先天性心脏病合并尿道下裂的临床研究

目的:研究先天性心脏病合并尿道下裂的发病情况和两者的关联性。方法:调查2002年1月至2012年12月我院先天性心脏病男性患儿中合并尿道下裂的发病率和程度,与普通人群尿道下裂发病率进行比较,并分析不同类型先天性心脏病与尿道下裂发病率和程度的相关性。结果:本组先天性心脏病男性患儿7 385例,合并尿道下裂者134例,总体发病率1.81%,高于普通人群的0.33%~0.40%,差异有统计学意义(P0.01)。其中房间隔缺损组(10/1 015,0.99%)与动脉导管未闭组(6/565,1.06%)尿道下裂发病率相对较低,而室间隔缺损组(65/3 275,1.98%)、法乐氏四联症组(17/770,2.21%)、大血管病变组(15/788,1.90%)和其他畸形组(21/972,2.16%)合并尿道下裂的发病率相对较高,差异有统计学意义(P均0.05)。不同先天性心脏病组合并尿道下裂的类型无显著差异(P0.05)。结论:尿道下裂是先天性心脏病男性患儿的常见合并畸形。不同类型的先天性心脏病合并尿道下裂的发病率也不同。两种疾病可能有共同的致病或易感因素。     

Adult congenital heart disease

For a decade now, it has been recognized that optimal management of adult congenital heart disease (ACHD) requires a skilled multidisciplinary team. The size and complexity of the population of adults with congenital heart disease (CHD) are increasing. This article reviews the general considerations for giving an anesthetic to an adult with CHD for cardiac or noncardiac surgery and provides further elaboration for a variety of complex patient types. Lastly, the advantages of an organized multidisciplinary approach to patients with ACHD are discussed.     

同种带瓣主动脉和肺动脉移植在复杂先心病治疗中的应用

目的　研究同种带瓣主、肺动脉移植应用于复杂性先天性心脏病矫治的疗效。方法　制备同种带瓣管道 89根 (主动脉 5 5根、肺动脉 3 4根 ) ,临床应用 3 1例 ,其中主、肺动脉单瓣作右室流出道跨瓣补片术 14例 ,Rastelli手术 11例 ,右心室 -肺动脉连接 (RV -PA) 3例 ,肺动脉瓣置换1例 ,Ross手术 1例 ,改良Fantan手术 1例。结果　主、肺动脉单瓣作右室流出道跨瓣补片组 14例 ,1例术后 3d死于右心功能不全 ,存活 13例 ,随访 ( 4 3± 3 .2 )个月 ,超声心动图检查显示移植后瓣膜启闭功能良好 ,无增厚及粘连。复杂性先天心脏病矫治组 17例 ,死亡 7例 ,存活 10例 ,随访( 3 4.3± 6.3 9)个月 ,管道血流通过顺畅 ,管壁无变薄、钙化。结论　①同种带瓣管道具有管壁弹性好 ,易于缝合 ,术后排斥反应少等优点 ,可广泛应用于复杂性先天性心脏病矫治术 ,左、右室流出道的重建 ;②同种带瓣管道补片既能加宽右室流出道 ,且有良好抗返流作用 ,有利于术后患儿心功能恢复 ,使部分复杂的病例能够一次手术根治     

Role of transesophageal echocardiography in the management of pediatric patients with congenital heart disease

Transesophageal echocardiography (TEE) has become a critical diagnostic and perioperative management tool for patients with congenital heart disease (CHD) undergoing cardiac and noncardiac surgical procedures. This review highlights the role of TEE in routine management of pediatric cardiac patient population with focus on indications, views, applications and technological advances.     

A case of congenital midline cervical cleft with congenital heart disease

A congenital midline cervical cleft (CMCC) is a rare developmental anomaly. It may represent failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck. Congenital heart disease along with CMCC is rarer, and most of the cases reported are associated with chest wall defects or thoracic ectopia cordis. The authors report a case of a 5-month-old girl with CMCC and an atrial septal defect (ASD) and discuss the clinical presentation, embryologic development, and treatment.     

Infective endocarditis with congenital heart disease

From 1988 to 2005, seven patients were operated at our hospital because of infectious endocarditis (IE) with congenital heart disease (CHD). Underlying CHD included ventricular septal defect (VSD) in 4 (2 previous operations with residual region), atrial septal defect (ASD) in 2 and bicuspid aortic stenosis (AS) in 1. No cases had preventive antibiotic prophylaxis for dental procedures. We could confirm bacteria origin from blood culture in all cases, but two patients had operations without gaining control of the infection. VSD or ASD closure and valve surgery were performed in four patients. One patient had a VSD closure, two patients had valve surgery. There were no operative or hospital deaths and there were no recurrences of IE during the study period. We successfully treated IE with CHD by enough debridement of the infective focus of IE, and valve surgery. It is important for patients with CHD to have preventive antibiotic prophylaxis for dental procedures.     

