72 research outputs found
Three-decade follow-up in pulmonary artery ectasia: Risk assessment strategy
No full textWe present a 29-year follow-up in a patient with ectasia of the pulmonary trunk and mild valvular stenosis. There was no progression from the first presentation at the age of 15 months until the actual age of 30 years, the z-value of the diameter of the pulmonary trunk remaining almost constant (z-value 22). This indicates that pulmonary dilatation with normal pressure may be a benign disease. (C) 2002 by The Society of Thoracic Surgeons
Circulatory failure due to left-to-right shunts in children with congenital heart disease - Pathophysiology and therapeutic consequences
No full textIn children with large left-to-right shunts secondary to congenital heart defects the imbalance between the pulmonary and systemic perfusion may lead to circulatory congestion with clinical signs similar to those of heart failure. The circulatory function in this state was evaluated by using the invasive measurements performed during cardiac catheterisations in n = 64 young patients with ventricular septal defect (n = 56) or complete atrioventricular septal defect (n = 8) aging 0.1 - 23.7 years (median 1.1 years). The mean shunt ratio was Qp/Qs = 2-4 (range 1 - 8). With increasing shunt ratio the pulmonary perfusion raised (r = 0.84), but the systemic output dropped significantly (r = -0.77) while the total cardiac output (Qp + Qs) increased slightly not exceeding 14 l/min/m(2). In infants, the systemic hypoperfusion affects the hemoglobin content: Hb = 14.9 - 1.01xQs, r = 0.63, p < 0.01. This may be due to the deminished oxygen extraction reserve of 46%. With dropping systemic output, the vascular resistance increases and the mean aortic pressure (MAP) remains normal. The actual pressure values layed near to the curve of the normal aortic pressure calculated as MAP = QsxRs. This pressure-flow-resistance diagram was used to interpret the effects of vasodilators established by 7 studies: ACE-inhibitors, Hydralazine, and Na-Nitroprussid reduce the vascular resistance effectively but induce hypotension, because the systemic output fails to increase. In the chronic circulatory congestion secondary to a large intracardiac left-to-right shunt the pulmonary perfusion increases with the shunt ratio but the systemic output decreases and the total cardiac output is limited to a maximum of 14 l/min/m(2). In this state vasodilators cause systemic hypotension thus offering no acceptable therapeutic option
Familial Williams-Beuren syndrome showing varying clinical expression
No full textWilliams-Beuren syndrome (WBS) is a contiguous gene syndrome that occurs mainly sporadically, with an estimated frequency of 1:13,700 to 1:25,000 [Grimm and Wessel-hoeft, 1980; Martin et al,, 1984; Udwin, 1990], The cases of monozygotic twins concordant for WBS and dizygotic twins discordant for the syndrome have been reported. In addition, a few familial cases have been described since 1993, The clinical diagnosis has been supported by molecular genetic findings in only two patients, however. We herein report on two families in which the WBS was inherited in girls from their mothers. All four patients showed the typical hemizygous deletion at 7q11.23 [46,XX, ish,del(7)(q11.23q11.23 ) (ELN/LIMK1/D7S613x1, D7S486/D7S522x2)1, hut the clinical picture was strikingly variable within and between families. (C) 2001 Wiley-Liss, Inc
Normality of cardiopulmonary capacity in children operated on to correct congenital heart defects
No full textBackground: One of the main goals of corrective surgery of congenital heart defects in children is the improvement of quality of life, which in young children is predominantly determined by exercise capacity. It is not known whether this goal can be achieved in school-aged children who have undergone cardiac surgery in infancy. Objectives: To determine if primary school-aged children who under-went surgery to correct congenital heart defects in infancy are physically as fit as their peers. Methods: We examined 84 children with congenital heart defects, aged 4 to 11 years, after surgical repair. Fifty-two children had simple defects (ie, atrial or ventricular septal defect, coarctation of the aorta). Thirty-two children had complex defects (ie, tetralogy of Fallot, pulmonary atresia with ventricular septal defect). All patients underwent exercise testing performed on a specially modified bicycle ergometer. Ninety-eight sex- and age-matched healthy children served as the control group. Results: There was no gender difference, either in healthy children or in the group with congenital heart defects, regarding exercise testing and that the healthy children reached a mean +/- SD normalized maximal performance of 2.8 +/- 0.3 W/kg. The same range was found for the children who had undergone surgery to correct simple heart defects. The children operated on to correct complex heart defects showed significantly impaired mean normalized maximal performance, although this tended to be lower in the group that had pulmonary atresia with a ventricular septal defect than in the group with tetralogy of Fallot (mean normalized maximal performance, 1.9 W/kg vs 2.3 W/kg). Conclusions: The goal of normal exercise capacity in childhood after heart surgery is achieved in those with simple heart defects only. In children with complex heart defects impaired exercise performance persists, depending on the severity of the heart defect and probably on chronotropic incompetence
Growth retardation in cyanotic children after aorto-pulmonary shunt: due to a reduced growth in the first year of life?
No full textBackground:The influence of cyanosis, and cardiac index in children with cyanotic heart disease on their growth. Patients: 37 infants with cyanotic heart disease needing a systemico-pulmonary anastomosis. Methods: Body weight, length, head circumference, body mass-index and transcutaneous oxygen-saturation were measured (mean follow-up 610 days). In 25 children cardiac index was recorded by cardiac catheter before their next operation. Results: At 9 months the percentiles had fallen from the 50th at birth to the 3rd for body weight, 10th for length, 25th for head circumference and 25th for the body mass-index. Growth-velocity normalized in the second year. Growth patterns were independent of the oxygen-saturation. Cardiac index was initially reduced but normalized during the 2nd year of life. Conclusion: Growth of infants with a systemico-pulmonary shunt was asymmetric. Growth velocity was reduced in the first year of life, and not influenced by the oxygen-saturation. A causal relationship between cardiac index and decreased growth seems possible
"Closing the R&D Gap, Evaluating the Sources of R&D Spending"
No full textBoth spending and tax policies have been implemented in the United States with the goal of stimulating private sector research and development (R&D). Karier questions whether current R&D policy, especially the research and experimentation tax credit, can contribute to closing the gap between nondefense expenditures on R&D in the United States and such expenditures in other countries, such as Japan and Germany. He also explores possible changes to our current R&D policy to make it more effective.
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