先天性心脏病体外循环术后透析治疗临床研究——附41例临床分析

目的:总结和分析先天性心脏病(先心病)体外循环术后透析治疗患者的临床特点,探讨临床相关危险因素。方法:以2006年1月至2009年12月于我科行先心病体外循环手术,并于术后行透析治疗的41例患者为研究对象,回顾性分析其临床资料并总结其临床特点。结果:1.体外循环手术各病种透析发生率,由高至低依次为左冠状动脉起源于肺动脉、完全性大动脉转位及右心室双出口、法洛四联症7.62%、完全型房室隔缺损、主动脉弓缩窄/离断、单心室、完全性肺静脉异位回流及室间隔缺损。2.透析患者中存活23例(23/41,56.1%),存活患者尿量及肾功能均恢复正常。死亡18例(18/41,43.9%)。死亡患者中以完全型房、室间隔缺损、法洛四联症最常见。3.透析患者体外循环时间95～395 min,平均185 min,心肌阻断时间82～188 min,平均132 min。4.透析患者手术风险分布:1级1例,2级24例,3级14例,4级5例。5.出现少尿与透析开始时间相距(1～9)h,平均5 h,透析持续时间10～1 008 h,平均75 h,透析效果满意。6.41例透析患者全部合并诊断低心排出量综合征(低心排),18例死亡患者中5例诊断多器官功能障碍综合征。结论:低年龄、长时间体外循环及心肌阻断时间、畸形矫治不彻底、术后低心排是透析治疗的有关危险因素,正确掌握透析适应证及时机是治疗关键。     

120例先天性心脏病术后心律失常的分析

目的：探讨先天性心脏病心脏直视术后心律失常的发生情况。方法：120例先天性心脏病患者(室间隔缺损86例，房间隔缺损34例)在心脏直视缺损修补术后转入监护室持续心电监护，随时描记标准12导联心电图，了解发生心律失常的类型及预后。结果：59．2％(67／120)的患者术后发生心律失常，其中室缺修补术后发生心律失常的患者占47．5％，以右柬支传导阻滞为主；房缺修补术后发生心律失常的患者14例，占34例的41％，以阵发性室上速和右柬支传导阻滞为主。结论：先心病手术后发生心律失常的情况与缺损部位，手术方式、路径及术后血流动力学的稳定状况有极大的关系。     

儿童先天性心脏病介入治疗术后镇痛研究进展

介入手术治疗是目前先天性心脏病患儿常见的临床治疗方式之一,但术后不仅会加快患儿心率、增加血压和氧消耗量,而且因疼痛引起的咳嗽还会引起肺不张和低氧血症等并发症的发生,不利于患儿术后的康复,并严重影响患儿及其家长的生活质量。本文就儿童先天性心脏病介入治疗术后影响疼痛发生的因素、术后疼痛对机体的影响以及目前常用的术后镇痛的方法等进行文献综述     

儿童先天性心脏病术后认知功能障碍的研究现状与进展

影响儿童先天性心脏病术后神经认知功能的原因很多,先天性大脑发育异常、遗传综合征及心脏畸形导致的大脑缺血缺氧增加了术后神经功能障碍的危险性.术中麻醉、体外循环及深低温停循环时间、主动脉阻断时间、炎症反应及大脑灌注不足、微栓等均可加重神经发育障碍;患儿术后恢复情况亦会影响远期认知功能.     

儿童先天性心脏病术后认知功能障碍的研究现状与进展

影响儿童先天性心脏病术后神经认知功能的原因很多,先天性大脑发育异常、遗传综合征及心脏畸形导致的大脑缺血缺氧增加了术后神经功能障碍的危险性。术中麻醉、体外循环及深低温停循环时间、主动脉阻断时间、炎症反应及大脑灌注不足、微栓等均可加重神经发育障碍;患儿术后恢复情况亦会影响远期认知功能。     

