Introduction: Aortic arch anomalies and anatomical variants are relatively common, encompassing a broad spectrum of malformations in embryonic development. Double-lumen aortic arch is one of these anomalies, first described in 1969, and its incidence remains uncertain, as only a few case reports have been published in the literature since then.

Case report: Female infant was born prematurely, with very low birth weight, intrauterine growth restriction and dysmorphic features, without investigation of congenital heart disease during the prenatal follow up. After birth, transthoracic echocardiogram was performed, revealing a wide perimembranous ventricular septal defect and patent ductus arteriosus. Patient developed signs and symptoms of heart failure within the first few weeks of life, in this manner, anti-congestive therapy was initiated. Due to the persistence of that scenario, she was referred to a tertiary center for cardiac surgery. Following referral, echocardiography was repeated, confirming the previously described findings, but additionally revealed a hyperrefringent image along the transverse aortic arch, apparently septating that. Pre-operatory computed tomography angiography confirmed the presence of a double-lumen aortic arch, with no additional aortic arch anomalies. Patient underwent palliative pulmonary artery banding and ductus arteriosus ligation, and after recovery was discharged in good condition for outpatient follow-up. Conservative approach was adopted in relation to the aortic arch anomaly, given that the condition caused no hemodynamical repercussion.

Discussion and conclusions: Double lumen aortic arch is a rare and possibly underdiagnosed anomaly, especially in the absence of associated heart defects, since it has no clinical manifestation. Despite significant advances in imaging modalities, its diagnosis by echocardiography remains challenging. Insufficient awareness of the anomaly may hinder—or even preclude—the performance of hemodynamic procedures via cardiac catheterization, which could be required for unrelated clinical indications later in the patient’s life. Although first described more than five decades ago, this entity is still not fully understood. A thorough understanding of the anomaly, along with familiarity with the embryological mechanisms involved, may enhance diagnostic accuracy and facilitate earlier recognition in clinical practice.

Vanessa Ferrari is a specialist in pediatric cardiology and echocardiography, with clinical practice in leading referral centers for pediatric cardiac surgery in Salvador – Bahia – Brazil. Additionally, she is part of the preceptorship for the pediatric cardiology and echocardiography medical residency program at Martagão Gesteira Hospital, the largest exclusively pediatric hospital in the North and Northeast regions of the country.