Aortic valvular atresia

Abstract

The pathological anatomy of 109 specimens of aortic valvular atresia was reviewed for the purpose of identifying the cardiovascular anomalies associated with that condition. We found the most commonly associated anomaly to be coarctation of the aorta, which was present in 71 percent of our cases and judged to be hemodynamic significance in one-third of the involved cases. Other associated anomalies, in order of decreasing frequency, were mitral atresia, anomalous systemic and pulmonary venous connections, abnormalities of branching of the aortic arch, and ventricular septal defect. The study demonstrated that aortic atresia is associated with a significant incidence of other cardiovascular anomalies. Additional anomalies, when present, may complicate emerging attempts at surgical correction of this condition.

Educational Series in Congenital Heart Disease Congenital left-sided heart obstruction

“…Abnormal flow distribution during foetal life with decreased aortic flow has long been suspected. This is supported by the observation that 71% of cases of aortic atresia also have coarctation ( 38 ). The role of genetic factors is increasingly recognised.…”

“…There is a wide spectrum of anatomy, but there are distinguishable morphologic patterns, based on age at the time of diagnosis: in foetal life, in early infancy and in older children and adults. In the foetus and infant, the following pattern is most often seen: the distal transverse arch (between the left common carotid and left subclavian arteries) is elongated and hypoplastic (tubular hypoplasia), the angle between the transverse arch and ascending aorta is acute and the isthmus is diffusely hypoplastic ( 38 ) with an almost always widely patent arterial duct ( Fig. 17 ).…”