Stenting Coarctation of the “Fifth Aortic Arch”: A Safe and Attractive Therapeutic Alternative to Surgery

Abstract

<jats:p> “Persistence of the fifth aortic arch” is a rare congenital cardiovascular anomaly that consists of an abnormal vessel arising from the distal ascending aorta connecting with the systemic or pulmonary circulation. We report a case of a type A interruption of the aortic arch and a coarctation of the fifth aortic arch, which connected the ascending with the descending aorta. No cardiac surgery was required because a covered stent was successfully implanted in the fifth aortic arch when the patient was 4 years old. A chromosome 9 q arm duplication of uncertain significance was also found, an anomaly never described before in this clinical context. </jats:p>

Persistent fifth aortic arch: a comprehensive literature review

“…In our reviewed 104 cases,five patients underwent interventional balloon dilation; among them, two patients experienced long-term restenosis and required secondary balloon dilation or surgery ( 66 , 67 ). Six patients received stent implants, and follow-up results showed normal ventricular ejection function and no definitive stenosis ( 24 , 42 , 47 , 66 , 68 , 69 ).…”

“…Furthermore, some patients with PFAA may exhibit distinct facial and bodily abnormalities in clinical practice, which may be linked to genetic and chromosomal abnormalities, such as 22q11.2 chromosome deletion ( 16 , 20 , 45 , 46 ), PHACE ( 25 ), and Cornelia de Lange syndromes (CdLS) ( 47 ). Based on our literature review, six cases of PFAA were associated with 22q11.2 chromosome deletion syndrome, and they primarily presented with scoliosis ( 48 ), palate abnormalities, facial deformities, hypocalcaemia ( 46 ), T-cell-mediated immune deficiency ( 49 ), and mild-to-moderate growth retardation.…”

“…The patient exhibited typical clinical manifestations of PHACE syndrome, such as frontotemporal cutaneous haemangioma, posterior fossa malformation, arterial abnormality, cardiac defects, and ocular abnormalities ( 52 ). Furthermore, the repeats of 22q11.2 ( 20 ) and 9q ( 47 ) are associated with PFAA. Therefore, clinicians should consider the possibility of PFAA in cases where a patient exhibits distinct facial features and abnormal cardiac haemodynamics.…”

“…PGE1 is used to dilate blood vessels, reduce blood pressure, and prevent platelet aggregation ( 58 ). The surgical procedure typically involves resection of the narrow part or ligation of the fifth arch to reconstruct the fourth arch, patch enlargement of the arch stenosis, and patch augmentation of the fifth arch to replace the fourth arch, sometimes using the Gore-Tex tube grafts or stenting ( 8 , 47 , 59 – 61 ).…”