Four hundred twenty-six patients with esophageal atresia with or without tracheoesophageal fistula have been primarily cared for at the University of Michigan Medical Center since Cameron Haight's initial experience with this entity. Over the period of observation, the incidence of new cases as well as the number of associated anomalies has remained constant. The long-term survival of these patients has steadily improved over the past half-century from 36% in the pre-1950 era to 84% during the most recent 20 years. Conversely, operative mortality has shown a progressive decline from 56% early in the authors' series to 6.9% more recently, despite a steady increase in the proportion of high-risk neonates seen at the University of Michigan Medical Center during this time span. In the last 9 years, there have been no postoperative deaths in group A or B risk infants (36 patients), while the rate has been 18.2% in group C risk babies (27 patients); almost all of these deaths were due to severe associated anomalies. During the last 10 years, the authors have changed their technique of anastomosis from a two- to a one-layer method while still advocating a primary repair via an extrapleural approach. Although this change has resulted in a modest increase in the rate of anastomotic leak (17% vs. 6.2%, p less than 0.03), the leaks have been small and asymptomatic because of the extrapleural approach and, as a result, have been managed conservatively without any untoward sequelae. Conversely, there has been a significant decrease in the rate of stricture formation with the one-layer anastomosis (4.3% vs. 23.3%, p less than 0.002). While this may in part be explained by the change in anastomotic technique, it is felt that the more aggressive diagnosis and surgical management of gastroesophageal reflux (seen in 37.9% of our recent group) have contributed greatly to this decrease. The steady improvement in survival over this 50-year period, in spite of the increasing number of high-risk infants, is attributable to major improvements in neonatal care before, during, and after operation.
Background-Short-and long-term outcomes after prosthetic mitral valve replacement (MVR) in children aged Ͻ5 years are ill-defined and generally perceived as poor. The experience of the Pediatric Cardiac Care Consortium (45 centers, 1982 to 1999) was reviewed. Methods and Results-MVR was performed 176 times on 139 patients. Median follow-up was 6.2 years (range 0 to 20 years, 96% complete). Age at initial MVR was 1.9Ϯ1.4 years. Complications after initial MVR included heart block requiring pacemaker (16%), endocarditis (6%), thrombosis (3%), and stroke (2%). Patient survival was as follows: 1 year, 79%; 5 years, 75%; and 10 years, 74%. The majority of deaths occurred early after initial MVR, with little late attrition despite repeat MVR and chronic anticoagulation. Among survivors, the 5-year freedom from reoperation was 81%. Age-adjusted multivariable predictors of death include the presence of complete atrioventricular canal (hazard ratio 4.76, 95% CI 1.59 to 14.30), Shone's syndrome (hazard ratio 3.68, 95% CI 1.14 to 11.89), and increased ratio of prosthetic valve size to patient weight (relative risk 1.77 per mm/kg increment , 95% CI 1.06 to 2.97). Age-and diagnosis-adjusted prosthetic size/weight ratios predicted a 1-year survival of 91% for size/weight ratio 2, 79% for size/weight ratio 3, 61% for size/weight ratio 4, and 37% for size/weight ratio 5. Conclusions-Early mortality after MVR can be predicted on the basis of diagnosis and the size/weight ratio. Late mortality is low. These data can assist in choosing between MVR and alternative palliative strategies. (Circulation. 2001;104[suppl I]:I-143-I-147.)
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