Case presentation
A 50-year-old woman presented to an outpatient cardiology clinic with a chief complaint of chest pain and dyspnoea on exertion. Physical exam was unrevealing. Chest radiograph showed prominence of the right pulmonary vasculature system in a characteristic curvilinear pattern suspicious for scimitar syndrome (Figure 1). Given this finding, electrocardiogram-gated coronary computed tomography angiography was obtained and demonstrated a large right pulmonary vein draining into the inferior vena cava (IVC), confirming the diagnosis of scimitar syndrome (Figure 1). Given her symptoms and a pulmonary-to-systemic flow > 2.14, the patient underwent surgical repair with creation of intra-atrial baffle to connect the anomalous right pulmonary vein to the left atrium. In the 9 years since her surgery, she reports complete resolution of her symptoms.
Figure 1.
Scimitar syndrome on imaging. (A) Posteroanterior chest radiograph with green arrow labelling the curvilinear opacity corresponding to the anomalous pulmonary vein, or ‘scimitar sign’. (B) Computed tomography three-dimensional reconstruction with pulmonary veins coloured in red and pulmonary arteries coloured in blue. A large anomalous right pulmonary vein, the scimitar vein (labelled ‘SV’), is seen emptying into the inferior vena cava (labelled ‘IVC’).
Scimitar syndrome is a rare congenital disorder, occurring in 1–3 per 100 000 live births, in which there is partial anomalous venous return of a right pulmonary vein into the IVC or right atrium. The syndrome is named for the characteristic curvilinear pattern of the anomalous pulmonary vein on chest imaging, which resembles a Turkish sword or ‘scimitar’. The adult form is often asymptomatic and discovered incidentally on imaging. In this case, the diagnosis of scimitar syndrome was suspected initially on plain film radiography, highlighting the importance of careful attention to the chest x-ray in all cases of chest pain.
Consent: The patient has provided written and verbal consent for her images and case details to be published in compliance with COPE guidelines.
Funding: This study was supported by T32 grant for cardiovascular trainee—5T32HL007895-23.
Contributor Information
Elizabeth Hutchins, Division of Cardiology, Department of Medicine, UCLA Medical Center, 757 Westwood Plaza, Los Angeles, CA 90059, USA.
Pooya Bokhoor, Division of Cardiology, Department of Medicine, UCLA Medical Center, 757 Westwood Plaza, Los Angeles, CA 90059, USA.
Data availability
No new data were generated or analysed in support of this research.
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Data Availability Statement
No new data were generated or analysed in support of this research.