SUMMARY . Seventeen heart specimens with aortic arch interruption, a ductus-dependent anomaly, were investigated . In alt specimens the cardiac anatomy favoured a preferential flow into the pulmonary artery (from which the blood could reach the descending aorta through the ductus), Ten specimens had the interruption proximal to the left subclavian artery (type B) and nine of these presented solely with an outflow tract septal defect . On the other hand, six of seven s pecimens with the interruption distal to the left subclavian artery (type A) were associated with more complex heart malformations .The ductus was studied histologically in the 12 available arches without surgical intervention . The structure of the ductal wall, the components of which could extend considerably into the descending aorta and the pulmonary artery, ranged from that seen in a normal, mature ductus with prominent intimal cushions, to a totally abnormal persistent type with no intimal cushions and with marked elastification . This variability in ductal wall structure and the distribution of ductal tissue in the descending aorta and pulmonary artery has clinical and surgical consequences . KEY WORDS : Cardiac anomalies -Ductus arteriosus -Aortic arch interruption -Anatomy and embryologyAbsence of a segment of the aortic arch is a rare anomaly which is often associated with intracardiac abnormalities, predominantly ventricular septal defects (VSDs) . The descending aorta is joined onto the ductus arteriosus and so onto the pulmonary artery .The characteristic clinical picture in a neonate with this anomaly, after a variable period of wellbelng, is of signs and symptoms related to diminished perfusion of the trunk, lower limbs and sometimes upper limbs (low blood pressure as measured in the limbs, renal insufficiency, acidosis) and increased Pulmonary blood flow (heart failure, cyanosis) . As a result of the constriction of the ductus arteriosus, the average age at death of these neonates is only 10 . There has been only a small number of reported longer survivals without surgical intervention, due to an effective collateral circulation with or without a patent ductus arteriosus [6,9, 19,33] .In this paper we present the types of aortic arch interruption and the associated cardiac anomalies of 17 post-mortem specimens . In view of the importance of the ductus arteriosus, histological examination was performed in order to determine whether from the structural characteristics spontaneous closure or persistent patency was to be expected [10] . In four cases complete serial sectioning of the arterial pole informed us about the relation of the ductus to the adjacent elastic arteries . Material, Methods, and DefinitionsThe aortic arch interruptions (17 cases of children born at term : Tables t and 2) were classified (Fig . i) as initially outlined by Celoria and Patton 141, the A segment being the aortic isthmus .
This anatomical study was designed to evaluate the concept that reduced blood flow through the embryonic preductal aorta contributes to the pathogenesis of dimensional aortic arch anomalies. For that purpose the intracardiac anatomy of 151 specimens was examined, of which 22 had an interruption, five atresia, 76 tubular hypoplasia, and 48 local coarctation of the aortic arch. Associated malformations were found in 148 specimens (98%); the remaining three (2%) had isolated local coarctation. Anomalies predisposing to reduced aortic blood flow were present in 128 specimens (85%). Among the potential obstructive factors affecting early morphogenesis, three left ventricular muscular structures seem to be particularly important: (1) the anterolateral muscle bundle, (2) the posteromedial muscle, and (3) leftward deviation of the anterior part of the ventricular septum. Obstructing combinations with these types of anomaly were identified in 77 cases (51%). The embryologic aspects of these muscular structures are discussed.
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