4 10 11 12 13 14 15 16 17 1974 1975 1980 1983 1985 1985 1987 1987 1989 1990 1991 1997 1997 1998 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 1999 1999 2002 2003 2004 2004 2005 2006 2006 2006 2006 2007 2007 2009 2009 2009 2011 2011 2011 2012 2012 2013 2013 2015 2016 2017 2015 Moes Lawrence Hirano Diaz- Gongora Cabrera Culham Gerlis Herrera Gerlis Geva Gibbin Einzig Donti Marinho-daSilva Wanga Lambert McMahon Yang Suda Parmara Park Baranowska Johnston Lee Khan Kirsch Bernheimer Santoro Kligerman Akhfash Linhares Khajali Camarda Tehrai Furtado Murugan Warrier Schicchi Naimo Tretter Nicholsona 1 1 1 1 1 1 Double-barreled aorta with patent arterial duct Double-barreled aorta with patent arterial duct Double-barreled aorta Double-barreled aorta with patent arterial duct Double- barreled aorta with patent arterial duct and coarctation Double-barreled aorta with coarctation Double-barreled aorta Double-barreled aorta Double-barreled aorta Double-barreled aorta Double-barreled aorta with coarctation Double-barreled aorta Double-barreled aorta Double-barreled aorta 15 16 17 18 19 20 21 22 23 24 25 26 27 28 1 1 1 1 1 1 1 1 1 1 1 1 Double-barreled aorta (right) and left pulmonary artery from aorta Double-barreled aorta with patent arterial duct and coarctation Double-barreled aorta Double-barreled aorta Double-barreled aorta with coarctation Double-barreled aorta (left) and left pulmonary artery from aorta Double-barreled aorta Double-barreled aorta with patent arterial duct Double-barreled aortic arch Double-barreled aorta Double-barreled aorta with coarctation and patent arterial duct Double-barreled aorta with patent arterial duct Double-barreled aorta Double-barreled aorta with coarctation Double-barreled aorta Double-barreled aorta with coarctation Double-barreled aorta Double-barreled aorta with coarctation Double-barreled aorta Double-barreled aorta Double-barreled aorta Double-barreled aorta Double-barreled aorta with patent arterial duct Double-barreled aorta with coarctation Double-barreled aorta Double-barreled aorta Double-barreled aorta (left) and left pulmonary artery from aorta 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 aCases that we interpret as requiring dual or bilateral fifth arch arteries to explain the anatomy FIG 48.7 Echocardiographic image obtained from a patient with a double-barreled aorta showing that the origin of the lower channel is opposite or distal to the take-off of the persistently patent arterial duct (Video 48.1) This makes an interpretation on the basis of persistence of a collateral channel just as likely if not more so than the alternative explanation of persistence of the artery of the fifth pharyngeal arch Aortic-to-Pulmonary Arterial Communications (Table 48.2) Aortic-to-Pulmonary Arterial Confluence When assessing the alleged findings of fifth arch arteries in developing human embryos, Congdon stated that “if it is established that these vessels are true fifth arches, their usual termination would indicate strongly that the upper end of the pulmonary arch is the homologue of the distal portion of the fifth.”2 We take this statement to indicate that if it is a fifth arch artery that feeds the pulmonary arteries, it must incorporate within the channel the dorsal component of one of the arteries of the sixth arch This inclusion of the patent arterial duct as part of the channel may explain the response to prostaglandin E1 infusion in some cases It is difficult to account for these abnormal connections on the basis of our current understanding of the development of pharyngeal arch arteries without invoking the presence of a fifth arch artery or, alternatively, a collateral channel.3,7–12 In either event, to qualify as fifth arch arteries, the channels must incorporate part of the original sixth arch arteries to feed the pulmonary confluence, and hence take a serpentine course Thus termination at the confluence of the pulmonary arteries provides the strongest support for inclusion of the fifth arch artery The channels must have an unequivocal origin from the ascending aorta proximal to the brachiocephalic artery rather than the transverse segment of the aortic arch Extensive remodeling of the arterial duct, nonetheless, can also provide an equally appropriate explanation in some of the reported cases.68,70 Table 48.2 All the Cases Published in the World Literature That Have Implicated Persistence of Artery of the Fifth Arch Producing Various Forms of Aortic-to-Pulmonary Arterial Connections Sl No Year Author Case Anatomy of the Putative Fifth Arch Reference 1974 1987 1987 1989 1 of 8 cases Aorta to pulmonary artery confluence Aorta to pulmonary artery Aorta to right pulmonary artery Aorta to pulmonary artery confluence mimicking distal AP window Aorta to pulmonary artery confluence Aorta to pulmonary artery confluence mimicking distal AP window Double-barreled aorta (right) and left pulmonary artery from aorta Aorta to pulmonary artery confluence mimicking distal AP window Aorta to pulmonary artery confluence mimicking distal AP window Aorta to pulmonary artery confluence mimicking distal AP window Aorta to pulmonary artery confluence mimics distal AP window Double-barreled aorta (left) and left pulmonary artery from aorta Aorta to pulmonary artery confluence mimicking distal AP window Aorta to pulmonary artery confluence mimicking distal AP window Aorta (lateral aspect) to pulmonary artery confluence Left pulmonary artery from aorta 56 57 21 23 Macartney Freedom Gerlis Gerlis 1993 Yoo 1995 Wu 1 1999 Wanga 1999 Lee 2000 Chiu 10 2000 Chiu 11 2003 Hwang 12 2004 Parmara 13 2006 Khan 14 2006 Khan 15 16 2007 Lee 2007 Koch 1 58 59 29 38 60 60 61 34 39 39 62 63