小儿先天性心脏病体外循环前、中、后不同阶段液体管理的临床观察

目的：观察小儿先天性心脏病体外循环（ＣＰＢ）前、中、后不同阶段液体出入量平衡，为围ＣＰＢ期的液体管理提供参考。方法：各类先天性心脏病患儿共计８０例，分别统计ＣＰＢ前、中、后有关血液动力学指标、尿量、输入晶体液量、输血量和时间等，同时取静脉血样进行血气分析。结果：小于１岁患儿液体总入量：ＣＰＢ前２２．３±８．４ｍｌ／（ｋｇｈ），ＣＰＢ中７８．３±３２．８ｍｌ／ｋｇ，ＣＰＢ后１８．７±１４．５ｍｌ／（ｋｇｈ）。１～３岁（包括３岁）患儿液体总入量：ＣＰＢ前１７．６±８．０ｍｌ／（ｋｇｈ），ＣＰＢ中４８．３±１６．１ｍｌ／ｋｇ，ＣＰＢ后６．９±１８．１ｍｌ／（ｋｇｈ）。３～６岁患儿液体总入量：ＣＰＢ前１４．８±５．０ｍｌ／（ｋｇｈ），ＣＰＢ中３７．２±１４．２ｍｌ／ｋｇ，ＣＰＢ后４．４±９．６ｍｌ／（ｋｇｈ）。结论：不同年龄段及患儿ＣＰＢ心脏手术不同阶段液体总入量不同     

阿魏酸钠对小儿先天性心脏病体外循环炎症反应的影响

将60例小儿先天性心脏病患者随机分为阿魏酸钠组（S组）和对照组（C组）各30例。两组均于体外循环（CPB）下手术,S组在CPB预充液中加入阿魏酸钠注射液8mg／kg,C组加入等剂量醋酸林格液。分别于体外循环前（T0）、结束（T1）、术后6h（T2）、术后24h（T3）抽取血标本,测定血清中肿瘤坏死因子（TNF-α）、白细胞介素6（IL-6）、白细胞介素10（IL—10）的浓度。结果CPB后两组TNF—α、IL-6、IL-10均明显增高,至T2达最高峰;与C组相比,S组T1、T2、T3时TNF—α和IL-6均降低,而IL—10增高。认为阿魏酸钠可通过减少CPB中促炎因子TNF-α和IL-6释放、增加抑炎因子IL-10分泌而减轻体外循环炎症反应。     

非体外循环下双向Glenn术治疗复杂先天性心脏病

目的探讨双向腔静脉—肺动脉吻合术（双向Glenn术）对复杂先天性心脏病的疗效。方法 2003年12月～2007年12月,对我院24例复杂先天性心脏病患者在非体外循环下行双向Glenn术。结果全组无手术死亡,14例术后出现头部水肿,10例出现胸腔积液,均经保守治疗痊愈。术后血氧饱和度、肺动脉压均较术前提高。结论非体外循环下的双向Glenn术是一种有效治疗复杂性先天性心脏病的方法。     

改良预充液用于婴幼儿体外循环效果观察

目的 通过对婴幼儿体外循环预充液的改良调整,对比其对婴幼儿术中术后内环境、血流动力学及术后ICU各项指标的影响.方法 将40例室间隔缺损（VSD）患儿随机分为常规组及改良组各20例.两组均于体外循环（CPB）下行VSD修补术.术中常规组采用库存血预充;改良组对库存血预充液进行零平衡超滤（ZBUF）,然后根据测得血气结果调整预充液pH值使其更接近机体内环境.观察两组血气分析指标及电解质、炎症因子TNF-α及IL-8水平变化;比较两组术后胸腔引流量、呼吸机辅助时间、ICU留观时间及血流动力学差异.结果 改良组预充液pH、PCO2、BE、K＋均低于常规组（P＜0.01）并达到正常生理范围;T1b时TNF-α水平低于T1a时及常规组T1时（P＜0.05）,T3、T4时TNF-α水平均低于常规组（P＜0.05）;T1b时IL-8水平低于T1a时及常规组T1时（P＜0.05）;T2、T3、T4时IL-8水平低于常规组（P＜0.05）;ICU期间呼吸机辅助时间短于常规组（P＜0.05）.结论 对库存血预充液行零平衡超滤改良调整后更适合婴幼儿体内环境,可明显减轻炎症反应、缩短呼吸机辅助时间、改善血流动力学、促进各器官功能恢复.     

