Bernadine H. Bulkley

Ankylosing Spondylitis and Aortic Regurgitation Description of the Characteristic Cardiovascular Lesion From Study of Eight Necropsy Patients

Clinical and cardiovascular necropsy findings are described in eight patients with combined ankylosing spondylitis and aortic regurgitation. All were men (aged 34-55 years), each had peripheral arthritis in addition to spondylitis, all had severe congestive failure, and six had conduction disturbances. In three patients aortic regurgitation was present before distinctive radiologic changes of ankylosing spondylitis were apparent and only two patients had advanced arthritic changes of ankylosing spondylitis. Thus, cardiac dysfunction may be present before signs of spondylitis are apparent, and aortic regurgitation may be severe when signs of spondylitis are minimal. A characteristic cardiovascular morphologic abnormality was present in each patient. The aortic valve cusps and the aorta behind and immediately above the sinuses of Valsalva were thickened, the latter by dense adventitial scar tissue and by intimal fibrous proliferation. In each patient the scar tissue in the root of aorta extended below the base of aortic valve to produce a subaortic fibrous ridge. The subaortic bump involves the base of anterior mitral leaflet and may cause mitral regurgitation. Extension of the fibrous scar into ventricular septum may cause heart block. The distinctive cardiovascular morphologic findings in patients with ankylosing spondylitis clearly separate this condition from syphilis and other entities associated with aortic regurgitation.

Prosthetic Aortic Stenosis A Method to Prevent Its Occurrence by Measurement of Aortic Size from Preoperative Aortogram

A cause of early death after aortic valve replacement with a caged-ball prosthesis is obstruction to left ventricular outflow because the prosthesis is too large for the aortic root. Of 68 patients dying within two months of aortic valve replacement, death in ten, each of whom had had intractable low cardiac output after operation, was attributed at necropsy to prosthetic aortic stenosis, despite the use of small sized (8A Starr-Edwards) prostheses in seven of them. The diameters of the aorta at the sinotubular junction, determined from the preoperative cineangiograms, in the seven patients with prosthetic stenosis were < 30 mm in all. Poppet clearances, defined as the differences between poppet and aortic root diameters, ranged from 4 to 12 mm (avg. 9). In contrast, the diameters of the aortas at the sinotubular junctions in eight control patients (unobstructed prosthetic aortic valves and early death from other causes) were > 30 in all but one, and the poppet clearances ranged from 12 to 19 mm (avg. 15). Thus, prosthetic aortic stenosis is likely to develop after aortic valve replacement with rigid-framed caged ball valves if the preoperative aortograms disclose aortic diameters at the sinotubular junctions to be < 30 mm. In such patients, either the aorta must be widened for a caged-ball prosthesis or a central flow valve must be used.

Heterografts as aortocoronary bypass conduits in human beings

Clinical and necropsy observations are described in a man in whom two sheep carotid arterial heterografts were inserted as aortocoronary bypass conduits 30 months before death, and in whom a canine saphenous vein heterograft was inserted several hours before death. All three grafts failed. The lumens of the sheep carotid arterial heterografts had closed by the time of catheterization 6 months after operation; at necropsy both grafts were totally occluded at their coronary and aortic ends and their media and adventitia were infiltrated by granulomatous inflammatory cells. Little information is available regarding the use of heterografts as coronary bypass conduits. Examination of previous reports describing heterografts for vascular reconstruction or bypass in both man and experimental animals and observations in our patient suggest that heterografts are unsatisfactory as aortocoronary bypass conduits.

Calcification of prosthetic valve anuli: A late complication of cardiac valve replacement

Abstract A previously undescribed late complication of cardiac valve replacement is calcification at the site of attachment of prostheses. Of 24 patients in whom purely incompetent, non-calcified mitral or aortic valves were replaced with rigid-framed prostheses 3 to 116 months earlier, nine (seven mitral, two aortic) of 26 valves had prosthetic anular calcific deposits. Of the eight prostheses in place for 70 months or longer all contained anular calcific deposits; only one of the 18 valves in place for less than 70 months had periprosthetic calcific deposits. The extent of prosthetic calcium also increased with time. The mechanism of formation of prosthetic anular calcium is uncertain, but accelerated wear of the tissues beneath the prostheses due to the constant to-and-fro motion of the rigid frames may be a factor. Possible complications of prosthetic anular calcific deposits include suture rupture, peribasilar leak, and increased hazard to reoperation.

Libman-Sacks Endocardoma: Diagnosis During Life with Radiographic, Fluoroscopic, and Angiocardiographic Findings

Libman-Sacks endocarditis, usually an autopsy diagnosis and rarely causing clinical problems, was recently diagnosed pre-operatively in an 18-year-old black woman with systemic lupus erythematosus and mitral valve disease. Unusual intracardiac calcification was seen on fluoroscopy. Angiocardiograms revealed an intracardiac mass which was successfully removed and proved to be Libman-Sacks endocarditis.