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].
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References
Keane JF, Lock JE, Fyler DC, Nadas AS, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders; 2006.
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.
Perloff JK. Clinical. Recognition of congenital heart disease. 4th ed. Philadelphia, PA: WB Sanuders; 1994.
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.
Lilje C, Weiss F, Weil J. Detection of partial anomalous pulmonary venous connection by magnetic resonance imaging. Pediatr Cardiol. 2005;26(4):490–1.
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.
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.
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.
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.
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.
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.
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.
Vanderheyden M, Goethals M, Van Hoe L. Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. 2003;89(7):761.
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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)
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)
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)
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)
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)
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)
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Sadeghpour, A., Alizadehasl, A. (2014). Partial Anomalous Pulmonary and Systemic Venous Connection (PAPVC). In: Sadeghpour, A., Kyavar, M., Alizadehasl, A. (eds) Comprehensive Approach to Adult Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-6383-1_20
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DOI: https://doi.org/10.1007/978-1-4471-6383-1_20
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