CONGENITAL stenosis of the pulmonary artery branches is an anomaly characterized by narrowed segments of one or more of the main or peripheral branches of the pulmonary artery. Such conditions lhave been variously labeled pulmonary coaretation,' peripheral pulmonary stenosis,2 and multiple peripheral stenoses 3 of the pulmonary artery. Several cases of this anomaly have appeared in the literature 1-17 since its first description by Oppenheimer4 in 1938. The problems produced by stenosis of the branches of the pulmonary artery are related to diagnosis and to the difficulties that they may present in the correction of associated cardiovascular defects.It is the purpose of this paper to review our experience with 18 cases of steniosis of the pulmonary artery branches (cases 1 to 18) encountered at the Mayo Clinic with particular regard for diagnosis in the presence of infundibular or valvular pulmonary stenosis and for the role of branch stenosis in the surgical therapy of associated cardiovascular abnormalities. Cases Studied and Procedures UsedTen of the 18 patients were males, and eight were females. Eight patients were 3 years old or less, six were 5 to 10 years old, and four were 17 to 21 years of age. A complete history and physical examination, roentgenogram of the thorax, and electrocardiogram were obtained in each case.Sixteen patients underwent special investigation that utilized cardiac catheterization for From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota.
The clinical features and follow-up data on 107 patients who had a clinical diagnosis of aneurysm of the thoracic aorta made at the Mayo Clinic in the period 1945 through 1955 were reviewed. The ratio of male to female was 2.8 to 1, and the average age at the time of diagnosis was 59.3 years. Fusiform aneurysms were four times as frequent as saccular aneurysms; the most common location of the former was the descending thoracic aorta and of the latter the ascending thoracic aorta. Most of the aneurysms (73 per cent) were probably arteriosclerotic in origin. Symptoms, usually pain, and physical signs were present in a minority of patients and indicated a large aneurysm with a poor prognosis. Diastolic hypertension was present in almost half of the patients at the time of diagnosis and had an adverse effect on the patient's survival. Associated arteriosclerotic cardiovascular disease was present in 40 (37 per cent) of the patients at the time of diagnosis; some of these patients had multiple associated cardiovascular lesions. The presence of associated coronary, cerebral, or other peripheral arterial occlusive or aneurysmal disease had the most deleterious effect on survival. The prognosis for patients with large aneurysms was poorer than for those with small aneurysms. Information as to the cause of death was available in 59 (83 per cent) of the 71 patients known to be dead at the time of follow-up. Approximately a third of the deaths were due to rupture of the thoracic aortic aneurysm, and approximately a half, to associated cardiovascular disease, particularly that due to arteriosclerosis. It is believed that this study of patients with untreated aneurysms of the thoracic aorta may be used as a guide to the selection of patients with aneurysm for surgical treatment and may serve as a basis for evaluating the long-term results of such treatment.
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