A Rare Case Presented with Asthma Symptoms: Scimitar Syndrome

Hatice Çelik Tuğlu¹, Önder Öztürk^1, , Merve Ergün², Mustafa Karabacak³, Veysel Atilla Ayyıldız⁴ and Ahmet Akkaya¹

¹Department of Chest Diseases, Medical School of Süleyman Demirel University, Isparta, Turkey

²Medical School of Süleyman Demirel University, Isparta, Turkey

³Department of Cardiology, Medical School of Süleyman Demirel University, Isparta, Turkey

⁴Department of Radiology, Medical School of Süleyman Demirel University, Isparta, Turkey

Cite this paper

Hatice Çelik Tuğlu, Önder Öztürk, Merve Ergün, Mustafa Karabacak, Veysel Atilla Ayyıldız and Ahmet Akkaya. A Rare Case Presented with Asthma Symptoms: Scimitar Syndrome. American Journal of Medical Case Reports. 2019; 7(11):280-283. doi: 10.12691/AJMCR-7-11-5

Abstract

Background: Scimitar syndrome is a rare constellation of congenital conditions pertaining to partial anomalous pulmonary venous return to the inferior vena cava, which may associate with variable right lung hypoplasia, right pulmonary artery hypoplasia, pulmonary sequestration together with the presence of aortopulmonary collaterals from the descending aorta towards the right lung. Depending on the severity of the defect, it can present as early as in neonatal period or occasionally later in life. In many cases, there are also other associated cardiac anomalies. Case characteristics: 23-year-old woman with recurrent episodes of breathlessness and wheezing symptoms since her childhood. Computed tomography angiogram revealed right pulmonary veins draining into inferior vena cava through a single vein with a single ostium diagnosed as Scimitar syndrome. Outcome: Successfully managed with surgical correction. Message: Scimitar syndrome should be considered in adulthood with asthma symptoms if it is not under control.

Keywords

scimitar syndrome, dyspnea, asthma, pulmonary vein anomalies

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	Dupuis, C., Charaf, L.A., Brevière, G.M., Abou, P., Rémy-Jardin, M., Helmius, G. The “adult” form of the scimitar syndrome, Am. J. Cardiol. 1992; 70(4): 502-507.
[2]	Jackson, N., Nokes. B.T., Sakata, K., Cummings, K., Vaszar, L. An unusual variant of scimitar syndrome predisposing to recurrent pneumonia. Respir Med Case Rep. 2019; 26: 240-243.
[3]	Khan, S.A., Ahmad, Z., Scimitar syndrome: A rare case of recurrent pneumonia. J Pak Med Assoc. 2019; 69(3): 423-425.
[4]	Midyat, L., Demir, E., Aşkin, M., Gülen, F., Ulger, Z., Tanaç, R., Bayraktaroğlu, S. Eponym. Scimitar syndrome. Eur J Pediatr. 2010; 169(10): 1171-1177.
[5]	Juraszek, A.L., Cohn, H., Van Praagh, R., Van Praagh, S. Isolated left-sided scimitar vein connecting all left pulmonary veins to the right inferior vena cava. Pediatr Cardiol. 2005; 26(06): 846-847.
[6]	Fraser, R.S., Müller, N.L., Colman, N., Parè, P.D. Diagnosis of Diseases of the Chest. 4th ed. Philadelphia: WB Saunders Company, 1999, 653-655.
[7]	Clements, B.S, Warner, J.O. Pulmonary sequestration and related congenital bronchopulmonary vascular malformations: no men clature and class ification based on anatomical and embryological considerations. Thorax 1987; 42: 401-408.
[8]	Neill, C.A., Ferenca, C., Sabiston, D.C. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous return, “scimitar syndrome” Bull Johns Hopkins Hosp. 1960; 107: 1-21.
[9]	Vida, V.L., Padrini, M., Boccuzzo. G., Agnoletti, G., Bondanza, S., Butera, G., Chiappa, E., Marasini, M., Pilati, M., Pongiglione, G., Prandstraller, D., Russo, M.G., Castaldi, B., Santoro, G., Spadoni, I., Stellin, G., Milanesi, O; Italian Society of Pediatric Cardiology. Natural history and clinical outcome of “uncorrected” scimitar syndrome patients: a multicenter study of the italian society of pediatric cardiology. Rev Esp Cardiol (Engl Ed). 2013; 66(7): 556-60.
[10]	Korkmaz, A.A., Yildiz, C.E., Onan, B., Guden, M., Cetin, G., Babaoglu, K. Scimitar syndrome: a complex form of anomalous pulmonary venous return, J. Card. Surg. 2011; 26 (5): 529-534.
[11]	Oshima, Y., Hashimoto, I., Shimazu, C., Ichida, F. Atypical infantile form of scimitar syndrome with bronchomalacia. Interact Cardiovasc Thorac Surg. 2003; 2(03): 298-300.
[12]	Winant, A.J., Cho, J., Alyafei, T.S., Lee, E.Y. Pediatric thoracic anatomic variants: what radiologists need to know. Radiol Clin 2017; 55: 677-691.
[13]	Woodring, J.H., Howard, T.A., Kanga, J.F. Congenital pulmonary venolobar syndrome revisited. Radiographics. 1994; 14: 349-369.
[14]	Örün, U.A., Özgür, S., Ceylan, O., Karademir, S., Şenocak, F., Yilmaz, O., Keskin, M. Horseshoe Lung and Scimitar Syndrome Associated with Congenital Hydrocephalus in an Infant: Case Report. Turkiye Klinikleri Cardiovascular Sciences. 2012; 24: 83-87.
[15]	Sun, J., Zhang, S., Jiang, D., Yang, G. Scimitar syndrome with the left persistent superior vena cava. Surg Radiol Anat. 2009; 31: 307-309.
[16]	Wang, C.C., Wu, E.T., Chen, S.J., Lu, F., Huang, S.C., Wang, J.K., Chang, C.I., Wu, M.H. Scimitar syndrome: Incidence, treatment and prognosis. Eur J Pediatr. 2008; 167:155-60.
[17]	Jackson, N., Nokes, B.T., Sakata, K., Cummings, K., Vaszar, L. An unusual variant of scimitar syndrome predisposing to recurrent pneumonia. Respir Med Case Rep. 2019; 26: 240-243.
[18]	Huddleston, C.B., Exil, V., Canter, C.E., Mendeloff, E.N. Scimitar syndrome presenting in infancy. Ann Thorac Surg. 1999; 67: 154-159.
[19]	Ward, K.E., Mullins, C.E. Anomalous pulmonary venous connections, pulmonary vein stenosis, and atresia of the common pulmonary vein. In: The Science and Practice of Pediatric Cardiology, Garson A Jr, Bricker JT, Fisher DJ, Neish SR (Eds), Williams and Wilkins, Baltimore, 1998, 1431.
[20]	Zagal, B., Book, S., Krasuski, R.A. Late “adult form” scimitar syndrome presenting with “infant form” complications J Invasive Cardiol. 2006; 18: E82-5.