This section is from the book "A Manual Of Pathological Anatomy", by Carl Rokitansky, William Edward Swaine. Also available from Amazon: A Manual of Pathological Anatomy.
This disease is manifested by attenuation, unusual delicacy and transparency of the valves, and in its more intense forms by the formation of apertures within them. We have already instanced a condition of attenuation of the valves, as the consequence of their hypertrophied state. We, moreover, observe attenuation of the auriculo-ventricular valves associated with excentric atrophies and adiposity of the heart. The more highly developed forms of atrophy, in which there are perforations in the valve, are only found in the arterial valves, and more especially in those of the aorta; we do not remember to have observed any case affecting the auriculo-ventricular valves, and we should indeed be disposed to regard this disease as exclusively belonging to the valves of the arteries, if Kingston had not seen a few (three) cases occurring in the former, two of which were in the tricuspid, and the other in the mitral valve. This perforated condition of the valves occurs almost invariably, associated with hypertrophy, in consequence of the dilatation of the corresponding ostium. These perforations are almost always situated near the free margin of the valves, and more especially near their insertion, where they originate, increasing in numbers as they spread towards the nodules of the valve. They are, at first, about the size of a scarcely appreciable pin-hole or of a poppy seed, but after gradually enlarging by the confluence of several into one, they finally attain the size of a grain of millet or a hemp-seed, or even of a pea. When several are present together, they impart a reticular broken appearance to the valve. The perforations are, moreover, surrounded by a smooth margin, and are never round, but oval, elliptical, or fissure-like, and their long axis is at right angles to the free margin of the valve. They are also generally bounded by the fibrous bundles of the valves, so that the atrophy, at least at first, attacks only the thinnest portions.
1 Dr. Löbl has been led, by clinical observations, to adopt the opinion, that a disease of the valves of the aorta, differing from endocarditis, does actually exist.
Besides considerable and appreciable attenuation of the valve, and in some cases even perforation, we occasionally find some portions, as, for instance, the free margin, the nodule, and the fibrous bundles passing from it, thickened or hypertrophied.
Perforation of the valves is not of importance unless the apertures are very large, or some among them are deep and seated in the middle of the valve, and cannot be closed by the approximation of the valves; very generally, too, the symptoms are influenced during life by the simultaneous occurrence of heart-disease, as, for instance, dilatation of the left ventricle, and occasionally by the insufficiency of the atrophied valves, that is to say, by their inconsiderable magnitude compared with the dilatation of the ostium. It is, moreover, very probable that attenuated valves, independently of all other conditions, give rise to a change in the sounds of the heart in consequence of diminished resistance, and modifications in the capacity for yielding sounds.
It is in the middle and advanced periods of life, more frequently than in any other, that we meet with atrophy of the valves, in the more intense form associated with perforation; there is thus, in this respect, a perfect harmony with the periods most prone to dilatation of the ostia, of which the aortic opening is the one more frequently diseased. These periods further correspond with the age at which we most frequently observe excessive depositions of new membrane in the trunk of the aorta, the so-called atheromatous process, and ossification with dilatation; and atrophy of the valves is not uncommonly associated with these diseased conditions of the aorta.
Malformations of the valves are alike frequent and varied; but as they are not possessed of any intrinsic importance we do not deem it necessary to enter upon any classification of them. They will be found under their respective sections, and it will therefore suffice to observe, that the malformations affecting the valves in consequence of endocarditis and of hypertrophy of the endocardium, are the most frequent and the most important. The valves of the left side of the heart would appear, from the observations hitherto made, to be the more especial seat of these malformations, more particularly when affecting the endocardium.
We need do no more than simply refer to the anomalous toughness and hardness of thickened or shrivelled valves, and to the decrease of consistence which accompanies inflammation of the tissue of the valves in the form of relaxation and lacerability, as this subject has already been treated of in a different form under the head of atrophy of the valves.
We would here, however, enter more fully into the consideration of a morbid condition of the valves hitherto but little observed, as we know no other place to which we could more appropriately refer the subject, when regarded in a scientific point of view. We allude to a diminution of consistence in the form of an abnormal softness and tendency to laceration of the valves, - an appearance of the greatest practical importance. This disease, when considered in a practical point of view, might be termed a gelatinous condition of the valve. The cases in which we have seen it have not been rare, but they were always limited to the valves of the left side of the heart. We find that the valve, either throughout its whole extent, or at individual portions is more yielding, softer, and more readily torn; the faint whitish color, and the gloss of the fibrous texture disappear, and are changed to a pale yellowish tinge, approaching here and there to a reddish hue, while the whole becomes translucent. The latter condition probably depends upon the gelatinous non-adhesive substance effused into the tissue of the valve; but yet it is difficult to comprehend how the other anomalies can be produced which we find in this condition of the valve. The tissue of the valve was always found to have disappeared wherever this gelatinous substance was present, and the valve itself, after the removal of this extraneous matter, was observed to be in a state of extreme attenuation or atrophy. The question here arises, is this gelatinous substance a new formation, - a blastema effused here in order to be metamorphosed into fibrous valvular tissue, and for the purpose of strengthening the atrophied valve, - or is it the softened, disintegrated fibrous tissue of the valve itself? The former view appears to us incomparably the more probable, and we are of opinion that this gelatinous substance is the same which, as we have already observed, presents itself in a more dense and tough state, and in the act of undergoing a metamorphosis into tissue, in hypertrophies of the valves. The valve which is rendered soft and lacerable by attenuation becomes still more so from the deposition of this gelatinous substance by which the remaining textural elements are forced asunder. The softness and tendency to laceration of the valve would thus appear to admit of explanation on a mechanical principle, and not on any actual softening process of the tissue.
This gelatinous condition occasionally produces lacerations, more especially of the valves of the aorta. These can be easily distinguished from the perforations already described as produced by atrophy, appearing either as true fringed rents passing lengthways through the valve from its free margin, as fissures in the middle of the valve, or as a laceration or detachment of the valve from its insertional margin. - The gelatinous condition of the valves must, therefore, be classed amongst the more important diseases affecting these structures. The diminished power of tension and resistance must necessarily occasion some modification of the heart's sounds.
This gelatinous condition of the valves undoubtedly admits of cure, since the gelatinous substance may be gradually converted into a fibrous tissue, and thus condensed, by which means the attenuated valve increases in bulk, and is enabled, if necessary, to enlarge and adapt itself to the size of the dilated ostium.
This condition occurs, at the same periods of life, and under the same circumstances, as atrophy of the valves, either with or without simultaneous hypertrophy.
 
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