Right aortic arch, isolated left common carotid and left subclavian arteries, and subclavian steal syndrome: A variant of polysplenia syndrome

The formal pathogenesis of isolated common carotid or innominate arteries: the concept of malseptation of the aortic sac

“…In previous case reports, however, (D'Cruz et al 1966;Levine et al 1966;Shaher et al 1972;Pantke et al 1975;Edwards 1977;Karczenski and Wozniewicz 1978;Martin et al 1979;Park 1979;Crump et al 1981;Nath et al 1981;Garti and Aygen 1982;Fong and Venables 1987;Tozzi et al 1989;Ghalili et al 1990;Huang and Wu 1996;Papagiannis et al 1996) the connecting vascular segment between a pulmonary artery and the isolated left common carotid or left innominate artery was interpreted as the left ductus arteriosus (Fig. 1C, D).…”

“…Thus, irrespective of possible differences in their formal pathogenesis, human cases with isolation of the innominate artery usually tend to show only a single pulmonary-systemic artery at the contralateral side of the descending aorta (D'Cruz et al 1966;Levine et al 1966;Shaher et al 1972;Pantke et al 1975;Edwards 1977;Karczenski and Wozniewicz 1978;Martin et al 1979;Park 1979;Crump et al 1981;Nath et al 1981;Garti and Aygen 1982;Fong and Venables 1987;Papagiannis et al 1996). More promising may be the situation in cases with isolation of the common carotid artery, because this anomaly is associated with an aberrant subclavian artery (Fong and Venables 1987;Tozzi et al 1989;Ghalili et al 1990;Huang and Wu 1996), allowing the presence of a ductus arteriosus on the contralateral side of the descending aorta (Fig.…”

“…Among the known congenital anomalies of the aortic arch arteries, a deficient connection (= isolation) of the common carotid or innominate artery to the aorta is rarely found in man. Based on the literature, only four cases with an isolated common carotid artery (Fong and Venables 1987;Tozzi et al 1989;Ghalili et al 1990;Huang and Wu 1996), and twelve cases with an isolated innominate artery (D'Cruz et al 1966;Levine et al 1966;Shaher et al 1972;Pantke et al 1975;Edwards 1977;Karczenski and Wozniewicz 1978;Martin et al 1979;Park 1979;Crump et al 1981;Nath et al 1981;Garti and Aygen 1982;Fong and Venables 1987;Papagiannis et al 1996) are known to us. The analysis of the published data (Table 1) shows three morphological characteristics: (1) If connected to the aorta, the isolated artery would have been the first branch of the aortic arch.…”

“…1). Based on these schemes, the isolation of a systemic arch artery from the definitive aorta is explained by abnormal obliterations of specific vascular segments proximal and distal to the respective artery, and connections between a systemic and pulmonary artery are explained by deficient obliteration (persistence) of a right or left ductus arteriosus (D'Cruz et al 1966;Levine et al 1966;Shaher et al 1972;Pantke et al 1975;Edwards 1977;Karczenski and Wozniewicz 1978;Martin et al 1979;Park 1979;Crump et al 1981;Nath et al 1981;Garti and Aygen 1982;Fong and Venables 1987;Tozzi et al 1989;Ghalili et al 1990;Huang and Wu 1996;Papagiannis et al 1996;Fig. 1C, D).…”

Left common carotid artery arising from the pulmonary artery in a patient with DiGeorge syndrome.

“…Only six cases have been reported. [1][2][3][4][5][6] Isolation of the left common carotid artery-that is, the left common carotid artery arising from the pulmonary artery, is even rarer, with only two cases having been reported.57 One of them was associated with tetralogy of Fallot.7 The other one was associated with only an atrial septal defect and persistent left superior vena cava. 5 We report a patient with DiGeorge syndrome with tetralogy of Fallot and an isolated left common carotid artery.…”