    Article  CAS  PubMed  Google Scholar 

  • Fish FA, Davies J and Graham TP Jr (1991) Unique variant of partial anomalous pulmonary venous connection with intact atrial septum. Pediatr Cardiol 12:177–180

    Article  CAS  PubMed  Google Scholar 

  • Forbess LW, O’Laughlin MP, Harrison JK (1998) Partially anomalous pulmonary venous connection: demonstration of dual drainage allowing nonsurgical correction. Cathet Cardiovasc Diagn 44:330–335

    Article  CAS  PubMed  Google Scholar 

  • Fraser RS, Müller NL, Colman N et al. (1999) Developmental anomalies affecting the pulmonary vessels. In: Fraser RS, Müller NL, Colman N, Paré PD (eds) Fraser and Paré’s diagnosis of diseases of the chest, 4th edn. Saunders, Philadelphia, pp 637–675

    Google Scholar 

  • Furuya K, Kaku K, Yasumori K et al. (2007) Hypogenetic lung syndrome with anomalous venous return to the left inferior pulmonary vein: multidetector row CT findings. J Thor Imag 22:351–354

    Article  Google Scholar 

  • Galetta D, Veronesi G, Leo F et al. (2006) Anomalous right upper lobe venous drainage. Ann Thorac Surg 82:2272–2274

    Article  PubMed  Google Scholar 

  • Gazzaniga AB, Matloff JM, Harken DE (1969) Anomalous right pulmonary venous drainage into the inferior vena cava and left atrium. J Thorac Cardiovasc Surg 57:251–154

    CAS  PubMed  Google Scholar 

  • Gerlis LM, Ho SY (1989) Anomalous subaortic position of the brachiocephalic (innominate) vein: a review of published reports and report of three new cases. Br Heart J 61:540–545

    Article  CAS  PubMed  Google Scholar 

  • Ghaye B, Szapiro D, Dacher JN et al. (2003) Percutaneous ablation for atrial fibrillation: the role of cross-sectional imaging. Radiographics 23:S19–33

    Article  Google Scholar 

  • Godwin JD, Tarver RD (1986) Scimitar syndrome: four new cases examined with CT. Radiology 159:15–20

    CAS  PubMed  Google Scholar 

  • Goodman LR, Jamshidi A, Hipona FA (1972) Meandering right pulmonary vein simulating the Scimitar syndrome. Chest 62:510–512

    Article  CAS  PubMed  Google Scholar 

  • Gotsman MS, Astley R, Parsons CG (1965) Partial anomalous pulmonary venous drainage in association with atrial septal defect. Br Heart J 27:566–571

    Article  CAS  PubMed  Google Scholar 

  • Grayet D, Ghaye B, Szapiro D et al. (2001) Systemic-to-pulmonary venous shunt in superior vena cava obstruction revealed on dynamic helical CT. AJR Am J Roentgenol 176:211–213

    CAS  PubMed  Google Scholar 

  • Grondin C, Leonard AS, Anderson RC et al. (1964) Cor triatriatum: A diagnostic surgical enigma. J Thorac Cardiovasc Surg 48:527–539

    CAS  PubMed  Google Scholar 

  • Gustafson RA, Warden HE, Murray GF, Hill RC, Rozar GE (1989) Partial anomalous pulmonary venous connection to the right side of the heart. J Thorac Cardiovasc Surg 98:861–868

    CAS  PubMed  Google Scholar 

  • Halasz NA, Halloran KH, Liebow AA (1956) Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 14:826–846

    CAS  PubMed  Google Scholar 

  • Haramati LB, Moche IE, Rivera VT et al. (2003) Computed tomography of partial anomalous pulmonary venous connection in adults. J Comput Assist Tomogr 27:743–749

    Article  PubMed  Google Scholar 

  • Healey JE Jr (1952) An anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Surg 23:433–444

    PubMed  Google Scholar 

  • Hijii T, Fukushige J, Hara T (1998) Diagnosis and management of partial anomalous pulmonary venous connection. A review of 28 pediatric cases. Cardiology 89:148–151

    Article  CAS  PubMed  Google Scholar 

  • James CL, Keeling JW, Smith NM et al. (1994) Total anomalous pulmonary venous drainage associated with fatal outcome in infancy and early childhood: an autopsy study of 52 cases. Pediatr Pathol 14:665–678

    Article  CAS  PubMed  Google Scholar 

  • Kalke BR, Carlson RG, Ferlic RM et al. (1967) Partial anomalous pulmonary venous connections. Am J Cardiol 20:91–101

    Article  CAS  PubMed  Google Scholar 

  • Kastler B, Clair C, Delabrousse E et al. (2002) Retours veineux systémiques et pulmonaires anormaux: aspects IRM et classification. Encycl Méd Chir (Elsevier, Paris) Radiodiagnostic-Coeur-Poumon 32-016-A-20:1–15