非体外循环下复杂先天性心脏病的姑息手术

目的:探讨非体外循环下复杂先天性心脏病姑息手术适应证及处理要点。方法:2008-01-2010-06对26例复杂先天性心脏心病患儿实行非体外循环下姑息手术,其中肺动脉环缩术5例,改良体肺分流术6例,双向腔肺分流术14例,双向腔肺分流+改良体肺分流术1例;其中功能单心室9例,法洛四联症肺动脉闭锁6例,右室双出口4例,三尖瓣闭锁3例,完全性大动脉转位2例完全心内膜垫缺损并肺动脉闭锁1例,Ebstein畸形1例。结果:死亡4例,22例患者顺利出院,死亡率15.4%;死亡原因包括低氧血症及心功能衰竭、肺部感染合并心功能衰竭;肺动脉环缩术、改良体肺分流术、双向腔肺分流术后平均血氧饱和度分别为(81.8±2.4)%、(90.0±3.2)%、(86.4±4.1)%;术后并发症包括肺部感染3例,胸腔积液7例,上腔静脉综合征1例。术后随访2个月～1年,无远期死亡,心功能NYHAⅠ级7例,NYHAⅡ级15例。结论:非体外循环下姑息治疗是复杂先天性心脏病的有效治疗手段,特别适用于有体外循环高危因素患者,手术适应证的正确把握、术中术后合理的处理是降低死亡率的关键。     

Preoperative Evaluation of Adult Congenital Heart Disease Patients for Non-cardiac Surgery

Purpose of Review

Highlight the extracardiac comorbidities that adult congenital heart disease patients and summarize the current recommendations for non-cardiac surgery.

Recent Findings

Adult congenital heart disease patients are living longer, becoming more complex and developing adult comorbidities as they age. These patients have multiorgan involvement including higher prevalence of kidney disease and decreased lung function. Non-cardiac comorbidities can complicate surgery in this patient population. Most patients have non-cardiac surgery in community settings. These settings may increase adverse events during and in the post-operative period. Survival is improved when moderate and complex patients are seen in regional referral centers.

Summary

Improved awareness of long-term complications of congenital heart disease and extracardiac comorbidities for adult congenital heart disease patients is needed. Appropriate care settings with both congenital heart disease expertise and adult subspecialty care reduces morbidity and mortality in these complex patients.

     

Use of Implantable Cardioverter-Defibrillators in Congenital Heart Disease and Pediatric Patients: Results from the German National Registry for Congenital Heart Defects

Background: Sudden cardiac death is a leading cause of death in patients with congenital heart disease (CHD). Risk stratification for implantable cardioverter defibrillators (ICD) remains difficult due to limited data about use and outcome of device therapy in CHD patients in larger community-based cohorts. Methods and results: Out of a dataset with more than 50,000 patients registered at the German National Register for Congenital Heart Defects, 109 patients (median age 35.5; IQR 23.75–46.00), 68 (62%) male) with an ICD were identified and were retrospectively analyzed. Although the number of implantations increased steadily throughout the investigated time interval from 2001 to 2015, only 0.2% of the CHD patients in the national register received an ICD. Indication for ICD implantation was secondary prevention in 84 patients (78%) and primary prevention in 24 patients (22%). 23 patients (21%) of the ICD patients received appropriate ICD therapy. 7 patients (6%) received an inappropriate ICD therapy. In 23 patients (21%) device complications were documented with a high number of lead fractures and insulation defects (n = 14,13%). Conclusion: The current study investigates the clinical uptake and use of ICD therapy based on a large national registry for CHD patients. Despite a steady increase in the number of implanted devices, ICD uptake remains relatively low, particularly for primary prevention. The data suggests a potential reluctance in utilization of device therapy in this patient cohort for primary prevention. Selecting patients in whom benefits outweigh the risks associated with lifelong ICD therapy remains challenging.     

Impact of Major Residual Lesions on Outcomes After Surgery for Congenital Heart Disease

