Familial Aspects of Coarctation of the Aorta

Coarctation of the thoracic aorta in adults: A follow-up study after surgical treatment

“…Evidently, these bicuspid valves are often associated with AR (75, 157). Increasing attention has recently been directed at the effect of coexisting and initially haemodynamically insignificant aortic valvular disease on the long-term prognosis of CoA patients (12,15,31,157 into account the fact that the follow-up time in the studies cited is longer than in the present study, a good agreement can be seen. It is not known whether the incidence would be less if the CoA repair were performed at an early age; the follow-up times for such cases are not yet long enough (156).…”

“…The finding of a gradient equal to or below 20 mmHg in this study seems to be of little practical significance; these patients had the same frequency of hypertension as those with no gradient between arm and leg. The incidence of residual or recurring CoA in this series is less than usually reported in the literature, where the average is about 1 l 0 / o (86,106,108,149,157). The fact that these studies include children, who are more susceptible to this complication, may help to explain the difference.…”

“…In one account of 197 closely monitored patients, seven of the nine postoperative deaths occurring 4 to 18 years postoperatively were ascribed to coexisting aortic valvular disease (91); no manifestations of coronary artery disease were noted. In another series of 190 cases, 11 cardiovascular deaths were reported one to 15.5 years after CoA repair (157). In an earlier adult patient series from this department the three late deaths among 108 patients were evidently due to cerebrovascular accidents (170).…”

“…Changes in blood flow patterns through the aortic isthmus and ductus arteriosus at about the time of birth are suggested aetiological factors (25,143). For reasons as yet unknown, males are affected 2 to 4 times more often; familial occurrence has been described, although the risk to a sib is small (19,117,157). The first known description of CoA was given by Morgagni (114) in 1760.…”

Atrial septal defect, ventricular septal defect, and coarctation of the aorta in sibs: An autosomal recessive disorder?

“…They also performed a literature review, which showed that only 26 families had been reported in which multiple relatives were affected, but in no more than two generations. Simon et al [1974] identified seven families in which coarctation of the aorta was documented in two successive generations, although this could still be consistent with multifactorial determination.…”