双主动脉弓合并复杂心内畸形患者的同期矫治

目的探讨双主动脉弓合并心内畸形患者的临床特点和同期矫治手术方法。方法回顾性分析4例双主动脉弓合并心内畸形患者的临床资料，着重分析手术方法和手术结果。结果4例双主动脉弓合并复杂心内畸形的患者中．2例合并法洛四联症，2例合并右心室双出口。手术经正中开胸，在锁骨下动脉远端切断一侧小弓．同时切断动脉导管或动脉韧带，松解气管食管周围组织，同期行心内畸形矫治。1例术后因呕吐误吸导致病情恶化死亡，其余3例存活，未出现与双弓矫治相关的并发症。结论双主动脉弓手术治疗可取得良好效果，对于合并心内畸形的双主动脉弓患者，可经胸骨正中切口同期矫治。     

婴幼儿主动脉缩窄合并心内复杂畸形的一期手术治疗

目的:探讨婴幼儿主动脉缩窄合并心内复杂畸形一期手术的治疗方法及手术效果.方法:回顾分析2002-11至2008-09收治的婴幼儿主动脉缩窄合并心内复杂畸形一期手术治疗的临床病例.共计18例,其中男12例,女6例.年龄平均(7.7±8.8)个月,体重(6.1±2.4)kg.主动脉缩窄包括合并主动脉弓发育不良5例,单纯主动脉缩窄13例.不合并主动脉弓发育不良者,切除缩窄段,端端吻合降主动脉;合并主动脉弓发育不良者,切除缩窄段,用自体心包或自体肺动脉片加宽主动脉弓.心内复杂畸形的矫治包括完全性大动脉转位大动脉调转术8例,右心室双出口根治术6例,右心室双出口陶西平畸形大动脉调转术3例,完全性心内膜垫缺损矫治术1例.结果:所有患儿出院前超声心动图检查均显示主动脉弓降部缩窄解除,吻合口通畅,血流正常.无中枢神经系统并发症.术后早期上、下肢收缩压基本相等者4例,上肢高于下肢15～20mmHg者8例,上肢低于下肢收缩压5～10mmHg者6例.死亡3例,死亡率16.7%.1例术后当天死于低心排综合征,1例术后9天死于肺部感染,1例术后7天死于突发恶性心律失常.术后另3例出现右侧膈肌麻痹行膈肌折叠术.结论:婴幼儿主动脉缩窄合并心内复杂畸形经胸骨正中切口一期手术治疗,主动脉弓及降主动脉上段显露良好.同期矫治心内复杂畸形手术效果满意.深低温停循环区域性脑灌注可有效降低神经系统并发症的发生.     

107例矫正型大动脉转位手术治疗中期结果分析

目的:报告并分析我院107例手术治疗矫正型大动脉转位(CCTGA)合并心内畸形患者的中期随访结果.方法:1996-01至2005-12,在我院手术治疗CCTGA合并心内畸形患者107例,其中男72例,女35例;年龄1.5～50岁,平均(11.5±8.4)岁;平均体重(31.6±4.7)kg.81例左位心,23例右位心,3例中位心.手术方式包括传统双心室功能矫治75例;单心室分期矫治14例;双心室解剖矫治18例.结果:术后早期死亡5例,死亡率为4.7%.传统双心室功能矫治死亡率为4.0%;单心室分期矫治死亡率为0%;心房动脉双调转手术死亡率为0%;心房心室双调转手术死亡率40.0%.手术死亡危险因素为复杂心内畸形合并肺动脉瓣狭窄(P<0.01),与其他危险因素相比差异有统计学意义.共随访99例,随访率为92.5%.随访期间死亡12例,死亡率为12.1%;其中10例(83.3%)属于传统双心室功能矫治患者,2例(16.7%)属于单心室矫治患者.双心室解剖矫治患者没有死亡.随访期死亡危险因素为传统功能矫治手术方式(P<0.05),心房心室双调转手术(P<0.05),与其他手术方式相比差异有统计学意义.结论:CCTGA传统手术方法的中期结果是令人失望的.对于适合单心室矫治的CCTGA病例,手术可以取得较好的中期结果.心房动脉双调转手术中期的死亡率低,并发症少,其手术结果是值得肯定的.     

