Giovanni Meliota1, Maristella Lombardi1, Pierluigi Zaza1, Maria Rosaria Tagliente1, Paolo Versacci2, Gabriele Scalzo1, Ugo Vairo1
1 Department of Pediatric Cardiology, Giovanni XXIII Pediatric Hospital, Bari, Italy
2 Department of Peditarics, Obstetrics and Gynecology, Sapienza - University of Rome, Rome, Italy
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.
Dr. Giovanni Meliota
Department of Pediatric Cardiology, Giovanni XXIII Paediatric Hospital, Via Amendola 207, 70126 Bari
Source of Support: None, Conflict of Interest: None
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