ECMO for neonatal respiratory failure

Extracorporeal membrane oxygenation (ECMO) has been offered as a life-saving technology to newborns with respiratory and cardiac failure refractory to maximal medical therapy. ECMO has been used in treatment of neonates with a variety of cardio-respiratory problems, including meconium aspiration syndrome (MAS), persistent pulmonary hypertension of the neonate (PPHN), congenital diaphragmatic hernia (CDH), sepsis/pneumonia, respiratory distress syndrome (RDS), air leak syndrome, and cardiac anomalies. For this group of high-risk neonates with an anticipated mortality rate of 80% to 85%, ECMO has an overall survival rate of 84%, with recent data showing nearly 100% survival in many diagnostic groups. This article reviews the current selection criteria for ECMO and the clinical management of neonates on ECMO, and discusses the long-term outcome of neonates treated with ECMO.     

The use of extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia

The use of extracorporeal membrane oxygenation (ECMO) has revolutionized the care of the critical infant born with a congenital diaphragmatic hernia (CDH). In some respects, this is surprising given our current lack of understanding regarding optimal preoperative ventilation strategy, identification of patients most likely to benefit from ECMO, and the correct timing of hernia repair for the infant treated with ECMO. Historically, repair of CDH was considered one of the few true pediatric surgical emergencies. Mortality, however, was high. In the 1970s, ECMO was first utilized as a rescue therapy following repair of CDH when conventional methods failed. In the 1980s, advancements in neonatal intensive care and an understanding of the pathophysiology of pulmonary hypertension associated with CDH led to a strategy involving preoperative stabilization and delayed surgical intervention. Historical reviews demonstrate an improvement of survival in infants treated with ECMO from 56% to 71%. This paper will outline the advances in the care of the CDH patient and the approach used for treatment with ECMO.     

The effect of extracorporeal life support on the brain: a focus on ECMO

Extracorporeal membrane oxygenation (ECMO) therapy has significantly improved outcome in the newborn, pediatric, and adult patient in respiratory and cardiac failure. Despite this therapy providing a life-saving technology, the morbidity in patients treated with ECMO therapy is primarily related to neurologic alterations and not pulmonary findings. For ECMO, this is not unexpected since most patients are being placed on ECMO support because of severe hypoxemia, with ECMO being considered a rescue therapy for respiratory failure in most instances. As use of ECMO becomes common place for infants and children in respiratory failure, our investigations into the outcome of these children must focus not only on survival versus nonsurvival, but on the causes of morbidity in this population. A further understanding of factors associated with morbidity may allow us to alter techniques used in extracorporeal life support (ECLS), hopefully to improve our long-term outcome in this population, while allowing us to expand use of these technologies to other populations such as the premature infant. This article will focus on the effect of ECMO on the brain, with the following chapter by Dr. Richard Jonas outlining the effect of cardiopulmonary bypass on the brain.     

An overview of medical ECMO for neonates

Extracorporeal membrane oxygenation (ECMO), a life-saving therapy for respiratory and cardiac failure, was first used in neonates in the 1970s. The indications and criteria for ECMO have changed over the years, but it continues to be an important option for those who have failed other medical therapies. Since the Extracorporeal Life Support Organization (ELSO) Registry was established in 1989, more than 29,900 neonates have been placed on ECMO for respiratory failure, with 84% surviving their ECMO course, and 73% surviving to discharge or transfer. In this chapter, we will review the basics of ECMO, patient characteristics and criteria, patient management, ECMO complications, special uses of neonatal ECMO, and patient outcomes.     

妊娠合并先天性心脏病伴肺动脉高压79例妊娠结局分析

目的分析妊娠合并先天性心脏病伴肺动脉高乐患者的妊娠结局。方法回顾性分析2009年1月至2013年6月北京安贞医院妇产科收治的79例妊娠合并先天性心脏病伴肺动脉高压患者的临床资料,根据肺动脉压力将其分为轻度组、中度组、重度组。分析各组先天性心脏病种类、心功能级别、终止妊娠的方式以及母婴结局。结果先心病类型以房间隔缺损（房缺）和室间隔缺损（室缺）为主,肺动脉高压轻度组心功能以Ⅰ～Ⅱ级为主,重度组心功能以Ⅲ～Ⅳ级为主。79例患者中,行医源性流产者16例（20．8％）,经阴道分娩5例（6．5％）,剖宫产56例（72．7％）。重度组医源性流产率高于其他组,足月分娩率低于其他组,差异有统计学意义（P〈0．05）。新生儿早产20例（32．8％）,足月产41例（67．2％）,死产1例,随着肺动脉压力的增加,早产的发生率增加,三组之间相互比较差异有统计学意义（P〈0．05）。结论肺动脉高压患者应在妇产科及心脏科医师指导下妊娠,不宜妊娠者应及早终止妊娠。孕产妇终止妊娠方式以剖宫产为主,麻醉方式以硬膜外麻醉为宜。     

