Symptomatology and Diagnosis.-1. Congenital ostium stenosis of the aorta cannot be differentiated from an acquired stenosis by the physical signs. Atresia of the mitral portion of the aorta, even when associated with patulous ductus, does not cause the production of murmurs. Murmurs may- be absent with high-grade congenital ostium stenoses of the aorta, if the main blood stream is canied through a pat ulous septum to the right and onward through the pulmonary artery and ductus directly to the arch of the aorta. The length of life is lim ited in these cases, rarely extending over several weeks.
2. Isthmus stenosis of the aorta produces typical symptoms in later life, of which the most important is the development of a collateral circulation, the duty of which is to supply blood to organs receiving their blood from beyond the isthmus, chiefly those of the lower half of the body. The following arteries take part in forming this: ante rior mammary, anterior intercostal, superior intercostal, dorsal and transverse scapular, subscapular and external thoracic arteries, which carry the blood to the superior epigastric and posterior intercostal arteries. These arteries are seen and felt as tortuous pulsating or also vibrating, projecting cords, feeling solid just beneath the skin. The internal mammary arteries are most dilated and a systolic murmur may be audible in them.
The majority of the eases occurring in childhood run their course without the formation of a collateral circulation, if the stenosis is not of high-grade and the hypertrophy of tbe left ventricle, which is never absent. is still capable of overcoming the stenosis.
It is possible to make the diagnosis more frequently in children from the murmurs, which are always purely systolic, heard over the sternum, and to the right of it, in the upper intercostal spaces, up to the neck; sometimes, if the murmurs have their points of maximum intensity upon the manulnium sterni, they are transmitted into the arteries of the neck. accompanied by a bulging forward of the arch of the aorta in the neck. In differentiating it from pulmonary stenosis, absence of weakening of the second sound at the ostium of the pul monary artery is noted. An increased second sound at the aortic car tilage, v,ith rapid, ringing rebound, if well marked, should be of great value in the diagnosis in children. Besides, retardation of the crural pulse and decided weakness of the pulse-wave in the arteries of the lower half of the body may be found very essential aids in support of the diagnosis, while the arteries of the neck and the arch of the aorta, which were still supplied with blood before the obstruction, are very full and dilated and show a bounding pulse.
The number of cases hitherto published is 90, according to Barie, of which by far the great majority occurred in males. According to Vierordt there are 69 males to 26 females with this affection.
These individuals may live a long time. Reynaud's case of a shoe maker aged 92 y-ears, is is-ell known. Vierorcit estimated the average length of life for persons with isthmus stenosis to be 31.1 years for males, 30.3 years for females. The fatal termination of the disease occurs with symptoms common to cardiac disease, in which dyspncea and dropsy play the principal part. Rupture of the aorta in front of the stenosed area, with sudden death, has repeatedly been seen. So, too, has rupture of the right auricle (Meckel) and of the right ventricle (Cooper) been observed with isthmus stenosis.
3. Congenital narrowness of the aorta and arteries of the body, with which Rokitansky was already familiar, has only been the object of comprehensive investigations since its relation to chlorosis was dis covered by Virchow. in typical cases the heart remains abnormally small. But as a large number of individuals with this condition show signs of cardiac hypertrophy in childhood, it must be understood that the heart, too small at first, later hypertrophies at the time of puberty. Probably the symptom-complex described by Germain See as insuf ficiency of the child's heart due to growth (see p. -16S) is nothing else than cardiac hypertrophy with congenital narrowness of the arteriesof the body-.
The clinical picture of this condition, in the investigation of which Bruberger, Leyden, Kulenkampf, Tuczek, Riegel, Kiissner, and Ortner deserve much credit, is, taken altogether, that of a valvular lesion of the left side of the heart. The most frequent complaints are of palpi tation, dyspnoia, and rapidly wearied mind and body.
The physical signs vary very greatly. If it is possible, radiographi. eally, to show an abnormally small heart, with an abnormally slender shadow of the vessels, this is of incalculable value in the diagnosis of the condition. With hypertrophy- of the heart the disproportion between the cardiac hypertrophy and tbe narrow carotid and radial arteries would be very real.