PULMONARY STENOSIS WITH TUBERCULOSIS AS A SECONDARY AND TERMINAL COMPLI- Lewis M. Gaines, A.B., M.D., Atlanta, Ga. Although pulmonary stenosis is the commonest of all congenital cardiac defects, it is of sufficient rarity to warrant careful consideration in each individual case. Furthermore, in cases occuring without the typical cyanosis of the classical congenital heart disease, the diagnosis is of extreme importance on account of the grave prognosis, and the fact that if precautions are taken there is strong likelihood of prolonging life a number of years by guarding the individual against infection, especially that of tuberculosis. An ideal classification of this condition is difficult. Cases of atresia are to be distinguished from those of stenosis, with which this paper is dealing. The atresia cases die very soon after birth and consitute the typical “blue babies.” Rauchfuss bases his classification of pulmonary stenosis upon the presence or absence of defects in the interventricular septum. He takes this to have an important. etiological bearing on the problem of deciding at what stage in embryonic or foetal life the stenosis took place. If there is a defect, the probability is that a developmental error occurred very early in foetal life. If no defect of the septum is present, it is taken to indicate inflammatory processes in a stage of foetal life after the heart has been fully formed. In regard to the frequency of pulmonary stenosis, the statistics are said to be inexact. In 1898, Vierordt estimated 300 cases. Among 181 anomalies of the heart, Peacock found 119 of this condition. In 579 cases analyzed by Kussmaul, Rauchfuss, Peacock, Vierordt, Theremin, and Abbott, 26 per cent. are cases of atresia, 74 per cent, stenosis. Defect of the interventricular wall exists in a large percentage of the cases, especially in stenosis. Thus Rauschfuss finds among 192 cases, 171 in which there is defective interventricular septum, 21 closed. Of the latter number 10 are atresia, 11 stenosis. In the stenosis cases the ductus arteriosus is nearly always closed. Rechtlage of the aorta is very frequently present in association with septal defect, especially in atresia. From the standpoint of pathological anatomy two general types of stenosis are described. In the first and smaller group of cases the stenosis is practically entirely valvular and suggestive of a pre-natal inflammatory process, the valves being thickened, shortened and even fused. The pulmonary artery is dilated above and thin walled, while below the conus is normal except, perhaps, for its general share in the right ventricle hypertrophy. In the second and larger group of cases, the stenosis is due not so much to valvular deformity, as it is to a rudimentary condition or deformity of some part of the pulmonary tract. Septal defect is almost the rule in this type, while rechtlage of the aorta is common. Such a combination strongly suggests developmental origin. According to Abbott, two types of conus are described: (1) The whole infundibulum is constricted, the musculature thickened, the pulmonary cusps delicate and healthy above the stenosis, but both they and the artery very small. Frequently, however, the valves are thickened and fused, often bicuspid. The artery is often short. In these cases septal defect is stated to be almost invariably present. (2) In the other group of conus deformity, a cavity resembling a third ventricle is cut off from the sinus of the right ventricle by a definite perforated septum. This form is relative infrequent. Symptoms-The majority of cases present the classical symptomatic picture of congenital cyanosis. Indeed, "Morbus Coeruleus" and pulmonary stenosis may almost be considered synonymous terms. However, the clinical symptoms vary with the variety of the deformity and the degree of narrowing. In stenosis with closed septum, cyanosis may be slight or absent the duration of life considerable, and the difficulties of diagnosis increased. In such cases, where the individual has reached or passed the age of puberty, there is a striking lack of general bodily and mental development. Physical Signs-The physical signs are often indefinite or confusing, but the most suggestive are the following: (1) An increase of cardiac dullness to the right. This is often difficult to demonstrate, though a greater or less degree of hypertrophy of the right ventricle is to be expected in all cases which survive any length of time. (2) Praecordial bulging. (3) Intense cardiac impulse. (4) Thrill, most marked usually over the second and third left interspaces, but frequently fairly diffuse. Thrill, however, is not so frequent a sign as one might suppose, being present in only