Scimitar syndrome: diagnostic approach and management

I.Yu. Avramenko, N.S. Kosmynina


The article presents a clinical case of scimitar syndrome in a child, who is 3 years old. The manifestation of clinical signs was observed in a child at the age of one month as paleness, perioral cyanosis and episode of apnea. This child was hospitalized for further examination. The girl had perioral cyanosis, SpO2 90 %, respiratory rate is 36/min. Puerile breathing is auscultative in the lungs, and weakened on the right, on the anterior surface of the chest. Heart tones are rhythmic, systolic murmur is 2/6 at the upper left edge of the chest and 4/6 in the interlobular area on the left. Due to echocardiography it was diagnosed non-critical coarctation of the aorta, a secondary defect of the interventricular septum. Chest X-ray: in the area of the right pulmonary field there is an intense shadow from the apex to the level of the 5th rib with fuzzy edges. What does the child have: dextocardia, dextroposition, pneumonia, atelectasis or thymomegaly? Providing the bronchoscopy, it was revealed the obstruction of the bronchi of the upper and middle parts of the right lung with thick murmur of white color. The bronchial tree was rehabilitated. Saturation was normalized after bronchoscopy. But, despite the elimination of the cause of atelectasis, the control radiography was still unchanged. For further diagnosis, CT scan of the chest with contrast was performed. The child was diagnosed with scimitar syndrome in combination with aortic coarctation and aberrant right subclavian artery. Balloon dilation of aortic coagulation was performed at the age of 2.5 months. At the age of 2 years, surgical correction was performed — elimination of abnormal drainage of the pulmonary veins and the aortic arch plastic. Intraoperative anatomy: mesocardia, right heart sections are dilated, lower part of the right lung is hypoplasic. Our patient did not actually have symptoms characteristic of the scimitar syndrome because there were no signs of heart failure, the girl did not have respiratory disease. A diagnosis for a baby at infancy was made due to an aspiration episode. But atelectasis and mesocardia prevented visualization of the characteristic radiological feature of scimitar syndrome. Therefore, a complete study using contrast-enhanced CT is mandatory for such patients.


scimitar syndrome; children; diagnosis

Full Text:



Golubeva MV, Ilyina NA, Kagan AV. Features of pulmonary circulation in children with congenital pulmonary venolobar syndrom. Regional blood circulation and microcirculation. 2019;18(1):55-65. doi:10.24884/1682-6655-2019-18-1-55-65. (in Russian).

Ciçek S, Arslan AH, Ugurlucan M, Yildiz Y, Ay S. Scimitar syndrome: the curved Turkish sabre. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2014;17(1):56‐61. doi:10.1053/j.pcsu.2014.01.003.

Gustainyte V, Miller M, Towbin T, Towbin AJ, Neville Kucera J. Scimitar syndrome. Appl Radiol. 2019;48(5):37-39.

Al-Shamrani A, AlSadi RS, Elhoury ME, AlHarbi AS. Infantile scimitar syndrome with unusual associations. Saudi Med J. 2017;38(7):764‐767. doi:10.15537/smj.2017.7.18365.

Lark M, Cai A, Rideout P, et al. Novel Technique of Surgical Management of Scimitar Syndrome. Case Rep Cardiol. 2019;2019:6932680. doi:10.1155/2019/6932680.

Di Filippo. Epidemiology and Physiopathology of Scimitar Syndrome. In: Vida V,bed. The Complete Reference for Scimitar Syndrome. Anatomy, Epidemiology, Diagnosis and Treatment. Academic Press; 2017. pp. 57-66. doi: 10.1016/B978-0-12-810406-4.00005-9.

Jackson N, Nokes BT, Sakata K, Cummings K, Vaszar L. An unusual variant of scimitar syndrome predisposing to recurrent pneumonia. Respir Med Case Rep. 2019;26:240‐243. Published 2019 Jan 23. doi:10.1016/j.rmcr.2019.01.010.

Lugones I, García R. A new surgical approach to scimitar syndrome. Ann Thorac Surg. 2014;97(1):353‐355. doi:10.1016/j.athoracsur.2013.05.105.

Brink J, Yong MS, d'Udekem Y, Weintraub RG, Brizard CP, Konstantinov IE. Surgery for scimitar syndrome: the Melbourne experience. Interact Cardiovasc Thorac Surg. 2015;20(1):31‐34. doi:10.1093/icvts/ivu319.

Vida VL, Padalino MA, Boccuzzo G, et al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation. 2010;122(12):1159‐1166. doi:10.1161/CIRCULATIONAHA.109.926204.

Copyright (c) 2020 CHILD`S HEALTH

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.


© Publishing House Zaslavsky, 1997-2020


   Seo анализ сайта