Calcific aortic stenosis and congenital bicuspid aortic valves.

Most cases of aortic stenosis are congenital, rheu- matic, or calcific.For a long time only isolated cases were reported as congenital and some thought that nearly all were rheumatic, the sclerotic and calcific changes being secondary to earlier infection.This paper is concerned mainly with the clinical evidence about the nature of calcific aortic stenosis, with the frequency of sudden deaths in all forms of aortic stenosis, and with the incidence of congenital bicuspid aortic valves and the part they play in producing aortic stenosis.After a few early cases and views have been men- tioned, 60 cases of lone calcific aortic stenosis from Dr. Keith Simpson's necropsy records are analysed.The clinical evidence that bicuspid aortic valves become thickened and rigid at an early age and be- come calcified earlier than other valves is then re- viewed on the basis of my own cases, some unpub- lished evidence kindly put at my disposal by Dr. Ariela Pomerance, and other published papers.SoME EARLY CASES AND VIEWS "A Parisian tailor, not yet old, having dined and left his house had walked hardly 40 paces when he suddenly fell to the ground and expired.His body was opened and no disease was found except that the three semilunar cusps leading to the aorta were bony" (Bonet, 1679, translated by White, 1951).Lloyd (1847) reported two cases.One was a man, aged 23, who had good health till the last 8 months of his life when dyspnoea, attacks of sternal pain, and finally oedema of the ankles developed.He had long-standing aortic stenosis and his heart weighed 680 g.Another man, aged 52, died sud- denly during intercourse, having had some bron- chitis for 7 years.The aortic orifice hardly admitted a probe, the valves being extremely ossified: the mit- ral orifice also was surrounded by a ring of bone.