Neonatal ECMO: neuroimaging and neurodevelopmental outcome

Intracranial injury continues to be a major complication associated with extracorporeal membrane oxygenation (ECMO)-treated neonates. The reported frequency of abnormal neuroimaging has ranged from 28% to 52%, depending on neuroimaging techniques and methods of classification. The purpose of this chapter is to describe types of imaging techniques commonly used to evaluate the ECMO neonate, to specify different types of injuries that have been reported, and to identify factors which increase the risk of injury. We will then describe the functional impact at age 5 years following neonatal brain injury among ECMO infants.     

The effect of extracorporeal life support on the brain: cardiopulmonary bypass

This article reviews the mechanisms of brain injury associated with cardiopulmonary bypass. These include embolic injury of both a gaseous and particulate nature as well as global hypoxic ischemic injury. Ischemic injury can result from problems associated with venous drainage or with arterial inflow including a steal secondary to systemic to pulmonary collateral vessels. Modifications in the technique of cardiopulmonary bypass have reduced the risk of global hypoxic/ischemic injury. Laboratory and clinical studies have demonstrated that perfusion hematocrit should be maintained above 25% and preferably above 30%. Perfusion pH is also critically important, particularly when hypothermia is employed. An alkaline pH can limit cerebral oxygen delivery by inducing cerebral vasoconstriction as well as shifting oxyhemoglobin dissociation leftwards. If deep hypothermia is employed, it is critically important to add carbon dioxide using the so-called "pH stat" strategy. Oxygen management during cardiopulmonary bypass is also important. Although there is currently enthusiasm for using air rather than pure oxygen, ie, adding nitrogen, this does introduce a greater risk of gaseous nitrogen emboli since nitrogen is much less soluble than oxygen. The use of pure oxygen in conjunction with CO2 to apply the pH stat strategy is recommended. Many of the lessons learned from studies focusing on brain protection during cardiopulmonary bypass can be applied to the patient being supported with extracorporeal membrane oxygenation.     

Adult congenital heart disease and pregnancy

Adults with congenital heart disease now form the largest group of women with cardiac disease becoming pregnant in the developed world. This is both a mark of impressive steps forward in the management of congenital heart disease and also a challenge to the medical community to develop systems of care that will best serve these women and their babies. Each woman with congenital heart disease presents a unique pattern of challenges for the cardiologist, obstetrician, and anesthesiologist, and their care should be tailored to deal with their individual circumstances. As this population of patients continues to grow, we must continue to learn and improve our diagnostic tools and management strategies to refine their care. This review intends to focus on reviewing the outcomes in this set of patients and also an approach to the assessment and the management of these patients, primarily for an audience of obstetricians, pediatricians, and anesthesiologists.     