368例右室双出口的解剖特点与双心室矫治术

目的通过分析右室双出口(DORV)的解剖类型、合并心脏畸形及双心室矫治方法 ,指导临床诊断,探讨个体化手术方案。方法回顾368例经双心室矫治术证实的DORV,分析手术诊断和矫治方法。结果 (1)解剖类型:室缺位于主动脉下95例,肺动脉下44例,双动脉下13例,远离两大动脉98例。大动脉位置关系正常40例,左右并列关系51例,右位型主动脉101例,左位型主动脉43例。主动脉骑跨142例,肺动脉骑跨36例,双动脉骑跨2例,两大动脉完全发自右室157例。(2)合并心脏畸形共862处,其中常见畸形依次为肺动脉狭窄223例,动脉导管未闭99例,房间隔缺损93例,右位主动脉弓68例,永存左上腔静脉64例,冠状动脉畸形60例,镜面右位心24例。(3)手术方法 :心室内隧道修补术(IVR)270例,大动脉调转术(ASO)59例,Rastelli术14例,改良REV术7例,Nikaidoh术7例,双动脉根部调转术(DRT)11例,同期行Senning术6例,Mustard术1例。结论 DORV解剖类型复杂多样,可合并多种畸形,有多种矫治方法。只有早期准确诊断DORV,才能在合适的手术时机,根据不同的解剖特点,制定最佳的个体化手术方案。     

同期手术治疗动脉导管未闭合并心内畸形8例分析

目的总结动脉导管未闭合并其他心脏畸形的外科治疗经验,以提高手术效果。方法8例PDA合并心内畸形的病人,5例行经胸骨正中切口体外循环插管前动脉导管结扎术,3例行经胸骨正中切口体外循环转流后经肺动脉切口动脉导管缝闭术。结果本组无早期死亡病例,术后均恢复顺利,治愈出院,随访4个月至5年,无晚期死亡和导管再通。结论同期手术矫治动脉导管未闭合并心内畸形,可以减少手术风险和两次手术创伤的痛苦。     

冠状动脉瘘28例诊断及外科治疗

目的 总结冠状动脉瘘的诊断和手术治疗效果。方法 28例不同部位的冠状动脉瘘患者采用超声心动图和选择性冠状动脉造影,明确冠状动脉瘘发生位置,全部采用外科治疗,统计其疗效。结果 单纯冠状动脉瘘20例,合并其他心内畸形8例;右冠状动脉瘘18例,左冠状动脉瘘8例,双冠状动脉瘘2例。瘘入右心室13例,瘘入右心房12例,瘘入左心室1例,瘘入肺动脉2例。心腔内双瘘口及三个瘘口各有1例,余26例为单一瘘口。所有病例行手术治疗,8例合并其他心内畸形同期矫治。全组无死亡及残余瘘,效果满意。结论 心脏直视手术治疗冠状动脉瘘效果肯定,合并其他心内畸形应同期矫治。选择性冠状动脉造影对明确冠状动脉瘘发生位置和（或）瘘人心腔的位置非常必要。     

主动脉弓缩窄或中断合并其他心内畸形的手术配合

目的探讨主动脉缩窄(CoA)或主动脉弓中断(IAA)合并其他心内畸形的一期矫治手术的手术配合。方法2013年1月至2015年8月广东省妇幼保健院手术治疗主动脉弓缩窄或中断合并其他心内畸形患儿57例,其中主动脉缩窄42例,主动脉弓中断15例。年龄57.6 d(3 d~2岁),体质量3.8(2.1~13.0)kg。患儿均经胸骨正中切口在深低温体外循环下行一期矫治手术。术前手术室做好充分评估与充足的物品、药品、设施等准备,患儿人室后配合麻醉做好各项术前准备;手术中做好台上配合、温度管理、体位摆放、皮肤管理等;手术后再次核对检查,做好交接。结果 56例患儿顺利完成手术,脱离体外循环,治愈出院,1例死亡。结论主动脉缩窄或中断合并其他心内畸形一期矫治手术,手术室护士完善的手术配合对手术成功提供了重要保障。     

