完全性肺静脉异位连接的外科治疗

目的:探讨完全性肺静脉异位连接(total anomalous pulmonary venous connection,TAPVC)的手术方法及疗效。方法:选取5例TAPVC患者,其中1例心上型患者行左心房共同静脉吻合;1例心内型患者将房间隔缺损扩大,应用补片将异位引流的肺静脉隔至左心房;3例混合型患者中有2例垂直静脉与左心耳吻合,补片将异位引流的肺静脉共同腔隔至左心房,1例扩大房间隔缺损后行心包片补片将异位引流的肺静脉隔至左心房。结果:所有患者均痊愈并获得随访,随访时间8个月～2年,心功能基本正常。结论:TAPVC患者一旦诊断明确应及早手术,正确认识肺静脉复杂的解剖,选择适当的手术方法可获良好的治疗效果。手术治疗的关键在于肺静脉流入左心房足够宽畅,避免狭窄。     

完全性肺静脉异位引流的外科治疗

目的总结完全性肺静脉异位引流(TAPVD)的外科治疗经验和体会.方法12例患者采用全身麻醉中度低温体外循环下手术,10例心上型横切右心房和左心房后壁,行肺总静脉与左心房后壁吻合,2例心内型切开ASD与冠状静脉窦口之间的残余房间隔壁,自体心包补片将肺静脉开口与冠状静脉窦隔入左心房.结果患者均手术治愈出院,随访6个月至3年,恢复良好,心功能明显提高.结论完全性肺静脉异位引流手术治疗的关键在于术前正确诊断,术中吻合口应足够大,避免狭窄.     

完全性肺静脉异位引流矫治术后肺静脉梗阻的治疗探讨

目的:总结完全性肺静脉异位引流(TAPVC)矫治术后肺静脉梗阻的治疗经验。方法:回顾性分析2011-01至2015-12于青岛市妇女儿童医院心脏中心施行TAPVC矫治术后发生肺静脉梗阻16例患儿的临床资料,其中男性10例。所有患儿均出院前、术后1个月、3个月、6个月、12个月、24个月回院复诊,包括经胸心脏超声多普勒、心电图、X线胸部正侧位片。以经胸心脏超声多普勒检查肺静脉流速2 m/s为梗阻标准,诊断肺静脉梗阻。结合患儿临床表现(反复心力衰竭、生长发育迟缓)确定二次手术时机。二次手术采用sutureless缝合技术和常规补片扩大技术。结果:术后发生肺静脉狭窄16例患儿无失访,其中7例吻合口周围狭窄(混合型1例,心下型3例,心上型2例,心内型1例);7例单支肺静脉狭窄,2例双支肺静脉狭窄,无三支及以上肺静脉狭窄。按术前Darling类型分:混合型2例,心下型5例,心上型5例,心内型4例。11例(68.8%)术后肺静脉狭窄流速增快发生在术后3~6个月。5例二次手术患儿中,4例采用sutureless缝合技术,1例采用常规补片扩大技术,均为吻合口周围梗阻狭窄,2例死亡,3例患儿随访中。结论:TAPVC矫治术后吻合口周围狭窄为主要二次手术适应证,并且需早期即行手术。     

完全性肺静脉异位引流的外科治疗

目的总结完全性肺静脉异位引流（total anomalous pulmonary venous drainage，TAPVD）的外科治疗经验。方法TAPVD27例（心上型15例，心内型12例）均在体外循环下手术。心上型6例采用传统双心房联合切口，9例采用心上法，后者经上腔静脉与升主动脉之间显露左心房与共同静脉干行-一侧吻合：心内型切开房间隔缺损与冠状静脉开口之间的残余房间隔壁，自体心包将肺静脉开口与冠状静脉窦隔入左心房。结果早期死亡2例，其他病人痊愈出院，术后随访3个月-4年，恢复良好，无吻合口狭窄发生，出院后无因心、肺衰竭死亡病人。结论TAPVD早期诊断极为重要，手术治疗的关键在于左心房与肺总静脉吻合口应足够大，避免狭窄的发生。     