经食管超声心动图在小儿先天性心脏病围术期的应用价值

目的 评价经食管超声心动图(TEE)对指导小儿先天性心脏病(先心病)外科手术和介入治疗的应用价值及其安全性。方法 2000年11月至2005年1月，在上海市复旦大学附属儿科医院心血管中心接受外科开胸手术或经导管介入治疗的先心病患儿中有317例进行了TEE检查，年龄2个月至17岁6个月(平均4.7岁)。采用HP/SONOS2500超声诊断仪，频率5.5~7.5MHz双平面经食管探头，全麻状态下进行。结果 与术前经胸超声心动图(TTE)比较，术前TEE检查对诊断作出修正或补充者51例(16.1%)，其中因此而修正了手术治疗方案25例(7.9%)。术后TEE检查发现有并发症或残余问题57例(18.0%)，其中8例(2.5%)因此立即再次手术。所有病例均未因TEE检查而引起并发症。结论 TEE可安全地应用于小儿先心病围术期检查，对术前诊断做出修正或补充，术后可及时发现并发症或残余问题。 Abstract Objective To evaluate the utility of transesophageal echocardiography (TEE) for surgical and interventional repairs in children with congenital heart disease (CHD) and its safety as well.Methods 317 patients with CHD underwent TEE examinations at the age of 2 months~17 years and 6 months(mean 4.7 years).HP/SONOS2500 ultrasonic instrument with transesophageal biplane probe of 5.5~7.5MHz was used to perform TEE under general anesthesia.Results Compared with preoperative transthoracic echocardiography(TTE),TEE had new findings or made revision of the diagnoses in 51 cases(16.1%) before operations,which led to the changes of surgical or interventional precedure in 25 cases(7.9%).TEE had found residual problems or evolving situations in 57 cases(18.0%) after operations,which led to a second immediate surgical intervention in 8 cases(2.5%).There were no detectable complications due to TEE in all cases.Conclusion TEE is valuable in providing meaningful preoperative emendations or additions and finding residual problems after operations. The technique is safe in children. Key words Transesophageal echocardiography (TEE);Congenital heart disease (CHD);Perioperative period;Children     

妊娠合并先天性心脏病的综合管理

医学进步使得更多先天性心脏病妇女可以生存至生育期年龄。妊娠后母体和胎儿并发症风险都会增加,心脏功能也会进一步损害。风险取决于先天性心脏病类型、血流动力学受损程度及并发症情况。加强对此类患者的综合管理,进行仔细的个体化、多学科的风险评估,制定详细的妊娠期随访、分娩和产后治疗计划可降低风险,改善围产结局,对降低孕产妇病死率具有重要的意义。     

Update on extracorporeal life support 2004

Since its beginnings in 1989, the Extracorporeal Life Support Organization (ELSO) Registry has collated and reported data on over 30,000 patients. The majority of patients entered into the Registry have been neonates with respiratory failure from meconium aspiration, persistent pulmonary hypertension, or congenital diaphragmatic hernia. These patients suffer from refractory hypoxemia; thus, this supportive technique came to be called "Extracorporeal Membrane Oxygenation (ECMO)" for its ability to provide excellent gas exchange. With advances in prevention, diagnosis, and treatment measures for neonatal respiratory failure, need for ECMO support has fallen from the peak of 1500 cases in the early 1990s to 800 cases annually. Sixty-six percent (over 19,000) of patients in the Registry are under the category of neonatal respiratory failure, with a 77% overall survival reported to discharge. The success of neonatal ECMO has led to expansion of the field to pediatric, cardiac, and adult patients. An average of 200 pediatric patients receive ECMO for respiratory failure per year with an overall survival of 55%. Adult respiratory failure patients form a smaller group, with less than 100 cases reported to the ELSO registry per year. Survival mirrors that noted in the pediatric ECMO population. The application of ECMO or related techniques continues to increase for cardiac failure across all age groups. Overall survival in cardiac patients ranges from 33% to 43%. A novel form of extracorporeal support is "ECPR" or ECMO during cardiac arrest. Bypass circuits and equipment can be set up and instituted within a very short period of time in this circumstance, thus the name "rapid deployment ECMO" has become associated with this form of support. Overall survival in the near-600 patients placed on ECMO during resuscitation is 40%.     

妊娠合并先天性心脏病的围产期管理

过去几十年,随着诊断和治疗手段的发展,先天性心脏病（先心病）患者生存率显著提高。越来越多先心病女性患者可以存活到生育年龄,并考虑生育问题。与普通孕妇相比,先心病患者妊娠其母体风险和新生儿并发症,如早产、胎儿生长受限、胎儿死亡或新生儿死亡率增加。如何降低孕产妇病死率和改善围产结局?文章回顾妊娠合并先天性心脏病的相关文献,对围产期管理进行阐述。     

