CARDIOAORTIC FISTULAS AND ANEURYSMS OF SINUS OF VALSALVA IN INFANCY

Abstract

A report of an infant with a previously undescribed congenital malformation of the heart consisting of an aneurysm of the left sinus of Valsalva with rupture into the left atrium and associated atrial septal defects is presented. This cardiac anomaly, along with others in the literature, supplies evidence that sizable congenital aneurysms may arise from any of the three sinuses of Valsalva and may communicate directly with any of the four cardiac chambers, the pulmonary artery or the pericardial sac. Other contiguous intrathoracic structures should also be considered potential sites of ruptures. Such a consideration may prevent some instances of congenital aneurysms from being missed simply because they have not been reported. Embryologic factors are briefly discussed as is the possibility that the patient may represent a variant of Marfan's syndrome. Surgical implications are also mentioned. The patient reported presents a clinical syndrome that should be recognizable during life if the possibility of an aortic to left atrial shunt is kept in mind. The findings included evidence of heart disease soon after birth, a loud continuous murmur and thrill present maximally in the left axilla, bounding peripheral pulses and a wide pulse pressure. Roentgenograms showed marked cardiomegaly involving all cardiac chambers, a bulge in the cardiac silhouette to the left of the main pulmonary artery, and marked pulmonary vascular engorgement. There was electrocariodgraphic evidence of atrial and combined right and left ventricular hypertrophy. Cardiac catheterization revealed a peculiar pressure gradient between the left and right atria in spite of an atrial septal defect with a left-to-right shunt. Venous angiocardiograms and retrograde aortograms were not diagnostic, although a more adequate aortographic dye injection would have demonstrated the defect. Retrograde arterial catheterization should also permit an accurate diagnosis.