67例新生儿完全性肺静脉异位引流的外科治疗及早中期结果

目的:回顾总结67例新生儿完全性肺静脉异位引流的手术治疗经验。方法:自2011年1月至2018年1月,本中心共完成新生儿完全性肺静脉异位引流矫治手术67例。其中男性46例,女性21例;手术中位年龄12 d(1～28 d);手术平均体质量(3.2±0.5)kg(2.1～4.5 kg)。Darling分型:心上型30例,心内型7例,心下型29例,混合型1例。合并卵圆孔未闭18例,合并继发孔房间隔缺损49例,合并动脉导管未闭39例,术前肺静脉梗阻50例(74.6%)。全部患儿均在全麻中浅低温体外循环下手术。结果:全组术后早期死亡6例(9.0%)。随访59例,中位随访时间45个月(6个月～7年)。术后肺静脉梗阻5例(8.5%)。4例吻合口狭窄行吻合口扩大术,术后恢复良好。1例肺静脉开口及分支狭窄采用sutureless技术,术后死于低心排。余患儿发育良好。结论:完全性肺静脉异位引流自然预后差,应尽早手术。新生儿完全性肺静脉异位引流的手术治疗早期结果满意,术后肺静脉梗阻需要长期随访。     

完全性肺静脉异位引流——17年的外科经验

完全性肺静脉异位引流(TAPVD)的治疗是心脏外科的一个难题。近年来,随着外科技术的改进,已对新生儿和小婴儿施行纠治手术,并取得了满意的疗效。本文报道英国利物浦皇家儿童医院对80例 TAPVD 患儿的治疗经验,并分析了造成术后早期和晚期死亡、术后肺静脉阻塞以及需要再次手术的危险因素。     

先天性肺静脉异位引流的MSCT诊断

目的分析MSCT对先天性肺静脉异位引流中的诊断价值。方法回顾性分析我院经CTA检查的肺静脉异位引流患儿26例影像学资料。结果 MSCT正确诊断25例肺静脉异位引流,完全性肺静脉异位引流19例,其中心上型10例,心内型3例,心下型5例,混合型1例;部分性肺静脉异位引流7例。结论 MSCT能准确诊断出肺静脉异位及其分型,对临床手术方式的选择提供帮助。     

31例完全性肺静脉异位连接的外科治疗

目的探讨31例完全性肺静脉异位连接的手术方法及术后疗效。方法31例患者（心上型16例、心内型13例、混合型2例）均在体外循环下行矫治术。心上型6例采用传统双心房联合切口，10例采用心上法，后者经上腔静脉与升主动脉之间显露左心房与共同肺静脉干行侧一侧吻合；心内型切开房间隔缺损与冠状静脉窦开口之间的残余房间隔壁，自体心包将肺静脉开口与冠状静脉窦口隔入左房；混合型则根据不同的病理解剖特点行相应的矫治术。结果早期死亡2例，其他患者都痊愈出院。术后随访3个月至14年，恢复良好，无吻合口狭窄发生，心功能均为Ⅰ级，无因心肺功能衰竭死亡患者。结论完全性肺静脉异位连接早期诊断和及早行矫治术极为重要，手术治疗的关键在于左心房与肺总静脉吻合口应足够大，避免狭窄的发生。     

完全性肺静脉异位引流的外科治疗

目的:总结完全性肺静脉异位连接(TAPVC)患者的外科治疗经验,以提高手术疗效。方法:20例患者均在中度低温体外循环下行TAPVC矫治术。心上型12例,心内型7例,混合型1例。合并动脉导管未闭2例,肺动脉瓣狭窄及三房心各1例,三尖瓣关闭不全7例。结果:术后早期(30 d)死亡1例,死于术后严重低心排出量综合征,生存19例均获得随访,随访时间1～5年,心功能恢复良好;术后2周超声示右心房室明显缩小,左心房室扩大。结论:完全性肺静脉畸形引流一经确诊应尽早手术,手术的关键是保证吻合口够大和避免吻合口狭窄及心律失常。心上法可为肺静脉主干与左心房顶部吻合提供良好显露并可降低心律失常发生率,是心上型TAPVC左心房与肺静脉总干吻合良好途径。     

