Meliota Giovanni, Lombardi Maristella, Zaza Pierluigi, Tagliente Maria Rosaria, Versacci Paolo, Scalzo Gabriele, Vairo Ugo
Department of Pediatric Cardiology, Giovanni XXIII Pediatric Hospital, Bari, Italy.
Department of Peditarics, Obstetrics and Gynecology, Sapienza - University of Rome, Rome, Italy.
Ann Pediatr Cardiol. 2020 Jan-Mar;13(1):91-94. doi: 10.4103/apc.APC_53_19. Epub 2019 Nov 22.
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.
出生后第五主动脉弓持续存在（PFAA）是一种极其罕见且存在争议的心血管畸形。PFAA被定义为一条心包外血管，起自头臂动脉起源近端的升主动脉，通过持续开放的动脉导管终止于降主动脉或肺动脉。具有体肺分流的孤立性PFAA最符合这一定义，而绝大多数报告为PFAA的病例可能有其他胚胎学解释。我们报告了一例独特的5周龄患者，患有具有体肺分流的孤立性PFAA，表现为充血性心力衰竭。首先与远端主肺动脉窗和非典型动脉导管未闭进行鉴别诊断。对该病例的仔细分析和对文献的系统回顾使我们得出结论，该病例为孤立性PFAA，这是有史以来仅有的五例报告病例之一。
