Ребенок с синдромом Дауна, 2 месяца. ДМЖП, ДМПП, ОАП. Признаки легочной гипертензии (ДЛА 40 мм рт. ст.). Консультирован в Бакулевке, рекомендовано оперативное лечение, когда ребенок "подрастет", плановая консультация через полгода. Назначен дигоксин, верошпирон, аспаркам. Ребенок при рождении 2200 г, сейчас весит 3500 г.
Как известно, при синдроме Дауна закрытие ДМЖП показано в первые три месяца жизни. Может ли малый вес служить поводом для "выжидательной тактики"? Есть ли возможность прооперировать такого ребенка заграницей?
ДМЖП у ребенка с с-мом Дауна
Модераторы: Ren_Yumi, AOkhotin, Алексей Живов, Alon, dr.Ira
Re: ДМЖП у ребенка с с-мом Дауна
Вот что я нашлаAOkhotin писал(а):Как известно, при синдроме Дауна закрытие ДМЖП показано в первые три месяца жизни.
Surgical risk and mortality for patients with large VSDs are higher in the first 2 months of life than after age 6 months (10-20% versus 1-2%), however these data are improving, especially in centers with a large surgical experience.
Elective surgical closure of large VSDs should be planned within the first 6 months to 1 year of life to prevent the development of irreversible pulmonary vascular obstructive disease.
Surgical intervention in younger infants, especially those younger than 1 month of age, significantly increases mortality rates (10-20%). Surgical mortality is low (1-2%) in patients older than 6 months with isolated large muscular VSDs.
Pediatrics 2006
Делай, что должен, и будь, что будет.
Re: ДМЖП у ребенка с с-мом Дауна
Могу узнать конкретно про Израиль.AOkhotin писал(а):Есть ли возможность прооперировать такого ребенка заграницей?
Делай, что должен, и будь, что будет.
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Максим Осипов
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Самый частый порок сердца при синдроме Дауна -- открытый АВ-канал (endocardial cushion defect). Думаю, у ребенка именно он. Многое зависит от того, так это или нет, от того, насколько выражены ДМПП и ДМЖП, расщеплен ли митральный клапан. Хорошо бы мне посмотреть этого ребенка и послать фильмы в Германию, к знакомым кардиохирургам.
Pediatr Cardiol. 1992 Apr;13(2):80-4.
Down's syndrome affects results of surgical correction of complete atrioventricular canal.Morris CD, Magilke D, Reller M.
Department of Pediatrics, Oregon Health Sciences University, Portland 97201.
From 1981 through June 1989, 59 children had surgery for a complete atrioventricular (AV) canal defect at Oregon Health Sciences University. We compared the morbidity, mortality, and hemodynamic status of 47 children with and 12 without Down's syndrome through review of operative, clinical, and cardiac catheterization records. Overall, 10 children with Down's syndrome have died, nine from cardiac cause within 90 days of surgery. The 2-year survival of these children was 77 +/- 6% as compared to 100% in children without Down's syndrome (p = 0.08). Early age at surgery, the surgical anatomy of the AV canal, and gender had no significant effect on survival. Before surgery, the hemodynamic status of Down's syndrome and non-Down's syndrome children did not differ; in a small group of post-operative catheterization, right heart pressures and pulmonary vascular resistance remained significantly higher in the Down's syndrome as compared to non-Down's syndrome children. These trends to higher mortality and poorer postoperative hemodynamics in children with Down's syndrome may necessitate closer follow-up if confirmed in other cohorts.
Thoraxchir Vask Chir. 1976 Oct;24(5):424-30.
[Palliative and corrective cardiac surgery in Down's syndrome (author's transl)][Article in German]
Seybold-Epting W, Hoffmeister HE, Stunkat R.
