ANOMALY OF A VESSEL OF THE AORTIC ARCH (BRACHIOCEPHALIC ARTERY) ASSOCIATED WITH VENTRICULAR SEPTAL DEFECT.

Abstract

The purpose of this paper is to report the association of an anomaly of a vessel of the aortic arch, the brachiocephalic artery, with a defect of the ventricular septum and to suggest a diagnostic relationship when this anomalous vessel is demonstrated in a patient with congenital heart disease. Material At the University of Florida Teaching Hospital, Gainesville, Fla., from May 1960 to May 1963, retrograde aortography was performed in 94 infants and children with a wide variety of congenital malformations of the heart and great vessels. Twenty-three of the 94 children with congenital heart disease had ventricular septal defects which were unassociated with other intracardiac anomalies. The diagnosis was established by clinical or hemodynamic methods in 17 of the 23; by surgical intervention in 3 and by necropsy in the remaining 3 patients. In each of the foregoing children, retrograde aortography was performed because of the known association of a “silent” patent ductus arteriosus with ventricular septal defects (3). In 7 of the 23 children with ventricular septal defects, an associated anomaly which consisted of a large vessel from the apex of the aortic arch, the brachiocephalic artery (Fig. 1), was demonstrated on the aortograms. The vessel was not evident in any of the other 71 patients. The 7 children, 3 Negro boys and 4 white girls, ranged in age from one month to fourteen years. The diagnosis was established by cardiac catheterization in 5 patients, by clinical methods alone in 1, and by surgical intervention in the other. Cineradiographic Anatomy The usual or “normal” configuration of the vessels arising from the aortic arch is the sequential and independent origin of the innominate, the left common carotid, and the left subclavian arteries. This “normal” anatomic relationship was present in 16 of the 23 patients with defects of the ventricular septum. In the remaining 7 cases an identical anomalous arrangement of the vessels of the aortic arch was observed (Fig. 2). The innominate and left common carotid arteries had a common origin, the brachiocephalic artery, which arose from the summit of the aortic arch. This artery was approximately 2 cm. in length, and, in the lateral view, was directed superiorly and slightly posteriorly. It ascended for a short distance along the left lateral aspect of the trachea before bifurcation into the left common carotid and innominate arteries. The left common carotid artery ascended normally. The innominate artery, however, was directed almost transversely to the right, curved around the anterior aspect of the trachea, and then ascended to its usual position. In each patient, the lower trachea was displaced slightly to the right. In 2 cases, the trachea had additional slight dorsal bowing at the level of the innominate artery which was immediately below the thoracic inlet. This displacement of the trachea, without compression, is presumed to be a manifestation of the vascular anomaly.