The study was designed to analyze the results of central shunt procedure using Gore‐Tex grafts for treatment of patients with complex congenital heart diseases.

Objective: Ebstein's anomaly with isolated anterior leaflet downward displacement is rare and has not been reported in the literature to our knowledge. In this article, our experience of the surgical treatment in 6 cases with this anomaly is reported.Methods: From November 2005 to November 2013, 6 patients (3 male, 3 female, aged 2-39 years) with Ebstein's anomaly and isolated anterior leaflet downward displacement received anatomic repair at the First Hospital of Tsinghua University. The diagnosis was made by echocardiography and confirmed at operation. Surgery was performed under hypothermic cardiopulmonary bypass. Surgical technique included excision of a huge atrialized portion of the right ventricle located in the anterior wall of the heart; reconstruction of the right ventricle by repairing the "V''-shaped defect left by the excision procedure; detachment, repair, and reimplantation of the anterior leaflet; and reconstitution of the right atrioventricular connection. Intraoperative transesophageal echocardiography was used to evaluate the position, morphology, structure, and function of the tricuspid valve, as well as right ventricular function.Results: Five patients were discharged uneventfully, and 1 patient died of postoperative pneumonia. At follow-up from 2 months to 7 years, no notable tricuspid valve regurgitation or stenosis was found and all patients were maintaining a normal lifestyle. The 27-year-old female patient gave birth to a normal infant uneventfully 3 years after surgery.Conclusions: Ebstein's anomaly with isolated anterior leaflet downward displacement is a complex and severe abnormality, and has several unique anatomic and clinical features and specific surgical requirements. Preoperative diagnosis can be made by clinical investigation and echocardiography. Excellent results can be achieved by anatomic correction.

>先天性心脏病（先心病）是婴幼儿重症肺炎的高危因素之一,先心病合并重症肺炎患儿的病死率较高 [1-2] 。先天性心脏畸形婴幼儿因心内异常分流易导致反复的呼吸道感染,心肺功能均有不同程度的损害,一旦发生肺部感染,因患儿体质差或合并心功能不全,感染通常不易控制,易反复,且病情重,变化快 [3] 。因此,复杂重症先心病合并肺部感染的治

病历摘要 患儿男,2岁3个月,因发现心脏杂音2个月,于2011年7月29日收住入清华大学第一附属医院.患者人院前2个月,因感冒就诊于当地医院,查体发现心脏杂音.随后患儿入我院治疗.患儿发育较正常小儿稍差,身高82 cm,体质量10 kg.四肢末梢经皮血氧饱和度:98％～100％.心脏听诊:胸骨右缘第2肋间可闻收缩期Ⅱ～Ⅲ/6级杂音,胸骨左缘第4～5肋间可闻及舒张期中度杂音.血压:上肢100/52 mm Hg(1 mm Hg =0.133 kPa),下肢54/39 mm Hg.经胸心脏彩超提示:主动脉瓣狭窄,压差69 mm Hg,瓣环内径10 mm,先天性主动脉瓣二瓣化畸形;二尖瓣瓣上隔膜,二尖瓣狭窄,瓣叶开放受限,压差15 mm Hg;主动脉弓部发出左锁骨下动脉以远局部狭窄,压差69 mm Hg;动脉导管未闭,直径6 mm;左室舒张末期内径34mm,射血分数65％.

Objective To investigate the effects of radiofrequency catheter ablation (RFCA) under guidance of three dimensional electroanatomic mapping system (Carto) combined with appendectomy to cure atrial tachycardia(AT) originating from atrial appendages in children.And to evaluate the electrocardiographic characteristics.Methods Of the 17 children with AT receiving RFCA under Carto system in our electrophysiology laboratory(EPL),5 were diagnosed as AT originating from atrial appendages,age 3.2 ～ 12.8 (8.1 ±4.6)years.Clinical data and electrocardiographic characteristics of these 5 children were retrospectively analyzed.Ablations were performed by cold saline infused catheter at appendages targeting loci of AT origin under guidance of Carto system.For those recurred after RFCA,appendectomies were performed.Results Of the 17 children with AT receiving RFCA under Carto system,5 (29％) originated from atrial appendages.Three were from right atrial appendage (RAA) and 2 were from left atrial appendage(LAA).Three dimensional mapping portraits revealed increased focal automaticity.Immediate success rate for RFCA was 100％.Three recurred (60％) during follow-up period (2 ～ 15months).Appendectomies were performed in them individually at 1 week,1 month and 2.5 months after RFCA.During operations,heart rhythm immediately reversed to sinus while appendages were clenched.No recurrence was detected during 5 ～ 14 months follow-up.Characteristics of P wave configuration for AT from RAA were:①positive P wave in Ⅰ and aVL leads;②positive P wave in Ⅱ,Ⅲand aVF leads ;③negative and double-peak P wave in V1 lead.Characteristics of P wave configuration for AT from LAA were:①negative P wave in Ⅰ and aVL leads;② positive P wave in Ⅱ,Ⅲ and aVF leads;③positive P wave in V1 lead (1 case)or bidirectional P wave with positive tendency (1 case).Conclusions ① The electrocardiographic characteristics of AT originating from atrial appendages in children are peculiar; ② RFCA under Carto mapping system by cooled tip cather is safe and effective for AT originating from atrial appendages in children while recurrence rate is high; ③ Appendectomy for AT originating from atrial appendage is reliable,while the accurate location of AT origin should be determined by Carto mapping system preoperatively.