sixteen out of seventy-five cases. It was absent in my own case. (5) A prolonged, harsh rasping or perhaps blowing systolic murmur, heard over the whole cardia, sometimes over the whole chest front and back. The maximum intensity of the murmur is usually over the pulmonic area, and the sound is frequently transmitted a short distance upward along the pulmonary artery. The maximum sound may, however, be heard at the apex, or over the left nipple, or along the aortic area and extending to the carotids. Occasionally physical signs may be absent as in the case of a child of 5, with large septal defect, rudimentary valves, and the pumonary artery a small cord. The heart sounds were reported clear in this case. In regard to the course of the disease, the duration of life is relatively high. Peacock records a case surviving at the age of 45 years. The majority, however, die before the age of twenty. A very large number of the deaths are due to pulmonary tuberculosis, to which disease the unfortunate subjects of this form of heart disease are peculiarly susceptible. This susceptibility has been attributed by some to the undue anaemia of the lung. by others to the cyanosis which they suppose depresses resistance, and favors destructive tissue metabolism. The individuals live to just the age when tuberculosis is most likely to invade a receptive soil, whether this soil has been prepared by anaemia or cyanosis. Another mode of death is acute endocarditis developing at the seat of the lesion. One is apt to dismiss the treatment of a condition such as congenital stenosis with a shrug, but I conceive the prophylactic treatment against infection particularly that of tuberculosis to merit the closest attention. I have no doubt that many cases could have their lives prolonged if they are early diagnosed, kept in the best condition, and guarded carefully from infection. I have the pleasure of presenting the following case of pulmonary stenosis, which offers some points of unusual interest: L. F.-Colored. Female. Age, 15 years. Occupation, nurse. F. H.-Uncle has tuberculosis. Father and mother dead, cause unknown. Lives with infirm grandmother under the worst possible hygienic arrangements. P. H.-Patient states she has never been strong, and has always had some cough. She passed successfully through attacks of measles and whooping cough. She gives no history however, of any symptoms particularly referable to the heart, and though she was never robust, considered herself fairly well, and was engaged up to the time I saw her as nurse for a white family, doing considerable manual labor. She stated she had never menstruated. P. I. The principal complaint is of cough and pain in the chest. Cough is marked and always has been a symptom, though worse for the past three years. Pain and tenderness is noticeable on the right side of the chest in front. Night-sweats have been present for several months. She has lost some weight, but does not know how much. Expectoration is copious. Haemoptysis has occurred several times the past few months, the last one occurring the day before the patient presented herself. It was this symptom which caused her to seek advice. Appetite fair, digestion good. Sleeps well. Menstruation has never occurred, though the pains of menstruation have been present. Shortness of breath on exertion is fairly well marked. There has been slight oedema of the ankles. P. E.—Patient is an undersized, moderately emaciated, ill-developed girl. Weight, 78 pounds. Mental development about that of a child of eight years. No cyanosis is evident. The color of the mucous membranes is fairly good. The fingers are not clubbed, but rather thin and tapering. The breasts are rudimentary. Pubic hair almost absent. Lungs: the right lung presents all the evidences of well advanced tuberculosis. It is dull on percussion throughout almost its entire extent, while medium and fine moist rales are everywhere present, and the breath sounds are typically broncho-vesicular. The left lung presents the signs of compensatory emphysema. Heart on inspection, there is well marked pre-cordial bulging. The apex can be discerned in the fifth interspace in the mamillary line. The pulse is beating at the rate of 120 per minute, and small in volume, not sustained. No thrill is detectible. On account of the surrounding lung dullness, the right border of the heart. cannot be accurately outlined by percussion. On auscultation, there is heard over the entire chest in front an extremely harsh, rasping systolic murmur, which is of maximum intensity directly over the pulmonic area, and is loudly heard as high as the first rib above, and the apex of the heart below. It is not loudly heard to the |