BackgroundMany factors affect outcomes after congenital cardiac surgery.ObjectivesThe RLS (Residual Lesion Score) study explored the impact of severity of residual lesions on post-operative outcomes across operations of varying complexity.MethodsIn a prospective, multicenter, observational study, 17 sites enrolled 1,149 infants undergoing 5 common operations: tetralogy of Fallot repair (n = 250), complete atrioventricular septal defect repair (n = 249), arterial switch operation (n = 251), coarctation or interrupted arch with ventricular septal defect (VSD) repair (n = 150), and Norwood operation (n = 249). The RLS was assigned based on post-operative echocardiography and clinical events: RLS 1 (trivial or no residual lesions), RLS 2 (minor residual lesions), or RLS 3 (reintervention for or major residual lesions before discharge). The primary outcome was days alive and out of hospital within 30 post-operative days (60 for Norwood). Secondary outcomes assessed post-operative course, including major medical events and days in hospital.ResultsRLS 3 (vs. RLS 1) was an independent risk factor for fewer days alive and out of hospital (p ≤ 0.008) and longer post-operative hospital stay (p ≤ 0.02) for all 5 operations, and for all secondary outcomes after coarctation or interrupted arch with VSD repair and Norwood (p ≤ 0.03). Outcomes for RLS 1 versus 2 did not differ consistently. RLS alone explained 5% (tetralogy of Fallot repair) to 20% (Norwood) of variation in the primary outcome.ConclusionsAdjusting for pre-operative factors, residual lesions after congenital cardiac surgery impacted in-hospital outcomes across operative complexity with greatest impact following complex operations. Minor residual lesions had minimal impact. These findings may provide guidance for surgeons when considering short-term risks and benefits of returning to bypass to repair residual lesions.     

Speckle Tracking Echocardiography in Pediatric and Congenital Heart Disease

Assessment of myocardial strain using speckle tracking echocardiography is an emerging echocardiographic technique that is increasingly used in the diagnosis and management of acquired heart disease in adults. In pediatric heart disease, this is still mainly considered as a research tool as the application of this technology has been slowed by the lack of vendor‐independent technology and of normative data across the different age ranges. We believe that the technology has potential applications for the early detection of myocardial dysfunction, the quantification of ventricular function in congenital heart disease, and the detection of dyssynchrony.     

Cardiopulmonary Bypass and Serum Thyroid Hormone Profile in Pediatric Patients with Congenital Heart Disease

Objective: To study the effect of cardiopulmonary bypass (CPB) on serum thyroid hormone profile in children undergoing open‐heart surgery. Design: Prospective cross‐sectional study. Setting: Multispecialty tertiary level referral center. Patients: One hundred consecutive patients (age 15.9 ± 14.6 months, weight 6.7 ± 2.5 kg) undergoing open‐heart surgery under CPB. Interventions: None. Outcome Measures: Levels and trends of serum total thyroxine (TT4), free thyroxine (FT4), total tri‐iodothyronine (TT3), free tri‐iodothyronine (FT3) and thyroid stimulating hormone (TSH), survival, inotropic score, duration of mechanical ventilation, postoperative complications. Results: TT4 levels were 9.08 ± 3.6, 6.4 ± 2.5, 6.24 ± 2.1, 6.43 ± 2.4, 7.20 ± 3.0 µg/dL at baseline and at 1, 24, 48 and 72 hours; FT4 levels were 1.82 ± 0.5, 1.49 ± 0.3, 1.29 ± 0.3, 1.32 ± 0.4, and 1.43 ± 0.5 ng/dL; TT3 levels were 1.81 ± 0.4, 1.31 ± 0.3, 0.99 ± 0.2, 1.0 ± 0.37, and 1.17 ± 0.48 ng/ml; FT3 levels were 4.09 ± 1.0, 3.02 ± 0.8, 2.21 ± 0.6, 2.22 ± 0.7, and 2.66 ± 1.05 pg/ml; TSH levels were 5.40 ± 3.8, 2.0 ± 3.1, 1.24 ± 1.1, 2.90 ± 3.3, and 4.03 ± 3.4 mIU/L. There was significant fall (29.1% for FT4, 32.1% for TT4, 77% for TSH, 46% for FT3 and 45% for TT3, p < 0.0001). When area under curve (AUC) TT4 was compared between survivors (n = 87) and nonsurvivors (n = 12), significantly larger AUC was seen in survivors (492.81 ± 158.6) than nonsurvivors (360.75 ± 179.6 p = 0.0125). In survivors >72 hours, AUC TT4 was larger in patients with uneventful postoperative course versus those with postoperative complications (516.48 ± 18.6 vs. 394.78 ± 29.9, p = 0.001). AUC TT4 showed significant inverse correlation with inotropic score and borderline inverse correlation with duration of mechanical ventilation. Conclusion: Children undergoing surgery under CPB showed significant fall in thyroid hormones. Because TT4 level is modifiable, prophylactic administration of TT4 for improving outcomes needs to be studied further.