42例动脉导管未闭外科治疗

目的 回顾分析42例动脉导管未闭外科（patent duct arterious,PDA）治疗效果。方法 ①单纯PDA或同时合并心内畸形,动脉导管小～中,可采用左前或左后外侧胸切口或胸部正中切口结扎（30例单纯PDA,3例合并心内畸形）;②动脉导管粗大、钙化或同时合并其他心内畸形,胸部正中切口体外循环（cardiopulmonary　bypass,CPB）下经肺动脉切口闭合（9例）;③同期或分期矫治心内畸形。结果术中死亡1例（死于低心输出量综合征）。其他手术并发症包括小量残余分流2例、短暂声音嘶哑2例、乳糜胸2例。除2例残余分流病例外,其余病例无导管再通,心功能明显改善,痊愈出院。结论单纯结扎简便易行,适用于大多数病例：导管粗大、重度肺动脉高压、怀疑有动脉内膜炎、钙化、合并其他心内畸形病例,应采用CPB（结合深低温、低流量）下缝闭,以策安全。     

右腋下剖胸小儿心内直视术

目的 :探讨右侧剖胸小切口的手术要点及手术指征。　　方法 :本组 10 2例患者经右腋下小切口剖胸及体外循环下行小儿心内直视术 ,其中房间隔缺损 2 4例 ,室间隔缺损 6 8例 ,法乐四联症 5例 ,室间隔缺损合并房间隔缺损 3例 ,右心室双出口、Ebstein畸形各 1例。合并畸形 :左上腔静脉 4例 ,右心室双腔及动脉导管未闭各 1例。　　结果 :全组患者无死亡。　　结论 :切口的正确选择是术野良好显露的前提 ,主动脉插管是手术成功的关键。该切口适用于大部分常见先天性心脏病的矫治。     

先天性主动脉弓病变合并心内畸形新生儿期手术的临床分析

目的:探索先天性主动脉弓病变合并心内畸形新生儿期手术的治疗策略。方法:回顾性分析我院2013年1月1日至2016年12月31日,先天性主动脉弓病变合并心内畸形21例新生儿期手术的临床资料,其中男性11例,女性10例;年龄7~28d,平均天数(13±5)d;体质量2.1~5.5kg,平均体质量(3.5±1.2)kg。其中18例为一次性根治;3例为其他手术方式。结果:主动脉弓病变合并心内畸形21例新生儿中,19例成功治愈出院。手术成功率90.5%,治疗效果较显著。结论:个体化治疗方案,可以有效提高主动脉弓病变合并心内畸形的新生儿的治疗成功率。     

Surgical repair of double aortic arch: 16-year experience

Double aortic arch (DAA) is a complete form of vascular ring causing tracheoesophageal compression. We analyzed long-term results of a series of DAAs, over a period of 16 years. Between 1987 and 2003, 29 children underwent surgery for airway and/or esophageal compression secondary to a DAA. Dominant symptoms were stridor, dysphagia, choking episodes, and life-threatening apneic spells (n = 7). Diagnosis was established by barium studies, bronchoscopy, echocardiogram, angiogram, computed tomography (CT), and magnetic resonance imaging (MRI). Seven patients had concurrent cardiac anomalies. Two children had an associated tracheoesophageal fistula. Surgery was accomplished by left thoracotomy (n = 25), right thoracotomy (n = 2) or median sternotomy (n = 2). The operative mortality was zero. There was one late death due to respiratory failure. Four (13.8%) patients had a surgical complication (chylothorax, 3 cases; acute renal failure, 1 case). Follow-up (mean 7.1 years; range 6 months to 16 years) was complete in all patients, and showed complete improvement in 22 patients and partial improvement in 6 patients. Early surgical repair of DAA is associated with low mortality, and results in marked symptomatic relief in most patients. Patients with tracheomalacia or associated asthma, constitute a high-risk group and may manifest persistent symptoms and require adjunctive procedures.     

Diagnostic Role of Magnetic Resonance Imaging in Identifying Aortic Arch Anomalies

Purpose. The aim of this article was to assess the role of MRI in the diagnosis and management of a variety of complex aortic arch anomalies. Materials and Methods. Imaging was performed on a 1.5T Philips Gyroscan Intera. We retrospectively reviewed all cardiac MR scans performed from November 2003 to February 2007 at our institute to identify aortic arch anomalies. Magnetic resonance imaging included three‐dimensional (3D) sagittal volume images, cine images, short axis ventricular volumes, phase contrast flow of great vessels, and 3D gadolinium magnetic resonance angiogram. Results. Three major categories of anomalies were identified in 47 patients: double aortic arch (n = 9), right‐sided aortic arch with mirror image branching (n = 28), and aberrant right subclavian artery (n = 10). In the double aortic arch group, six patients were symptomatic, and four of them underwent a surgical division. Magnetic resonance imaging was used to plan the surgical management. In the other two groups, no patient presented with symptoms of airway or esophageal compression, and the arch abnormalities were noticed during investigation for other associated cardiac disease. However, we noticed a strong correlation with well‐defined subgroups of congenital heart disease. Right‐sided aortic arch was seen in patients with pulmonary atresia with ventricular septal defect (46.4%), tetralogy of Fallot (32.1%), and double outlet right ventricle with right atrial isomerism (14.2%). Seventy percent of the patients with aberrant right subclavian artery had aortic coarctation, and another case presented a complete aortic arch interruption. Conclusion. Magnetic resonance imaging is an important diagnostic tool in identifying anomalies of the aortic arch and its branches and can be considered the imaging technique of choice when planning surgical management, especially when there are associated cardiac anomalies.     