儿童非紫绀型先天性心脏病体外循环术后急性肾损伤相关危险因素分析

目的探究儿童非紫绀型先天性心脏病体外循环术后急性肾损伤(AKI)相关危险因素。方法选取2013年8月至2016年8月在湖南省儿童医院心胸外科行体外循环手术治疗的非紫绀型先天性心脏病患儿450例作为研究对象,将患儿按术后是否出现AKI进行分组,AKI组80例,非AKI组370例。收集患儿的一般资料及临床资料,通过单因素分析以及Logistic回归分析探究其相关危险因素。结果两组患儿在性别、体质量、尿素氮、左室射血分数、血红蛋白方面比较差异无统计学意义(P0.05)。两组在年龄、血肌酐、肺炎或心力衰竭史、体外循环时间及主动脉阻断时间、术后低血压方面比较差异有统计学意义(P0.05)。通过Logistic对因素回归分析发现,年龄≤1岁、肺炎或心力衰竭史、体外循环时间以及主动脉阻断时间是危险因素。结论在非紫绀型先天性心脏病患儿行体外循环手术治疗中,选择手术年龄、缩短体外循环时间、缩短主动脉阻断时间以及预防并发症是有效的控制手段,可以降低患儿AKI的发病率。     

44例妊娠合并先天性心脏病母儿结局临床分析

目的探讨孕妇先天性心脏病类型与母儿不良事件的相关性。方法回顾性分析1998年1月至2008年12月北京大学人民医院产科分娩的44例先天性心脏病孕妇及胎儿临床资料。结果孕妇先天性心脏病类型前3位依次为房间隔缺损36.36%(16/44)、动脉导管未闭18.18%(8/44)、室间隔缺损15.91%(7/44)和二尖瓣脱垂15.91%(7/44)。共发生20次不良事件,其中5次为心脏不良事件,15次为产科不良事件。发生不良事件的前3位孕妇先天性心脏病的类型依次为房间隔缺损40%(8/20)、动脉导管未闭20%(4/20)和主动脉瓣狭窄20%(4/20)。产科不良事件中,子痫前期3例,早产4例,胎儿生长受限2例,新生儿需入住NICU3例,新生儿轻度窒息2例,子代先心病1例。结论孕妇先天性心脏病中房间隔缺损合并肺动脉高压可能会增加母儿风险,但大部分母儿结局良好。     

Fetal congenital heart disease and intrauterine growth restriction: a retrospective cohort study

Objective: Fetal congenital heart disease may lead to abnormal fetal growth. Our objective was to estimate the association between fetal congenital heart disease (CHD) and intrauterine growth restriction (IUGR) in an effort to better inform clinical management of continuing pregnancies complicated by fetal congenital heart disease. Methods: In a retrospective cohort study, outcome data was collected from singleton pregnancies undergoing routine anatomic survey at a tertiary medical center between 1990 and 2008. Dedicated research nurses collected information on delivery outcomes in an on-going manner. Subjects with a prenatal diagnosis of fetal CHD were compared to those without CHD. Stratified analyses for isolated fetal CHD and major CHD were performed. The primary outcome was IUGR less than 10th percentile by the Alexander growth standard. Logistic regression was used to adjust for confounding variables and refine the estimates of risk. Results: Among 67,823 patients, there were 193 cases of fetal CHD (0.3%) and 5,669 cases of IUGR (8.4%). Prenatal diagnosis of CHD was associated with an increased risk of IUGR (23.8% vs. 8.5%, adjusted odds ratio [aOR] 3.3, 95% confidence interval [CI] 2.4–4.6), and the risk was greatest in fetuses with major CHD (16.5% vs. 8.5%, aOR 2.1, 95% CI 1.3–3.2). Isolated CHD was also associated with an increased risk of IUGR (17.8% vs. 8.5%, aOR 2.2, 95% CI 1.4–3.7). Conclusion: Patients with a prenatal diagnosis of fetal CHD have a three-fold increase in risk of developing IUGR; patients with isolated fetal CHD are twice as likely to develop IUGR. Based on our findings, serial growth assessment may be a reasonable option for patients with fetal CHD diagnosed at routine anatomic survey.     

Predictors of psychological functioning in mothers and fathers of infants born with severe congenital heart disease

This study examined mental health and coping styles in both mothers and fathers of infants born with a severe congenital heart defect. Factors associated with mental health outcomes were elucidated. Parents of 70 infants, recently born with a severe congenital heart defect, completed questionnaires which examined psychological functioning and coping strategies. Disease, surgical and psychosocial factors were examined for their significance in predicting psychological functioning. Findings indicated elevated levels of clinically significant psychological distress in mothers, compared to fathers, and differences between parents in coping styles. Regression analyses suggested that the extent of distress in both parents was not primarily predicted by illness or demographic factors. Rather, certain coping styles, knowledge, subjective worry and family functioning emerged as significant predictive variables. Implications for early intervention are discussed.