完全性肺静脉异位引流的外科治疗

１９８０年至１９９２年期间，手术矫治完全性肺静脉异位引流５例（男２例，女３例），年龄５～１３岁。其中心上型４例（Ⅰａ型２例、Ⅰｂ型２例），心内型１例。术后全部成活。本文重点讨论右侧径路进行左房和肺静脉总干吻合法和Ⅱｂ型的手术方法。     

Diagnostic echocardiographic features of the sinus venosus defect.

To establish the diagnostic criteria for a sinus venosus atrial septal defect cross sectional echocardiograms, cineangiograms, and surgical notes of all patients with this diagnosis seen at the Children's Hospital of Pittsburgh between 1986 and 1988 were reviewed. Seven patients were identified. In each the extent of the atrial septum and the nature of the junction of the superior vena cava with the atria were evaluated echocardiographically from the subcostal position. All had overriding of the superior vena cava and abnormally connected right pulmonary veins. Six patients had undergone cardiac catheterisation and cineangiography. Five patients underwent surgical repair. The operative findings were consistent with the expected morphology in all five, and these features were additionally confirmed in a specimen from the cardiopathological museum. Therefore, the basic anatomical feature of a superior sinus venosus interatrial communication is a biatrial connection of the superior vena cava. This, together with anomalous drainage of the right sided pulmonary veins, results in an interatrial communication outside the confines of the true atrial septum. Overriding of the superior vena cava across the upper rim of the oval fossa is suggested as the pathognomonic diagnostic feature that can clearly be demonstrated echocardiographically from the subcostal position. In essence the lesion is an interatrial communication rather than an atrial septal defect.     

Diagnostic echocardiographic features of the sinus venosus defect

To establish the diagnostic criteria for a sinus venosus atrial septal defect cross sectional echocardiograms, cineangiograms, and surgical notes of all patients with this diagnosis seen at the Children's Hospital of Pittsburgh between 1986 and 1988 were reviewed. Seven patients were identified. In each the extent of the atrial septum and the nature of the junction of the superior vena cava with the atria were evaluated echocardiographically from the subcostal position. All had overriding of the superior vena cava and abnormally connected right pulmonary veins. Six patients had undergone cardiac catheterisation and cineangiography. Five patients underwent surgical repair. The operative findings were consistent with the expected morphology in all five, and these features were additionally confirmed in a specimen from the cardiopathological museum. Therefore, the basic anatomical feature of a superior sinus venosus interatrial communication is a biatrial connection of the superior vena cava. This, together with anomalous drainage of the right sided pulmonary veins, results in an interatrial communication outside the confines of the true atrial septum. Overriding of the superior vena cava across the upper rim of the oval fossa is suggested as the pathognomonic diagnostic feature that can clearly be demonstrated echocardiographically from the subcostal position. In essence the lesion is an interatrial communication rather than an atrial septal defect.     

