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CASE 3

DIAGNOSIS - CASE 3

A total of 21 answers for this case were divided as follows:

- Bicuspid Valve (congenital malformation) with Stenosis and Calcifications: 17
- Rheumatic Aortic Valve with Stenosis: 4

Send comments and questions about this case to:
Francisco G. La Rosa, MD
Pathologist
TelePathology Consultants, PC
Lakewood, Colorado 80227 - USA

DISCUSSION - CASE 3

Although aortic stenosis (with or without regurgitation) may occur as an isolated valvular involvement in chronic rheumatic carditis, there is substantial evidence that most isolated calcific aortic stenoses are non rheumatic in origin. In the great majority of instances, there is no rheumatic history, neither are stigmata of RF (e.g. Aschoff bodies) present. In addition, there is an increasing frequency of calcific aortic stenosis with advancing age. Most important, there is often an underlying congenital malformation of the aortic valve, particularly a bicuspid valve, which prior to the calcification induced no stenosis of the aortic valve. It is hypothesized that "wear and tear" of such a congenitally abnormal valve may lead to fibrosing stenosis and calcification even in the relatively young, whereas with a normally formed aortic valve, calcific stenosis does not appear until the advanced years of life. The calcification occurring in bicuspid aortic valves is of the dystrophic type, but is identical to that, which may occur, in a normally formed valve. The narrowed sinuses of Valsalva become filled with moundlike excrescences of calcium covered by a thin layer of endothelium. Frequently, the fibrosis and calcification so overwhelm the aortic valve as to make difficult the identification of the preexisting architecture of the leaflets. Gross commissural fusion or vascularization of cusps was not observed in this aortic valve presented in this case, as it could be seen in patients with previous rheumatic or other inflammatory disease such as infective endocarditis.

The obstruction to left ventricular outflow leads to a pressure gradient between the left ventricle and the aorta during systolic ejection (pressure overload), which gradually increases over the course of many years. The left ventricular output is maintained by the development of left ventricular hypertrophy as observed in this case. A large pressure gradient may exist for many years without a reduction of cardiac output, or left ventricular dilatation, or the development of symptoms. As the severity of the stenosis increases, the left ventricular systolic pressure continues to rise (it may reach 300 mm Hg) increasing the myocardial burden. Once symptoms appear, however, the prognosis is poor, with a median survival of only two to three years. Interestingly enough, the patient this case had clinical symptoms of "asthma", which may correlate with symptoms of aortic valve stenosis. Critical obstruction constitutes a two-thirds reduction in valve area or a pressure gradient of 50-mm Hg. Although cardiac failure is the usual sequel, 10 to 20% of adults with this valvular disease die suddenly; thus, valvular replacement is urgent when possible.

Based on the above findings, we can attribute the cause of death of this patient was a severe aortic valve insufficiency due to a primary bicuspid malformation of the aortic valve with severe fibrotic and calcific stenosis. No other evidence of malformations or pathology to explain the death of this individual was found.

Excerpts taken from Robins Basic Pathology, page 578, 1982 Ed.

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