Delany Dennis R, Gaydos Stephanie S, Romeo Deborah A, Henderson Heather T, Fogg Kristi L, McKeta Angela S, Kavarana Minoo N, Costello John M
Department of Pediatrics, Division of Pediatric Cardiology, Medical University of South Carolina, 10 McClennan Banks Drive, MSC915, Charleston, SC 29425 USA.
Department of Medicine, Division of Cardiology, Medical University of South Carolina, 30 Courtenay Drive, MSC 592, Charleston, SC 29425 USA.
J Congenit Cardiol. 2021;5(1):7. doi: 10.1186/s40949-021-00061-3. Epub 2021 Apr 20.
Approximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.
约50%的唐氏综合征新生儿患有先天性心脏病。也可能存在非心脏合并症。先天性心脏病患者围手术期评估和管理的许多原则及策略适用于唐氏综合征患者。尽管如此，仍需要对心脏手术进行精心规划，评估心脏和非心脏疾病，并仔细考虑肺动脉高压的风险。在本手稿中，对于患有唐氏综合征且有血流动力学显著意义的先天性心脏病患儿，我们将总结需要干预的心脏缺陷的流行病学情况。我们将回顾针对这一特殊人群的围手术期规划，包括麻醉注意事项、常见术后问题、营养策略和出院规划。还将讨论单心室姑息治疗和心脏移植评估的特殊注意事项。总体而言，唐氏综合征儿科患者进行心脏手术的死亡率不高于普通人群，但功能性单心室心脏缺陷患者除外。潜在的合并症可能导致术后并发症并延长住院时间。深入了解唐氏综合征患儿的心脏和非心脏相关因素将有助于临床医生优化围手术期护理和长期治疗效果。
