Congenital Aneurysms of the Aortic Sinuses with Cardioaortic Fistula

¹ The Departments of Medicine and Pediatrics, University of Minnesota Medical School.

1. Although congenital aneurysms of the aortic sinuses (of Valsalva) are of rare occurrence, 71 cases of this defect have been reported in the literature. Ten patients from the University of Minnesota Hospitals with clinical or autopsy evidence of this malformation are also included in this report.

2. Of the total of 78 cases, 59 had undergone rupture of the aneurysm with creation of a cardioaortic fistula. The most common site of termination was the right ventricle (34 cases).

3. Symptomatology of this lesion is described. The essential lesion is a lack of continuity between the annulus fibrosus of the aortic valve and the elastic aortic media. In at least six cases, clinical history and autopsy findings are compatible with a cardioaortic fistula present at birth. Most commonly the unruptured aneurysm is relatively asymptomatic, the patient being aware only of a heart murmur or mild dyspnea. In contradiction to repeated impressions in the literature, rupture of the aneurysm was not a sudden dramatic event in nine of our ten cases. The typical continuous murmur heard after rupture Is differentiated from that of patent ductus arteriosus by location and quality. Findings on the electrocardiogram and roentgenogram and at cardiac catheterization are reviewed. The definitive diagnosis can be made by angiocardiography or retrograde aortography.

4. Successful surgical closure of a ruptured congenital aortic sinus aneurysm has been reported in eight cases within the past two years utilizing cardiopulmonary bypass with a pump-oxygenator and retrograde coronary perfusion. Rupture of the aneurysm does not result in immediate death (except in the extremely rare instances of rupture into the pericardial sac). The usual patient can be carried on medical therapy for several years, If need be. However, with the availability of the pump-oxygenator, definitive demonstration of a cardioaortic fistula must be considered a strong indication for its closure under "open heart" techniques.