Surgery in children with atrial septal defects without cardiac catheterization

We describe the results of surgical repair of atrial septal defects in 36 children who did not undergo pre-op cardiac catheterization. These cases were seen at the Hospital de Cardiologia y Neumología Dr. Luis Méndez del Centro Médico Nacional. There were 24 (67%) females and twelve (33%) males. The mean age was 6.4 +/- 2.4 years with a range from three to thirteen. All cases had auscultatory findings typical of atrial septal defect. Five patients with associated tricuspid murmur (chest film showed grade I cardiomegaly in 21 (58.3 per cent), grade II cardiomegaly in fifteen (41.7 percent). Pulmonary artery shadow was normal in 24 (66.6 percent) and increased in twelve (33.3%). Pulmonary blood flow was increased in all of them. Electrocardiogram showed sinus rhythm in 35 (97.2%). In one instance left atrial rhythm; all EKGs demonstrated right axis deviation, complete right bundle branch block and right ventricular hypertrophy with diastolic overload. Only three had right atrial hypertrophy. The M-mode echocardiogram showed right ventricular dilatation in all and paradoxically septal motion in 26 (72.2%). Two-dimensional echo with the subxiphoid view allowed direct visualization of the defect in all cases. We performed contrast echocardiogram in eight cases and Doppler echocardiogram in six of them. Cardiac surgery findings were ostium secundum atrial septal defect in 34 (94.4%). Two of them also had partial anomalous venous connection. All had uneventful recovery. We conclude that in typical atrial septal defects operative repair is feasible without prior cardiac catheterization.     

Total anomalous pulmonary venous drainage: is a surgical correction possible on the sole basis of echocardiography?

Between April '83 and August '89, 33 patients with total anomalous pulmonary venous drainage were studied at the department of Cardiology in Bergamo. There were 18 males and 15 females, aged between three days and 8 months (average: two months). In this study only cases of isolated total anomalous pulmonary venous drainage were taken into consideration. The patients underwent echocardiographic examination with ATL MK 600, Vingmed 700, ATL Ultramark 9 with 3.5; 5; 7.5; MHz transducers; in the last three years the echocardiographic examination was integrated by continuous and pulsed wave Doppler and, in the last year, by color Doppler. The morphologic diagnosis was routinely established by means of the sequential approach method. A common feature in all types of total anomalous pulmonary venous drainage was the impossibility of defining the connections of the pulmonary veins with the left atrium. Furthermore, patients had a volume overload of the right heart, and atrial septal defects of various sizes. The site of anomalous drainage of the pulmonary veins was assessed by means of multiple cuts from subcostal, precordial and suprasternal windows. The echocardiographic diagnosis was exact and complete in 29 cases (87.9%), and in four cases it was incomplete but basically correct (12.1%). The anatomical findings were confirmed during cardiac surgery in 32 cases and by autopsy in one case of supracardiac total anomalous pulmonary venous drainage in a critically ill patient, who died before surgery. Of the 32 patients who underwent surgical correction, 20 (62.5%) had only an echocardiographic diagnosis, which resulted correct in all cases. The majority of patients with isolated TAPVD can be confidently diagnosed by means of echocardiography, thus, avoiding preoperative catheterization.     

静脉窦房间隔缺损的外科治疗

目的:总结我科1999年7月至2004年4月26例静脉窦房间隔缺损手术病例的临床资料,探讨静脉窦房间隔缺损的外科手术方式和经验。方法:采用浅低温体外循环心脏不停跳技术行静脉窦房间隔缺损及合并畸形的手术治疗。其中男性11例,女性15例,年龄6个月～27岁,平均(12.1±5.7)岁。合并部分肺静脉异位引流19例,其中引流入上腔静脉8例,引流入右心房11例;合并动脉导管未闭1例、左上腔静脉引流入冠状静脉窦2例。合并室间隔缺损1例,三尖瓣返流3例,二尖瓣重度返流1例。体外循环转流时间25～98min,平均(56.4±6.2)min,全组均用自体心包补片修补,术中8例用右心房外侧切口避开窦房结延长至上腔静脉,3例用另外心包加宽上腔静脉与右心房结合部;5例术中扩大房间隔缺损利于异位引流的肺静脉回流,1例同期行二尖瓣置换。结果:术后平均重症监护时间(4.2±2.1)d,平均住院时间(15±6.5)d。全组无死亡病例。1例术后早期窦房结功能失调,经使用临时起搏器辅助3d后恢复,术后1例转为心房颤动心律,余均为窦性心律。1例出现上腔静脉梗阻,二次手术加宽上腔静脉后好转。结论:运用经食道超声心动图较易诊断静脉窦房间隔缺损和确定有无肺静脉异位引流,外科手术治疗静脉窦房间隔缺损的效果满意,选择适当的手术方式可以降低上腔静脉梗阻、窦房结功能失调等并发症。     