Deciphering the Mysteries of Crisscross Heart by Transthoracic Echocardiography

Background: Accurate diagnosis of crisscross heart and its associated anomalies is important but problematic for cardiologists. This study aimed at identifying unique transthoracic echocardiographic features and common associated lesions of this complex condition. Method: Clinical and echocardiographic features of 10 patients with crisscross anatomy were studied. Echocardiographic findings were verified by cardiac magnetic resonance imaging or surgical inspection. Results: Crisscross anatomy (10 patients, age at diagnosis ranged from 1 month to 25 years, five female) was identified in 0.076% of patients with congenital heart diseases from 1985 to 2006. All patients had cyanosis and 80% of them were underweight. Superior–inferior ventricles (SIV) and crossed ventricular inflow streams were seen in 90% and 100% of patients, respectively. All patients had abnormal ventriculo–arterial (VA) connections: five with transposition of great artery (L‐type: n = 2; D‐type: n = 3) and five with double outlet right ventricle. Commonly associated anomalies included ventricular septal defects (100%), right ventricular outflow tract obstruction (60%), atrioventricular valves straddling or overriding (50%), atrial septal defect (40%), and right ventricular hypoplasia (30%). Seven patients received cardiac surgery for the relief of cyanosis. Conclusions: SIV and crossed inflow streams are important diagnostic features for crisscross heart by transthoracic echocardiogram. The hemodynamic consequences of abnormal VA connections and associated defects impact surgical management. (Echocardiography 2011;28:104‐108)     

Significance of Doppler echocardiography in the diagnosis and management of children with heart disease

OBJECTIVE: To assess the accuracy of Doppler-echocardiography in the diagnosis and management of the child with heart disease. MATERIAL AND METHODS: Prospective echocardiographic study of 132 consecutive patients aged 1 day to 16 years old with heart disease confirmed by cardiac catheterization (n = 109) or anatomic examination (surgical = 21, necropsy = 2). RESULTS: Of the 258 cardiovascular anomalies diagnosed by cardiac catheterization and anatomical examination, 247 were correctly identified by echocardiography (sensitivity = 96%). There were 11 missed and 4 false positive diagnosis (specificity = 98%), all of them were vascular anomalies. Intracardiac anatomy and segmentary alignment were always correctly identified. The 21 cardiovascular anomalies operated without cardiac catheterization were correctly diagnosed. In 16 patients (12%) a management plan could not be established only on clinical echocardiographic grounds and, in another patient, the plan was incomplete. In the remaining 115 patients (87%) the management plan was correct and complete. CONCLUSIONS: The clinical-echocardiographic study allows: 1) an excellent anatomo-functional evaluation of the heart and great vessels, 2) surgical repair of some selected cardiovascular anomalies without previous cardiac catheterization.     

Atriale Isomerie und viszerale Heterotaxie

Atrial isomerism and visceral heterotaxy describe complex pathoanatomic findings with defects in the determination of lateralization. Differentiation of right and left atrial isomerism was based on the anatomy of the atrial appendages and bronchial tree and often associated with asplenia in right atrial isomerism and polysplenia in left atrial isomerism. In these pa tients complex cardiac anomalies determine clinical symptoms and diagnostic procedures.At primary diagnosis in the neonatal period, systemic venous and pulmonary venous connections and intracardiac anatomy are identified using the sequential segmental approach. Therapeutic decisions and palliative procedures can usually be based on these initial echocardiographic findings. As many of the patients were found to have a functionally univentricular heart, surgical procedures following the Fontan principle (lateral tunnel or extracardiac conduit procedure) were introduced in patients with heterotaxy syndrome and successfully performed. Early survival and long-term outcome vary depending on associated cardiac and systemic and pulmonary venous anomalies.The therapeutic options and mid-term results in patients with heterotaxy syndrome undergoing the Fontan procedure are reported.     