    Google Scholar 

  • Kim HJ, Kim HS, Lee G (1994) Anomalous left brachiocephalic vein: spiral CT and angiographic findings. J Comput Assist Tomogr 18:872–875

    Article  PubMed  Google Scholar 

  • Kissner DG, Sorkin RP (1986) Anomalous pulmonary venous connection. Medical therapy. Chest 89:752–754

    CAS  Google Scholar 

  • Marco de Lucas E, Canga A, Sadaba P et al. (2003) Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI. Pediatr Radiol 33:716–718

    Article  PubMed  Google Scholar 

  • Mardini MK, Sakati NA, Nyhan WL (1981) Anomalous left pulmonary venous drainage to the inferior vena cava and through the pericardiophrenic vein to the innominate vein: left-sided scimitar syndrome. Am Heart J 101:860–863

    Article  CAS  PubMed  Google Scholar 

  • Masui T, Seelos KC, Kersting-Sommerhoff BA et al. (1991) Abnormalities of the pulmonary veins: evaluation with MR imaging and comparison with cardiac angiography and echocardiography. Radiology 181:645–649

    CAS  PubMed  Google Scholar 

  • Mathey J, Galey JJ, Logeais Y et al. (1968) Anomalous pulmonary venous return into inferio vena cava and associated bronchovascular anomalies (the scimitar syndrome) Thorax 23:398–407

    Article  CAS  PubMed  Google Scholar 

  • Mehta RH, Jain SP, Nanda NC et al. (1991) Isolated partial anomalous pulmonary venous connection: echocardiographic diagnosis and a new color Doppler method to assess shunt volume. Am Heart J 122:870–873

    Article  CAS  PubMed  Google Scholar 

  • Moes CA, Goldman BS, Mustard WT (1967) Anomalous pulmonary venous drainage from the left lung into a left vertical vein. J Can Assoc Radiol 18:377–381

    CAS  PubMed  Google Scholar 

  • Mohiuddin SM, Levin HS, Runco V, Booth RW (1966) Anomalous pulmonary venous drainage. A common trunk emptying into the left atrium and inferior vena cava. Circulation 34:46–51

    CAS  PubMed  Google Scholar 

  • Nakib A, Moller JH, Kanjuh VI et al. (1967) Anomalies of the pulmonary veins. Am J Cardiol 20:77–90

    Article  CAS  PubMed  Google Scholar 

  • Neill CA (1956) Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return. Pediatrics 18:880–887

    CAS  PubMed  Google Scholar 

  • Okamoto K, Kodama K, Kawai K et al. (2004) An anatomical study of the partial anomalous pulmonary venous return with special references to the bronchial vein. Anat Sci Int 79:82–86

    Article  PubMed  Google Scholar 

  • Peel AA, Blum K, Kelly JC et al. (1956) Anomalous pulmonary and systemic venous drainage. Thorax 11:119–134

    Article  CAS  PubMed  Google Scholar 

  • Pennes DR, Ellis JH (1986) Anomalous pulmonary venous drainage of the left upper lobe shown by CT scans. Radiology 159:23–24

    CAS  PubMed  Google Scholar 

  • Peynircioglu B, Williams DM, Rubenfire M et al. (2005) Endograft repair of partially anomalous pulmonary venous connection with dual drainage. J Vasc Surg 42:1221–1225

    Article  PubMed  Google Scholar 

  • Posniak HV, Dudiak CM, Olson MC (1993) Computed tomography diagnosis of partial anomalous pulmonary venous drainage. Cardiovasc Intervent Radiol 16:319–320

    Article  CAS  PubMed  Google Scholar 

  • Pucelikova T, Kautznerova D, Vedlich D et al. (2007) A complex anomaly of systemic and pulmonary venous return associated with sinus venosus atrial septal defect. Int J Cardiol 115:e47–48.

    Article  PubMed  Google Scholar 

  • Remy-Jardin M, Remy J (1999) Spiral CT angiography of the pulmonary circulation. Radiology 212:615–636

    CAS  PubMed  Google Scholar 

  • Rey C, Vaksmann G, Francart C (1986) Anomalous unilateral single pulmonary vein mimicking partial anomalous pulmonary venous return. Cathet Cardiovasc Diagn 12:330–333

    Article  CAS  PubMed  Google Scholar 

  • Saalouke MG, Shapiro SR, Perry LW et al. (1977) Isolated partial anomalous pulmonary venous drainage associated with pulmonary vascular obstructive disease. Am J Cardiol 39:439–444

    Article  CAS  PubMed  Google Scholar 

  • Sakurai H, Kondo H, Sekiguchi A et al. (2005) Left pneumonectomy for lung cancer after correction of contralateral partial anomalous pulmonary venous return. Ann Thorac Surg 79:1778–1780

    Article  PubMed  Google Scholar 

  • Schatz SL, Ryvicker MJ, Deutsch AM et al. (1986) Partial anomalous pulmonary venous drainage of the right lower lobe shown by CT scans. Radiology 159:21–22

