The anatomy, natural history, clinical features and symptomatology of 101 cases of congenitally corrected transposition of the great arteries (C-TGA) diagnosed over a 20-year period at Hospital for Sick Children, Toronto, Ontario, is reported. Only 1 patient had no association lesion and only 14 an intact ventricular septum. Pulmonary stenosis was present in a little more than half of the patients. Left A-V value involvement existed in 21 patients. In spite of the hich incidence of associated lesions with prognosis was better than expected. Seventy-six patients were alive at the end of the study, no one being critically ill. No death occurred among the patients with intact ventricular septum. Patients with pulmonary stenosis as a group developed symptoms later, were less handicapped and had a better prognosis than those without a pulmonary stenosis. One third of the patients had dysrhythmias. Eight patients having a 3rd degree AV-block. Auscultatory findings were not of too great a help when diagnosing C-TGA. Forty-seven patients had a single second sound, of these 37 had pulmonary stenosis. In 60% of the patients, where information was available, the second sound was reported to be of normal intensity. Our surgical experience is documented and discussed. Palliative procedures were done with successful results and low mortality. Totally corrective procedures were performed in 9 patients with 4 deaths.
Aperia, A., Bjarke, B., Broberger, 0. and Thoren, C. (Department of Paediatrics, Karolinska Institutet, S:t Giiran's Children's Hospital, Stockholm, Sweden).Renal Function in FaUot's Tetralogy. Acta Paediatr Scand, 63: 398, 1974.-Renal function was examined in 11 adults with Fallot's tetralogy. The following aspects of renal function were studied: GFR, PAH clearance, filtration fractions, the natriuretic response to an oral salt load and renal regulation of acid base balance. The GFR was found to be only moderately reduced while PAH clearance showed an almost 50% reduction in most cases studied. Consequently the filtration fraction was abnormally high. The natriuretic response to an oral salt load was generally low. The urinary sodium elimination rate was found to he independent of deviations in GFR and correlated inversely with the filtration fraction. The renal response to ammonium chloride induced acidosis WBS studied in 7 patients and was delayed in at least 3 of those. The pathological response was thought to he due to hypocapnic depression of renal HCO, reabsorption. It is suggested that this could contribute to the mild nonlactic acidosis onen present in congenital cyanotic heart disease.
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