Congenital and Acquired Aortic Sinus Aneurysm

Abstract

ALTHOUGHit has been a century and a quarter since the first published account by Hope in 1839 1,2 of a ruptured congenital aneurysm of the sinus of Valsalva, there have been fewer than 100 cases of congenital aortic sinus aneurysms reported in the medical literature.3 Except for the work of Edwards et al,4,5 detailed description of the histopathology of the lesion is lacking. The incidence of acquired aortic sinus aneurysms is more difficult to determine from the literature, but the general impression gained is that the acquired type is even more rare. Recently, we have had the opportunity of seeing two patients, admitted within 14 days of each other, in whom the diagnosis of aortic sinus aneurysm was later confirmed by autopsy. Both because of the rarity of this condition and the paucity of detailed histologic studies, these cases are being presented. masses or tenderness ; bowel sounds were normal. Rectal examination showed no abnormalities. There was no peripheral edema, clubbing, or cyanosis, and there were no splinter hemorrhages. The femoral pulses were forceful, but there was no bruit. Neuro¬ logical examination was unrevealing. The clinical impression was aortic insufficiency due to rheumatic heart disease.The urine had a specific gravity of 1.020 and was otherwise normal. The hemoglobin was 11.5 gm/ 100 ml, the white blood cell count 14,500/cu mm with 83% neutrophils and 17% lymphocytes, and the hematocrit 35%. A fasting blood sugar was 110 mg and the blood urea nitrogen 96 mg/100 ml. A battery of liver function tests gave results within the normal range.The serum sodium was 137 mEq, potassium 5.4 mEq,

Aneurysms of two sinuses of Valsalva with a ventricular septal defect and aortic regurgitation.

“…The factors responsible for the development of a congenital aneurysm are not understood. The theories suggested include failure of the aortic media to join the annulus of the aortic valve (Edwards, Burchell, and Christensen, 1956), weakness of the annulus (Kwittken et al, 1965), cystic medial degeneration of the aorta (Steinberg and Geller, 1955), defect in the development of the elastic tissue at the base of the aorta (Venning, 1951;Heiner, Hara, and White, 1961), and defective development of the bulbar septum (Mall, 1912;Abbott, 1919;Jones and Langley, 1949). Several congenital anomalies have been described in association with ruptured aneurysm of the sinuses of Valsalva.…”

“…Congenital aneurysms of one of the sinuses usually bulge into a cardiac chamber and eventually rupture causing an aortocardiac shunt which is a serious condition and can lead to death in a short time (Jones and Langley, 1949;Kwittken et al, 1965). Multiple aneurysms arising from different sinuses are rare.…”

Surgical Correction of Sinus of Valsalva Fistulas

“…The common opinion is that there is either a discontinuity between the annulus fibrosus and the elastic-muscular elements in the media of the aorta (Edwards & Rurchell, 1957), or there is primarily a defect in the annulus fibrosus, vulnerable to the turbulence of blood in the coronary sinuses (Kwittken, Christopoulos, Dua & Bruno, 1965). The common opinion is that there is either a discontinuity between the annulus fibrosus and the elastic-muscular elements in the media of the aorta (Edwards & Rurchell, 1957), or there is primarily a defect in the annulus fibrosus, vulnerable to the turbulence of blood in the coronary sinuses (Kwittken, Christopoulos, Dua & Bruno, 1965).…”