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Case Reports
. 2020 Jan-Mar;13(1):91-94.
doi: 10.4103/apc.APC_53_19. Epub 2019 Nov 22.

Isolated persistence of the fifth aortic arch in an infant presenting with congestive heart failure

Giovanni Meliota  1 , Maristella Lombardi  1 , Pierluigi Zaza  1 , Maria Rosaria Tagliente  1 , Paolo Versacci  2 , Gabriele Scalzo  1 , Ugo Vairo  1
Affiliations
Case Reports

Isolated persistence of the fifth aortic arch in an infant presenting with congestive heart failure

Giovanni Meliota et al. Ann Pediatr Cardiol. 2020 Jan-Mar.

Abstract

The persistence of the fifth aortic arch (PFAA) in postnatal life is an extremely rare and controversial cardiovascular malformation. PFAA is defined as an extra-pericardial vessel arising from the ascending aorta proximal to the origin of the brachiocephalic arteries, terminating either in the dorsal aorta or in the pulmonary arteries through the persistently patent arterial duct. An isolated PFAA with systemic-to-pulmonary connection best fits this definition, while the vast majority of cases reported as PFAA may have alternative embryological explanations. We present a unique case of a 5-week-old patient with an isolated PFAA with systemic-to-pulmonary connection, who presented with congestive heart failure. A first differential diagnosis was made with distal aortopulmonary window and an atypical patent arterial duct. A careful analysis of the case and a systematic review of the literature made us conclude for an isolated PFAA, which is one of the only five cases ever reported.

Keywords: Aortic arch anomalies; aortopulmonary window; congestive heart failure; persistence of fifth aortic arch.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Freedom's classification of PFAA. Top left: Type 1, systemic-to-systemic PFAA. A case of double lumen aortic arch is shown. Top right: Type 2, systemic-to-pulmonary PFAA, in a case associated with pulmonary atresia. Bottom left: Type 3, pulmonary-to-systemic PFAA associated with left-sided obstructive lesions. A case of PFAA associated with aortic atresia and Type B aortic arch interruption. Bottom right: Type 4, Bilateral PFAA, with right double lumen aortic arch and left PFAA. PFAA: Persistence of fifth aortic arch
Figure 2
Figure 2
Echocardiographic assessment. Panel A. From a modified parasternal short-axis view, a large and short connection (asterisk) is highlighted between ascending aorta and distal pulmonary artery, just proximal to bifurcation, resembling a distal aorto-pulmonary window. Panel B. Subxiphoid long axis view confirms the presence of a large connection (asterisk) between ascending aorta and pulmonary trunk, but points out a well developed aorto-pulmonary septum (arrow-head)
Figure 3
Figure 3
Cardiac catheterization. Panel A. Anterior-posterior projection. A short and large vessel (asterisk) arises proximal to brachiocephalic artery (which is not filled in by contrast, yet) and connects ascending aorta and pulmonary trunk, leading to a significant left-to right shunt. Panel B. Aortography in lateral projection. Contrast flows directly in the abnormal vascular connection (asterisk), which originates beneath brachiocephalic artery (arrow-heads) and has anterior and inferior course to pulmonary artery
See this image and copyright information in PMC

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