Transcatheter Embolization for Hemoptysis Associated with Anomalous Systemic Artery in a Patient with Scimitar Syndrome

Background: Scimitar syndrome can present with a wide clinical spectrum of symptoms either early in the neonatal period or later in life.

Case Description: We report a case of a 62-year-old woman with anomalous systemic arterial supply to the basal lung with scimitar syndrome presenting as recurrent hemoptysis. Bronchoscopy revealed normal major bronchial branches without bronchial atresia, indicating that sequestration of the lung was not confirmed. The anomalous drainage of the scimitar vein was to the inferior vena cava, and an anomalous artery from the aorta supplied the right basal lung. There were no findings of pulmonary hypertension and arteriovenous malformation such as an anomalous artery to the scimitar vein. The distal portions of anomalous arteries were embolized using gelatin sponge particles and the proximal portion was embolized using fibered detachable coils. Although a small pulmonary infarction was observed as a complication, the patie nt has not experienced any subsequence recurrence of the hemoptysis during a follow-up period of 6 months.

Discussion And Evaluation: Deformities of the blood vessels and the lungs are frequently complex in scimitar syndrome. Although patients treated with surgical repair of this disorder may be at higher risk than those treated less invasively, we believe that transcatheter embolization was a useful strategy for the treatment of the anomalous systemic arterial supply to the basal lung, particularly in this patient with scimitar syndrome.

Conclusion: Hemoptysis in a patient with scimitar syndrome associated with anomalous systemic arterial supply to the basal lung was successfully treated with transcatheter arterial embolization. However, it might be better to avoid the use of gelatin sponge particles in patients with a similar anomaly without pulmonary artery distribution because of the possibility of causing severe pulmonary infarction.