Between 1962 and 1979, 87 patients with congenital aortic stenosis (11 infants from 4 days to 5 months, and 76 patients from one year to 24 years) underwent open aortic valvulotomy; in 14 patients an additional subvalvular membraneous ring or hypertrophic subaortic stenosis was resected. There were 3 early deaths (3%), all in infants less than one year of age. A second operation was necessary 3 months to 10 years (mean 6.3 +/- 4 years) after the initial procedure in 12 of the 84 survivors. In 9 patients an aortic valve replacement and in 3 patients a second valvulotomy was performed without perioperative mortality: in the latter group valve replacement had to be performed later. There were 5 late deaths (6%). The causes of death were endocarditis (2), thrombosis of the prosthesis (1), accident (1) and sudden death (1). The overall actuarial survival curve shows a 5-year survival of 90% and a 10-year survival of 87%; 87% are reoperation-free after 5 years and 75% after 8 years. At present 7 patients are scheduled for surgery because of recurrence of stenosis; 61 patients are symptom-free. It is concluded that aortic valvulotomy has immediate and long-term benefit in the large majority of patients. Operative mortality and morbidity are low in patients older than one year of age. Nevertheless aortic valvulotomy, which should not be performed too late, represents a palliative treatment and will lead to reoperation in approximately 25% of patients after 8 years.

We report a full-term newborn girl with a giant vein of Galen malformation and extreme cerebral atrophy of prenatal origin. She presented on the 3rd day of life with intractable congestive heart failure. The diagnosis of the vascular malformation was confirmed by ultrasound and magnetic resonance imaging.

Multiple embolizations were the hallmark of the disease in an 8-year-old boy with a left atrial myxoma. Embolizations occurred initially in both hands and legs, later in the brain with generalized seizures and hemiparesis, and finally in the left eye with occlusion of the central retinal artery and consecutive severe visual impairment. Echocardiography demonstrated the tumour which was removed without complications.

A method is described for the direct measurement of upper airway circumference and length with mercury strain gauges fixed to the trachea and main bronchus. The gauges were permanently placed in dogs and were used in serial studies in the intact animal, anesthetized and unanesthetized. The method proved to be reliable and relatively unaffected by extraneous factors. Three types of tracheal motion were observed during eupnea: 1) Primarily passive changes in tracheal length and circumference during each respiratory cycle; the circumference changes could be related to changes in transmural pressure and the length changes related to the over-all downward movement of the lungs during inhalation. 2) The breath-by-breath tracheal motion was superimposed on rhythmic alterations of length and circumference mediated through the vagus nerves. 3) There was a tonic tracheoconstriction which was also dependent on vagal innervation. All changes in airway dimensions involved the entire trachea as a unit, with no evidence for tracheal peristalsis. Changes in airway volume were calculated from the length-circumference data. Note: (With the Technical Assistance of Bernard Minter) direct measurement of airway dimensions; mercury strain gauge; vagal innervation and tracheal dimensions; tracheal volume Submitted on July 13, 1964

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We report a boy with an unusual course of pulmonary hypertension of vascular origin. He suffered from pulmonary perinatal complications due to a Pierre Robin anomaly. Pulmonary hypertension was diagnosed at the age of 18 months. He died from progressive right heart failure at the age of 19 years.

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