Echocardiographic evaluation of left ventricular function, mass and wall stress in children with isolated ventricular septal defect

M-Mode echocardiography was performed in 22 normal children and 22 children with ventricular septal defects. Left ventricular and left atrial chamber dimensions and wall thicknesses were measured in all patients. Utilizing these data, indices of left ventricular function were derived: shortening fraction, velocity of fiber shortening, peak diastolic fiber lengthening, end-systolic wall stress, radius thickness ratio, and ventricular mass. The results showed that ventricular septal defect was associated with enlarged left ventricular and atrial dimensions and increased shortening fraction, but that velocity of shortening and early diastolic lengthening remained normal. Left ventricular mass was increased, thus maintaining normal wall stress and radius/thickness ratio. Cardiac failure complicating ventricular septal defect was associated with enlarged left ventricular and atrial dimensions (indexed for weight). Ventricular mass, wall stress and function, however, were similar in subjects with ventricular septal defect, with or without cardiac failure. Since left ventricular mass was adequate to maintain wall stress and function in subjects with heart failure, other factors were presumably responsible for heart failure complicating ventricular septal defect.     

阵发性心房颤动患者心脏大静脉肌袖电活动特征的临床研究

总结和探讨阵发性心房颤动(简称房颤)患者心内电生理标测中肺静脉和上腔静脉内肌袖电活动的特征,及其与房颤的关系。选择无器质性心脏病的顽固性阵发性房颤患者 126例,用环状标测电极行肺静脉和 /或上腔静脉电生理标测,分析引起房性心律失常时肌袖内的电活动类型、频率、发作方式及其与房颤之间的联系。结果:有50例(39. 7% )经心电生理标测证实其房性心律失常与大静脉肌袖的自发电活动相关。共标测心脏大静脉 354根,心律失常相关大静脉 59根,包括肺静脉 49根、上腔静脉 10根。电活动类型呈现单一或连发电活动 38根,呈短阵性快速电活动 8根,呈连续快速( >300次 /分)电活动 27根,呈连续较慢电活动 ( <100次 /分 ) 2根。房颤的发生均与静脉肌袖内连续快速的、规律或紊乱的电活动对心房的引发有关。结论:根据房性心律失常发作时的大静脉肌袖内电位的特征以及与心房电位的关系,可以明确判定房颤的相关肌袖。同一根大静脉肌袖可表现为 2种或 2种以上的电活动类型,其中以单一和连续快速电活动多见,房颤均为静脉肌袖内的连续快速电活动所引发,而非单一房性早搏诱发。     

Echography in aneurysm of the interatrial septum

Atrial septal aneurysm can be detected by subcostal echocardiography as a bulge of the intermediate interatrial septum, ballooning toward the right atrium. We retrospectively revised 5412 echo examinations, consecutively performed in our laboratory, and we found 14 cases of atrial septal aneurysm (0.26%), mean age 36 +/- 15 years, 9 males and 5 females. In 7 patients atrial septal aneurysm was wide, including the whole atrial septum; in 5, only cranial two-thirds of the septum were involved and in 2, it regarded only the intermediate septum. No patients referred to arrhythmias, syncope, embolism, endocarditis or transient ischemic neurologic disorders. Cardiac abnormalities or defects were associated to atrial septal aneurysm in 12/14 patients: they consisted of atrial septal defect, mitral valve prolapse, false ventricular tendons or persistent Chiari network. Atrial left-to-right shunt was detected in all 6 cases with atrial communication. Considering each single associated cardiac abnormality, the prevalence of atrial septal aneurysm was 7% in patients with atrial septal defect, 1.7% in those with mitral valve prolapse, 6.6% in persistent Chiari network and 0.9% in false ventricular tendons. In conclusion, echocardiography is the first-choice technique to detect atrial septal aneurysm and other related cardiac defects.