    CAS  PubMed  Google Scholar 

  • Schramel FM, Westermann CJ, Knaepen PJ et al. (1995) The scimitar syndrome: clinical spectrum and surgical treatment. Eur Respir J 8:196–201

    Article  CAS  PubMed  Google Scholar 

  • Sider L, Fisher MR, Mintzer RA (1984) The evaluation of partial anomalous pulmonary venous return with the use of digital subtraction angiography. Chest 86:97–99

    Article  CAS  PubMed  Google Scholar 

  • Snellen HA, Dekker A (1963) Anomalous pulmonary venous drainage in relation to left superior vena cava and coronary sinus. Am Heart J 66:184–196

    Article  CAS  PubMed  Google Scholar 

  • Snellen HA, van Ingen HC, Hoefsmit EC (1968) Patterns of anomalous pulmonary venous drainage. Circulation 38:45–63

    CAS  PubMed  Google Scholar 

  • Sugimoto S, Izumiyama O, Yamashita A et al. (1998) Anatomy of inferior pulmonary vein should be clarified in lower lobectomy. Ann Thorac Surg 66:1799–1800

    Article  CAS  PubMed  Google Scholar 

  • Takada Y, Narimatsu A, Kohno A et al. (1992) Anomalous left brachiocephalic vein: CT findings. J Comput Assist Tomogr 16:893–896

    Article  CAS  PubMed  Google Scholar 

  • Takeda S, Imachi T, Arimitsu K et al. (1994) Two cases of scimitar variant. Chest 105:292–293

    Article  CAS  PubMed  Google Scholar 

  • Takei H, Suzuki K, Asamura H et al. (2002) Successful pulmonary resection of lung cancer in a patient with partial anomalous pulmonary venous connection: report of a case. Surg Today 32:899–901

    Article  PubMed  Google Scholar 

  • Tansel T, Harmandar B, Dayioglu E et al. (2006) Anomalous dual drainage of the right pulmonary veins in a patient with cor triatriatum: report of a case without scimitar sign. J Cardiovasc Surg (Torino) 47:75–78

    CAS  Google Scholar 

  • Thorsen MK, Erickson SJ, Mewissen MW et al. (1990) CT and MR imaging of partial anomalous pulmonary venous return to the azygos vein. J Comput Assist Tomogr 14:1007–1009

    Article  CAS  PubMed  Google Scholar 

  • Tretheway DG, Francis GS, MacNeil DJ et al. (1974) Single left pulmonary vein with normal pulmonary venous drainage: a roentgenographic curiosity. Am J Cardiol 34:237–239

    Article  CAS  PubMed  Google Scholar 

  • Tsao HM, Wu MH, Yu WC et al. (2001) Role of right middle pulmonary vein in patients with paroxysmal atrial fibrillation. J Cardiovasc Electrophysiol 12:1353–1357

    Article  CAS  PubMed  Google Scholar 

  • Tse HF, Lau CP, Lee KL et al. (2003) Atrial tachycardia arising from an epicardial site with venous connection between the left superior pulmonary vein and superior vena cava. J Cardiovasc Electrophysiol 14:540–543

    Article  PubMed  Google Scholar 

  • Van Meter C, Jr., LeBlanc JG, Culpepper WS 3rd et al. (1990) Partial anomalous pulmonary venous return. Circulation 82(5 Suppl):IV195–198

    PubMed  Google Scholar 

  • Vesely TM, Julsrud PR, Brown JJ et al. (1991) MR imaging of partial anomalous pulmonary venous connections. J Comput Assist Tomogr 15:752–756

    Article  CAS  PubMed  Google Scholar 

  • White CS, Baffa JM, Haney PJ et al. (1997) MR imaging of congenital anomalies of the thoracic veins. Radiographics 17:595–608

    CAS  PubMed  Google Scholar 

  • Winter FS (1954) Persistent left superior vena cava; survey of world literature and report of 30 additional cases. Angiology 5:90–132

    Article  CAS  PubMed  Google Scholar 

  • Woodring JH, Howard TA, Kanga JF (1994) Congenital pulmonary venolobar syndrome revisited. Radiographics 14:349–369

    CAS  PubMed  Google Scholar 

  • Yazar F, Ozdogmus O, Tuccar E et al. (2002) Drainage patterns of middle lobe vein of right lung: an anatomical study. Eur J Cardiothorac Surg 22:717–720

    Article  PubMed  Google Scholar 

  • Zylak CJ, Eyler WR, Spizarny DL et al. (2002) Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics 22:S25–43

    Google Scholar 

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Ghaye, B., Couvreur, T. (2009). Partial Anomalous Venous Return. In: Rémy-Jardin, M., Rémy, J. (eds) Integrated Cardiothoracic Imaging with MDCT. Medical Radiology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-72387-5_21

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  • DOI: https://doi.org/10.1007/978-3-540-72387-5_21

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