A system of practical medicine. By American authors. Vol. 3

by Unknown

If the malformation is not of character or degree to develop cyanosis early in life, the child grows and passes through the usual stage of development, usually, however, feeble, poorly nourished, incapable of common exertion, but often without any special phenomena to attract attention, and the vice of formation is undetected unless by a special examination. There are several other symptoms frequently present in connection with malformation, but not of a pathognomonic character. Dyspnoea, though rarely occurring without cyanosis, may attract attention, and, if frequently brought on by active exercise, increases in violence, to be later accompanied with the cyanosis originally absent. Palpitation is not uncommon, especially in cases of great hypertrophy with dilatation, in hearts struggling to overcome an obstruction; in other cases it is absent or only occurs on exertion in connection with dyspnoea and cyanosis. The degree of animal heat varies greatly, judging by the various opinions expressed by writers. The sensation of patients able to express their feeling is often that of chilliness, and in some cases the surfaces of the body feel cold, although the indications of the thermometer show no great variation from the normal temperature. It is obvious that no very great variation from this standard is compatible with the long duration of life, although a depression may exist during or immediately after paroxysms of dyspnoea or cyanosis. Cough is also frequent, but is probably always due to some acquired pulmonary disease.

The physical signs offer increased facilities for the recognition of defects of development. In the early reported cases there are of course no records of these conditions, and there is therefore a lessened number of instances from which to collate the physical signs. In the early days of life it has been shown wherein the presence of a cardiac murmur may lead to the distinction between malformation and atelectasis. In later periods of life the physical signs cannot be regarded as characteristic. There are no signs by which a malformation can be distinguished accurately from an acquired cardiac disease, so that without the clinical history and a grouping of symptoms the diagnosis cannot be made from the physical examination.

Inspection and palpation of the chest often show the heart to be in an unusual position, placed more centrally under the sternum. It must be remembered that transposition of the heart to the right side is not unfrequently unaccompanied with any malformation of its ostia giving rise to symptoms; and this organ may be even more markedly displaced without being malformed, although under both these conditions irregularities of the principal trunks are usually found.

Percussion frequently shows enlargement of the area of cardiac dulness, but, on the other hand, at the post-mortem examination the heart is often found markedly defective without externally showing variation of its size or shape, or of its position within the thorax; hence in such cases no deviation from the normal will be revealed on percussion or inspection of the chest. It is probable that cardiac murmurs are not always to be detected in cases of even marked defects of development, but when present it is recorded most frequently that a single murmur is heard over the base of the heart, blowing in character and systolic in time. Such a sound is probably produced by the passage of the blood through an abnormal opening between the ventricles or through the foramen ovale. Other murmurs may also be present; if the arterial ostia are defective from narrowing, roughness, or insufficiency of their valvular apparatus, abnormal sounds of different characters, diastolic or systolic in time, may be heard. Too few observations as yet exist for a general diagnostic scheme to be formulated. Auscultation of the intra-uterine heart may in the future become sufficiently accurate to enable us to prognosticate a congenital cardiac malformation or disease; there is one case on record in which a correct diagnosis was made in this way.

The ends of the fingers and toes are frequently described as bulbous. This rounding and retraction of the nails, frequently spoken of as clubbing, does undoubtedly exist in many cases, but the condition cannot be regarded as characteristic of malformation of the heart, since it comes with even more frequency in tubercular disease of the lungs, in chronic pleurisy, and in other chronic pulmonary maladies.

Lebert has recently insisted on the connection between stenosis of the pulmonary artery and tuberculosis, not merely as a coincidence, but as the cause of the development of the tubercles in the lungs. Many others have spoken of this connection, and very many are the cases recorded—perhaps nearly one-quarter of the whole number. In some cases large or small single cheesy masses exist; in others cavities form, and in rare cases a miliary tuberculosis exists, still more rarely affecting other organs than the lungs. In view of the recent dogmas of tuberculosis it is doubtful if many of these authors would at present insist on the connection between malformation of the heart and tuberculosis being other than a coincidence, since it is not apparent why such patients are more likely to be invaded by a bacillus of tuberculosis than other persons, and this organism is known to grow so readily wherever the spores chance to fall.

DURATION OF LIFE.—In connection with certain malformations some indications have already been given in respect to the duration of life in such defects. It is, however, apparent that the degree of the obstruction to an orifice or vessel, and still more the completeness of the secondary compensatory alterations, exert a greater influence than the seat of the malformation on the continuance of life. The occasional slight isolated malformations, such as open septa without obstruction of the orifices, in themselves often entail no symptoms, and, unless combined with acquired valvular disease, exercise no influence on the duration of life; here, however, the prognosis merges entirely into the acquired malady.

Of the other conditions of malformation, narrowing of the aorta and of the aortic conus seems to be, on the whole, compatible with a longer duration of life than any other condition, and these defects cause death in the early days or months in fewer cases than similar obstructions on the right side of the heart. This result apparently comes from the fact that the left ventricle seems to possess unlimited capacity for hypertrophy, and hence is able to overcome the obstruction; when the aortic valves allow of regurgitation the compensation fails and death comes sooner. When the main branches of the aorta are defective or when the descending aorta is derived from the pulmonary artery, the duration of life is much shortened.

In cases of pulmonary narrowing in general it may be stated that the greater the obstruction the shorter the life. This rule is subject to many exceptions; so frequent are the exceptions that the rule is almost valueless for determining the life in any given case. Complete closure of the pulmonary trunk has permitted of the continuance of life for sixteen years, and then ended from an intercurrent acute disease. When the septa are maintained open—when, therefore, the communications between the pulmonic and systemic sides of the heart are free—a greater age is attained than when these openings have become closed. This condition of the pulmonary artery in order to permit of a long duration of life must be coincident with a considerable development of the collateral circulation by which the blood freely enters the lungs for aëration; otherwise the compensation fails very soon.

In transposition of the main trunk relatively to the ventricles, with closure of the septum ventriculorum (very rare), life ends not many weeks after birth; if the septa remain open, which is not common, life may be prolonged for a year or two.