A case of cervical aortic arch is reported. To the best of our knowledge, it is the first to be associated with a serious intracardiac anomaly. In (Beavan and Fatti, 1947;Bender, Menges, and Shulze, 1964;Chang et al., 1971;Defrenne and Verney, 1968;De Jong and Klinkhamer, 1969;DuBrow et al., 1974;Gravier, Vialtel, and Pinet, 1959;Harley, 1959;Hastreiter, D'Cruz, and Cantez 1966;Mahoney and Manning, 1964;Massumi, Wiener, and Charif, 1963; Muflins, Gillete, and McNamara, 1973;Pitzus and Camoglio, 1974;Richie et al., 1972;Sheperd, Kerth, and Rosenthal, 1969;Shuford et al., 1972). Right-sided in the majority of instances, it has been found only as an isolated entity. The purpose of this article is to describe a case associated with pseudotruncus and which constitutes a new type of asymptomatic double aortic arch.Case report A 33-month-old undernourished male child,cyanotic since the age of 1 month, with a history of several upper respiratory infections was admitt3d for evaluation. Pertinent physical findings were a systolic thrill over the left supraclavicular region and a superficial venous network over face, neck, and upper trunk. Peripheral pulses were normal. Laboratory studies were within normal limits except for haematocrit (63%) and haemoglobin (17*3 g/100 ml). Electrocardiogram showed obvious right axis deviation, right atrial overload, pronounced right ventricular hypertrophy, and systolic overload. Phonocardiogram showed a modest systolic murmur at the second and third interspaces and an increased second heart sound. On chest x-ray film the heart was slightly enlarged; the apex was rounded and tilted upward. The pulmonary artery segment was concave and the hilar and pulmonary vascular markings were decreased. In the left anterior oblique projection the aortic knob was unusually high and prominent. Lungs were clear. At cardiac catheterization very low oxygen saturation values were found both in the venous (34-8% in right atrium) and arterial systems (38&9% in ascending aorta). The pulmonary artery could not be entered. The right ventricular pressure was 85/3-5 mmHg (11'3/04-O07 kPa).
CineangiocardiogramThe right infundibulum wrs atretic. A dilated ascending aorta overrode a large ventricular septal defect. The aortic arch was right-sided. The first aortic branch crossed the midline, ascended to the left side of the neck, then turned abruptly downward and rejoined the aorta at the level of the rightsided arch. The second and third branches were, respectively, the right subclavian and right common carotid arteries. The left brachiocephalic arteries could not be properly assessed, but the left common carotid artery seemed to arise from the descending portion of the left cervical arch. A hypoplastic main pulmonary artery was supplied by a small persistent ductus arteriosus which originated from the