BY 4.0 license Open Access Published by De Gruyter June 22, 2021

Scimitar syndrome

William Dennehy and Eric M. Neverman

A 42 year old woman presented in December 2018 to a primary care clinic with six weeks of rhinorrhea and cough following a viral respiratory infection. She was diagnosed with acute bacterial sinusitis and treated with amoxicillin. Her evaluation included a chest radiograph notable for a curvilinear structure of the right mid- to lower lungs (Image A). Subsequent investigations included computed tomography with contrast notable for anomalous pulmonary venous drainage of the right upper, middle, and lower lobes with drainage into the inferior vena cava (Image B). An echocardiogram demonstrated normal atrial anatomy and size without pulmonary hypertension or atrial septal defect. The patient’s symptoms resolved completely following completion of 10 days of antibiotic therapy.

Image A:

Image A:

Image B:

Image B:

Scimitar syndrome is a rare form of congenital heart disease that consists of total or partial anomalous venous drainage of the right lung to the inferior vena cava, right lung hypoplasia, and variable pulmonary arterial supply to the right lung [1]. The term “scimitar” refers to a curved Turkish sword, a term first used by Halasz et al. in 1956 to describe the curvilinear radiographic findings of the anomalous vein [2]. The severity and clinical presentation of this syndrome often depend on age of diagnosis and the presence of other potentially complex congenital heart disease [3], most commonly an atrial septal defect [4, 5]. Infants and children most often present with pulmonary hypertension or heart failure, whereas adults may remain asymptomatic with discovery only as an incidental finding [4, 6]. Treatment and monitoring is dependent upon the underlying pathology and the patient’s symptoms, with surgical correction often indicated in patients with symptoms related to pulmonary overcirculation [3].


Corresponding author: Eric M. Neverman, DO, MHA, UnityPoint Clinic, 101 E J Ave, Suite 120, Grundy Center, IA 50638, USA, E-mail:

  1. Author contributions: Both authors provided substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; both authors drafted the article or revised it critically for important intellectual content; both authors gave final approval of the version of the article to be published; and both authors agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  2. Research funding: None reported.

  3. Competing interests: None reported.

References

1. Vida, V. The complete reference for scimitar syndrome: anatomy, epidemiology, diagnosis and treatment. London: Academic Press; 2017. Search in Google Scholar

2. Halasz, NA, Halloran, KH, Liebow, AA. Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 1956;14:826–46. Search in Google Scholar

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Received: 2021-05-04
Accepted: 2021-05-24
Published Online: 2021-06-22

© 2021 William Dennehy and Eric M. Neverman, published by De Gruyter, Berlin/Boston

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