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Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood

Part of: Cardiac Morphology

Published online by Cambridge University Press:  29 August 2017

Michael L. O’Byrne ,
Russell R. Cross  and
Gerard R. Martin
Michael L. O’Byrne*
Affiliation:
Division of Cardiology, Center for Pediatric Clinical Effectiveness, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America Department of Pediatrics Perelman School of Medicine, The University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Russell R. Cross
Affiliation:
Department of Pediatrics, Division of Cardiology Children’s National Medical Center, George Washington University School of Health Sciences, Washington, District of Columbia, United States of America
Gerard R. Martin
Affiliation:
Department of Pediatrics, Division of Cardiology Children’s National Medical Center, George Washington University School of Health Sciences, Washington, District of Columbia, United States of America
*
Correspondence to: M. L. O’Byrne, MD, MSCE, The Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104, United States of America. Tel: +1 215 590 1790; Fax: 1-215-590-4620; E-mail: obyrnem@email.chop.edu
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Abstract

An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called “scimitar vein” without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.

Keywords

CHDCTtranscatheter intervention
Type
Brief Report
Information
Cardiology in the Young , Volume 28 , Issue 2 , February 2018 , pp. 329 - 333
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Copyright
© Cambridge University Press 2017 

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References

1. Dupuis, C, Charaf, LAC, Brevière, GM, Abou, P. “Infantile” form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol 1993; 71: 1326–1330.Google Scholar
2. Gao, Y-A, Burrows, PE, Benson, LN, Rabinovitch, M, Freedom, RM. Scimitar syndrome in infancy. J Am Coll Cardiol 1993; 22: 873–882.Google Scholar
3. Huddleston, CB, Exil, V, Canter, CE, Mendeloff, EN. Scimitar syndrome presenting in infancy. Ann Thoracic Surg 1999; 67: 154–159; discussion 160.CrossRefGoogle ScholarPubMed
4. Dupuis, C, Charaf, LA, Brevière, GM, Abou, P, Rémy-Jardin, M, Helmius, G. The “adult” form of the scimitar syndrome. Am J Cardiol 1992; 70: 502–507.CrossRefGoogle Scholar
5. Argueta-Morales, IR, Meador, LC, Nykanen, DG, DeCampli, WM. Infantile form of Scimitar syndrome with contralateral pulmonary vein stenosis. Pediatr Cardiol 2010; 31: 550–552.Google Scholar
6. Eckhauser, AW, O’Byrne, ML, Weinberg, PM, Ravishankar, C, Spray, TL, Gaynor, JW. Hypoplastic left ventricle and scimitar syndrome. Ann Thoracic Surg 2013; 96: 2232–2234.Google Scholar
7. Vida, VL, Padalino, MA, Boccuzzo, G, et al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation 2010; 122: 1159–1166.Google Scholar
8. Najm, HK, Williams, WG, Coles, JG, Rebeyka, IM, Freedom, RM. Scimitar syndrome: twenty years’ experience and results of repair. J Thorac Cardiovasc Surg 1996; 112: 1161–1168; discussion 1168–1169.Google Scholar
9. Reddy, R, Shah, R, Thorpe, JAC, Gibbs, J. Scimitar syndrome: a rare cause of haemoptysis. Eur J Cardiothorac Surg 2002; 22: 821.Google Scholar
10. Yamakawa, H, Shimizu, K, Michimoto, K, et al. Transcatheter embolization for hemoptysis associated with anomalous systemic artery in a patient with scimitar syndrome. SpringerPlus 2015; 4: S117–S117.Google Scholar
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