Electrocardiogram in double chamber right ventricle

Thirty patients with a double chamber right ventricle were seen during a 7 year period. The majority of patients had associated cardiac anomalies, most commonly a ventricular septal defect. In this series, as in others, the history, clinical examination, chest roentgenogram and echocardiogram were inconclusive for diagnosing double chamber right ventricle. Symptoms were generally related to the severity of the associated anomalies. In 40 percent of the patients reviewed, upright T waves were found in lead V3R as the only electrocardiographic finding suggestive of right ventricular hypertrophy. Because upright T waves in lead V3R in the absence of other evidence of right ventricular hypertrophy are not commonly seen in patients with an isolated ventricular septal defect or a defect associated with other forms of right ventricular outflow obstruction, this finding should alert the cardiologist to the possibility of the presence of double chamber right ventricle prior to cardiac catheterization.     

先天性双主动脉弓畸形的外科诊治

目的 总结先天性双主动脉弓畸形患儿的临床特点和手术治疗经验。方法 回顾性分析2013年3月-2015年8月,共5例双主动脉弓患儿临床资料。5例患儿均为男性。年龄7 -18月,均数(11.60±4.21)月;体质量8-9.5Kg,均数(8.50±0.50)Kg。左右弓均衡型1例,右弓优势型4例。临床表现为气喘、喉鸣、吞咽困难。5例患儿手术经左后外侧切口进胸,切断左弓,同时切断动脉导管或动脉韧带,松解气管、食管周围组织,解除对气管、食管的压迫。 结果 5例患儿均顺利完成手术,未出现与手术相关并发症,无围术期死亡病例。呼吸机使用时间2-4h,均数(3.30±0.83)h,住院时间9-20天,均数(12.40±4.39)天。5例患儿均得到随访,1例患儿术后半年行肺动脉闭锁、室间隔缺损、房间隔缺损根治手术,术后痊愈出院。1例患儿术后半年行双侧隐睾下降固定术。其余患儿临床症状均缓解,生长发育、活动量较正常儿童无明显差异。结论 DAA是一种少见的心血管畸形,易引起气喘、喉鸣、吞咽困难等症状,心脏大血管CT检查可确诊,早发现、早期手术治疗效果良好。     

Repair of total anomalous pulmonary venous connection in early infancy

From May 1995 through October 2001, 19 infants less than 90 days old underwent surgical correction of total anomalous pulmonary venous connection. In 15 babies with isolated total anomalous pulmonary venous connection, there was one operative death. In 4 with complex anomalies, there were 2 operative deaths. The vertical vein was not ligated in 6 cases for various reasons. Two patients died during reoperation for early pulmonary venous obstruction. In the late follow-up, 2 babies required reoperation for late anastomotic stricture; one needed additional balloon dilatation. Of the 14 surviving patients, one had a small residual gradient and infrequent supraventricular tachycardia, the others were asymptomatic and without gradients. Surgical correction of total anomalous pulmonary venous connection can be carried out in early infancy with low mortality and morbidity. However, associated complex cardiac anomalies and small caliber pulmonary arteries and veins carry higher risks. Recurrent pulmonary venous obstruction and diffuse pulmonary vein stenosis are causes of early reoperation and poor surgical outcome.     

Determinants of outcome in hospitalized infants with congenital heart disease

To identify determinants of adverse outcome in this era of early, definitive treatment, retrospective data were analyzed for 1988 on infants aged less than 1 year with congenital cardiac disease hospitalized at The Johns Hopkins Hospital. In this cohort of 108 infants, 34% (37 of 108) had complex cardiac disease, 33% (36 of 108) had major extracardiac anomalies, 88 patients (81%) underwent 116 surgical procedures, 37% (40 of 108) were hospitalized for greater than 28 days and 29% (31 of 108) died during the first year. Univariate analysis showed that complex disease (i.e., severe ventricular hypoplasia, ventricular septal malalignment or outflow atresia), extracardiac anomalies, early initial presentation, and prolonged stay in the intensive care unit were significantly associated with infant death, whereas surgery was associated with a significantly increased rate of survival. The findings for complex disease and surgery persisted in multiple logistic regression analysis. It is concluded that outcome in most infants with congenital cardiac defects is now extremely favorable, and that major research and preventive efforts should focus on complex congenital cardiac defects.