Persistent fifth aortic arch: a single-center experience, case series

Abstract

Background: Persistent fifth aortic arch (PFAA) is an extremely rare congenital cardiovascular malformation and there is limited data in the literature. The objective of this study is to enhance our understanding and diagnosis of PFAA from echocardiography and computed tomography angiography (CTA) findings, and to evaluate the application of echocardiography in the diagnosis of PFAA.Methods: We retrospectively reviewed five cases of PFAA diagnosed from October 2016 to September 2019 at the Affiliated Children's Hospital of Capital Institute of Pediatrics. We described their diagnosis by echocardiography and CTA findings, and medical history.Results: Five cases of PFAA were identified in the study. Patients aged from 3 to 48 months and weighed from 4 to 12 kg presented different clinical symptoms upon clinical examination. All the patients completed a primary echocardiographic assessment; however, the first two patients were misdiagnosed by echocardiography and was confirmed by supplemental CTA while the other three patients were directly diagnosed by echocardiography. Surgery was necessary for three patients, two of whom accepted and one refused. The other two patients only needed a follow-up assessment, which showed good results. Conclusions:The clinical manifestation of PFAA in our patient population was atypical, and their diagnosis depended on the use of echocardiography. In the case of uncertainty, the final diagnosis was confirmed by CTA. Although the nomenclature and embryonic origin of PFAA remains controversial, the accurate diagnosis of aortic arch abnormalities and associated malformations are imperative for time-sensitive treatments.

Confusion from prenatal diagnosis: Type C persistent fifth aortic arch or right‐side ductus arteriosus

“…The existence of the fifth arch artery remains debatable because of the lack of evidence from amniote embryology to prove the existence of a transient "5th" arch/pouch. 8 However, some type A or B fifth bows can be found in children or adults, 9,10 enabling us to find definitive evidence of the embryonic development of an immortal fifth arch in the future to support our conclusions.…”

Persistent fifth aortic arch: a comprehensive literature review

“…Furthermore, echocardiography can be used to assess the systolic function of blood vessels preoperatively and postoperatively. However, complex extracardiac anatomy may lead to missed or incorrect diagnoses ( 8 , 10 ). Computed tomography angiography (CTA) is considered to be more accurate than ultrasound in showing vascular origination, branching, aortic arch complexities, surrounding blood vessels, and their spatial structure.…”

Aortic Arch Variants and Anomalies: Embryology, Imaging Findings, and Clinical Considerations

“…PFAA arises proximal to the origin of the BCA, has an extra pericardial tortuous course, and terminates in the dorsal aorta or pulmonary artery. 76) …”

“… 78) 79) PFAA has various clinical presentations depending on the anatomical variations, including vascular ring formation, connection between the systemic and pulmonary vascular system, and combined anomalies. 76) For example, the LSA arising from the fifth aortic arch and persistence of a large posterior aortic diverticulum can result in a complete vascular ring, which can cause symptoms. 80) …”