Scimitar syndrome and horseshoe lung presenting with syncope and hemoptysis in late childhood, management challenges, and outcomes: a case report.

BACKGROUND: Horseshoe lung (HL) and scimitar syndrome are rare congenital cardiopulmonary anomalies that may coexist, posing complex diagnostic and management challenges. The following case presents the clinical manifestation of repeated hemoptysis episodes and syncope, a combination that has rarely been reported in this context. Moreover, there were treatment challenges regarding the utility of minimally invasive techniques in the iatrogenic occlusion of aberrant vessels, which has more recently been suggested in the literature to yield comparable outcomes compared with open surgeries.

CASE PRESENTATION: We described the case of a 15-year-old Iranian boy who presented with syncope and hemoptysis following physical activity. Imaging findings were consistent with concomitant HL, partial anomalous pulmonary venous return (PAPVR), right lung hypoplasia, and scimitar syndrome. The patient successfully underwent transcatheter arterial embolization (TAE); however, another episode of hemoptysis occurred after three weeks. This recurrence indicated the failure of TAE and necessitated a right lower lobe segmentectomy. After the surgical excision, the patient showed improvement in respiratory status, with no further episodes of hemoptysis. He was discharged on postoperative day seven. At the 2-month follow-up, there were no signs of recurrence or complications. Nonetheless, the patient was scheduled for subsequent open-heart repair of the PAPVR defect.

CONCLUSIONS: This case highlights that when HL or scimitar syndrome is identified, clinicians should be aware of associated congenital cardiopulmonary and vascular anomalies and perform a comprehensive clinical exam and multimodality imaging. Moreover, although TAE may yield outcomes comparable to open surgery with faster recovery and fewer postoperative complications, its effectiveness is questionable in patients with complex underlying conditions. Therefore, although the choice between the minimally invasive and invasive managements requires a multidisciplinary approach, it is recommended that patients with complications such as infections, bronchiectasis, large lumen aberrant vessels, multiple aberrant vessels, or unusual anatomies be planned for surgical resection of sequestrated lung tissue to avoid potentially less effective interventions.