Surgical outcomes in congenital heart disease: expectations and realities

The past 50 years of congenital heart surgery has produced enormous progress. Current results surpass expectations. Yet there are important residual problems in patients growing up after heart surgery for congenital heart disease. Our system of care must evolve to care for these people throughout their lives. The evolution of congenital heart surgery has reached a point in time when we should extend care to patients in under serviced emerging countries. Development of local expertise will be required within those countries that are willing to commit resources to an organized program of caring for people with congenital heart disease. Database technology is an essential tool for ensuring and improving quality of care in every congenital heart centre. Both Registry and Academic databases have much to offer in improving care for future patients. Yet overzealous privacy laws threaten the knowledge base provided by computerized databases. We need to guide our legislators in ensuring that the valuable resource provided by database technology is not lost.     

婴幼儿危重先天性心脏病的急诊手术

目的探讨婴幼儿危重先天性心脏病急诊手术的适应证、手术技术和围术期处理。方法2000年10月至2006年10月为128例危重先天性心脏病婴幼儿施行急诊或亚急诊手术;年龄7d～36个月。平均（4．8±1．6）个月;体重2．5～12．8kg,平均（5．8±2．4）kg;术前反复呼吸道感染、心力衰竭及呼吸衰竭109例,严重低氧血症19例;根治手术123例,姑息手术5例。结果全组住院死亡12例。9例死于术后低心排血量综合征（2例为完全型大动脉转位。行大动脉调转术;2例为法洛四联症及2例室隔完整型肺动脉闭锁;1例完全性肺静脉异位引流及2例室间隔缺损合并重度肺高压）。3例室间隔缺损合并重度肺高压,均为术前反复发生肺炎、心力衰竭,术后脱离呼吸机困难。死于呼吸衰竭和肺部感染。其余患者均治愈。结论婴幼儿危重先天性心脏病急诊、亚急诊外科治疗是可行的,可以挽救大部分患者的生命,正确、适时修复心脏畸形,恢复合理的血流动力学是提高手术成功率的关键。     

几种复杂先天性心脏病手术进展

当前复杂先天性心脏病外科治疗结果不满意,这与人们对复杂先天性心脏病结构、功能的认识以及手术技术密切相关.近年来,随着对复杂先天性心脏病病理解剖和病理生理的进一步认识,手术策略和技术都有新的发展.无论是以往比较成熟的手术,还是一直存在技术难点和障碍的手术都有所突破.从手术策略和技术应用上看,依据患者自身解剖和生理特点,设计和应用个体化的手术方式,是复杂先天性心脏病手术发展的重要方向.现对完全性房室隔缺损、大主肺侧支动脉单源化、完全型大动脉转位以及Fontan类手术等具有代表性的复杂先天性心脏病手术的最新认识和技术进展作一综述.     

新生儿危重先天性心脏病心内直视手术

目的　总结连续 12例新生儿危重复杂先天性心脏病体外循环外科治疗结果和经验 ,探讨新生儿心脏手术围术期处理措施。　方法　 2 0 0 1年 5月至 2 0 0 3年 1月 ,手术治疗 12例新生儿危重复杂先天性心脏病 (手术年龄 6～30天 ,体重 2 .8～ 4 .5 kg) ,包括完全型大动脉转位 4例 ,室间隔缺损合并房间隔缺损 5例 ,完全型房室隔缺损、梗阻性心上型完全性肺静脉异位引流、心脏多发性横纹肌瘤各 1例。 12例均在体外循环下行心脏大血管畸形解剖矫治。结果　术后呼吸机支持 10小时～ 9天 ,在 ICU监护 2～ 11天 ,术后 7～ 19天出院 ;术后并发症有低心排血量、纵隔感染、呼吸窘迫综合征、渗漏综合征和急性肾功能衰竭 ;12例患者全部治愈 ,随访 6个月～ 2年 ,生长发育正常。　结论　良好的体外循环管理和恰当的围术期处理是提高手术成功率的关键 ,积极使用腹膜透析治疗术后低心排血量、急性肾功能衰竭所致的少尿、水肿是有效和安全的。     

Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications

OBJECTIVESOur goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients.METHODSFrom February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Survival, freedom from reoperation and freedom from occurrence of major adverse advents were estimated.Open in a separate windowRESULTSThere were 33 patients (21 women, 12 men). Eighteen belonged to the moderate group, 15 to the severe group. There were 12 female patients (80%) in the severe group. Patients in the moderate group were younger than those in the severe group (P = 0.02): 32 ± 12 vs 44 ± 15 years old. Thirty tricuspid valve repairs and 3 replacements were performed. Repair was mainly performed in the moderate group (P = 0.02). Overall survival was 90.1 ± 5.4% at 9 months after the operation and did not change in the later follow-up period. It was 100% for patients in the moderate group and 80.0 ± 10.3% in the severe group (P = 0.07), and 75.0 ± 12.5% for female patients of in the severe group compared to 100% for the remaining patients (P = 0.025). Survival free from major adverse events, including reoperation, at 10 years was 60.0 ± 12.6% in the moderate and 38.1% ± 12.9% in the severe group (P = 0.03). No patient in the moderate group evolved to be in the severe group at late follow-up.CONCLUSIONAdult EA patients should undergo surgery earlier when they are still in the moderate ACHD AP classification.     

Management of a cadaveric orthotopic liver transplantation in a pediatric patient with complex congenital heart disease

Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case.