Since 1970 46 infants and children with Down's Syndrome were subjected to palliation of congenital heart disease, and 54 mongoloid children underwent correction of their cardiac defects. The most common cardiac malformation was endocardial cushion defect (72%). Palliation consisted in pulmonary artery banding (PAB) with or without division of a patent ductus arteriosus (PDA) in 16 infants, and sole division of a PDA in another 16 infants with large left to right shunts due to common canalis atrioventricularis (avcanal) or ventricular septal defect (VSD). An aorto-pulmonary anastomosis was performed for relief of severe hypoxia due to right ventricular outflow tract obstruction (RVOTO) in 14 patients. Operative mortality was 41% for PAB, 21% for aortopulmonary anastomosis, anastomosis, and 0% for division of a PDA. In the group of corrective cardiac surgery the operative mortality rate was 0% in ostium primum defects and 4% in VSD closure. Correction of complete av-canal and of malformations with RVOTO had a high mortality rate of 20 and 40%, respectively. Similar results were obtained in nonmongoloid children operated upon for the same cardiac defects. The complications and causes of death were due to the complexity of the cardiac malformations and had no relation to the Down's Syndrome itself. Mongoloid children do not pose additional medical problems to management of congenital heart disease. Their results do not differ from those obtained in nonmongoloid children.
Pediatr Cardiol. 1992 Apr;13(2):80-4.
Down's syndrome affects results of surgical correction of complete atrioventricular canal.Morris CD, Magilke D, Reller M.
Department of Pediatrics, Oregon Health Sciences University, Portland 97201.
From 1981 through June 1989, 59 children had surgery for a complete atrioventricular (AV) canal defect at Oregon Health Sciences University. We compared the morbidity, mortality, and hemodynamic status of 47 children with and 12 without Down's syndrome through review of operative, clinical, and cardiac catheterization records. Overall, 10 children with Down's syndrome have died, nine from cardiac cause within 90 days of surgery. The 2-year survival of these children was 77 +/- 6% as compared to 100% in children without Down's syndrome (p = 0.08). Early age at surgery, the surgical anatomy of the AV canal, and gender had no significant effect on survival. Before surgery, the hemodynamic status of Down's syndrome and non-Down's syndrome children did not differ; in a small group of post-operative catheterization, right heart pressures and pulmonary vascular resistance remained significantly higher in the Down's syndrome as compared to non-Down's syndrome children. These trends to higher mortality and poorer postoperative hemodynamics in children with Down's syndrome may necessitate closer follow-up if confirmed in other cohorts.
Thoraxchir Vask Chir. 1976 Oct;24(5):424-30.
[Palliative and corrective cardiac surgery in Down's syndrome (author's transl)][Article in German]
Seybold-Epting W, Hoffmeister HE, Stunkat R.
Since 1970 46 infants and children with Down's Syndrome were subjected to palliation of congenital heart disease, and 54 mongoloid children underwent correction of their cardiac defects. The most common cardiac malformation was endocardial cushion defect (72%). Palliation consisted in pulmonary artery banding (PAB) with or without division of a patent ductus arteriosus (PDA) in 16 infants, and sole division of a PDA in another 16 infants with large left to right shunts due to common canalis atrioventricularis (avcanal) or ventricular septal defect (VSD). An aorto-pulmonary anastomosis was performed for relief of severe hypoxia due to right ventricular outflow tract obstruction (RVOTO) in 14 patients. Operative mortality was 41% for PAB, 21% for aortopulmonary anastomosis, anastomosis, and 0% for division of a PDA. In the group of corrective cardiac surgery the operative mortality rate was 0% in ostium primum defects and 4% in VSD closure. Correction of complete av-canal and of malformations with RVOTO had a high mortality rate of 20 and 40%, respectively. Similar results were obtained in nonmongoloid children operated upon for the same cardiac defects. The complications and causes of death were due to the complexity of the cardiac malformations and had no relation to the Down's Syndrome itself. Mongoloid children do not pose additional medical problems to management of congenital heart disease. Their results do not differ from those obtained in nonmongoloid children.
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Igor Bulatov
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Мы оперируем таких детей в возрасте до 6 месяцев.У ребенка с Даун синдромом повышен риск быстрого развития легочной гипертензии при наличии AV канала или большого дефекта межжелудочковой перегородки.Поэтому,наиболее часто операции делаются в возрасте 1-2 месяцев.За последние 7 лет ни один ребенок,подвергшийся подобной операции у нас, не умер.
http://www.childrenscentralcal.org/cont ... upid=G0044
http://www.childrenscentralcal.org/cont ... upid=G0044