Objective To prospectively evaluate the mid-term results of mitral annuloplasty using autologous pericardium for mitral valve insufficiency.Methods From April 2004 to December 2011,48 patients underwent mitral annuloplasty using autologous pericardium,the average length of pericardial strips was (51.9 ±2.8)cm.Carpentier classification was class Ⅰ in 5 cases,class Ⅱ in 41 cases,and class Ⅲ in 2 cases.The mitral valve repair techniques included quadrangular resection in 30 cases,valve repair in 7 cases,chordae transposition in 4 cases,"edge to edge" technique in 2cases,and artificial chordae tendineae in 3 cases.Concomitant procedures included one case arotic valve replacement,11 cases tricuspid valve repair,9 cases coronary artery bypass grafting,1 case coronary artery muscle bridge resection,and 1 case permanent pacemaker implant.Datum on long-term outcomes were obtained by questionnaires and by phone interview [average follow-up time (62.2 ± 21.3) months].Results Compared with preoperative datum,the diameters of left ventricular end diastolic diameter (LVDD) and left atrial diameter (LAD) examined by echocardiography show significant reduction in postoperative [(58.6 ± 1.7) mm vs (45.1 ± 1.3) ram,t =12.85,P ＜0.01 ; (50.6 ± 1.6)mm vs (38.0 ± 1.4)ram,t =9.58,P ＜0.01].There was early postoperative death in one case,cerebral infarction in one case,but none of patients died in late postoperative period.One patient had moderate mitral valve regurgitation in long-term follow-up.None of patients had redo operation and hemolytic complications.Conclusions Mitral annuloplasty using an autologous pericardium was an acceptable technique with low anticoagulation complications,permanent,well left ventricular function maintenance,and an economic method.

OBJECTIVE:To investigate the feasibility, advantages and efficacy of implantation of left atrial and ventricular epicardial dual chamber pacemaker to treat pediatric complete atrioventricular block.METHOD:Eleven children with median age 4.0 years (0.5-7.6 years) diagnosed as complete atrioventricular block resisting to drug therapy received implantations of left atrial and ventricular epicardial dual chamber pacemakers. Six were male and five female. Temporal or permanent right ventricular pacing was used for all of them before implantation of left atrial and ventricular epicardial dual chamber pacemakers. Three cases showed cardiac dysfunction. Left lateral thoracotomy was performed at 4th intercoastal space along anterior axillary line under general anesthesia, the pericardium was incised vertically anterior to the phrenic nerve, two pacing leads were individually located at left atrial appendage and left ventricular lateral wall. After all the parameters were detected to be satisfactory, a pouch was made at left abdomen under coastal margin. Dual chamber pacemaker was connected with pacing leads through subcutaneous tunnels. The sizes of heart chambers, cardiac functions, parameters of pacemaker, sensitivity, pacing status, PR interval and QRS interval were closely followed up post-operatively.RESULT:Implantations of pacemakers were successful for all of the patients with no complications associated with operations. Preoperative electrocardiograms showed QRS interval (180 ± 33)ms under right ventricular pacing, it decreased to (140 ± 24)ms after implantation of left atrial and ventricular epicardial dual chamber pacemaker, significantly lower than right ventricular pacing (t = 8.8, P < 0.05) . Atrioventricular (AV) interval was set at 90 ms, PR interval (124 ± 4)ms. Echocardiograms performed within 2-3 days after implantation of left atrial and ventricular epicardial dual chamber pacemakers showed that for the 3 cases who were previously under right atrial and right ventricular dual chamber pacing presenting cardiac dysfunction, their left ventricular diastolic diameter (LVDd) decreased from (46.3 ± 12.5) (32.0-55.0) ms to (44.7 ± 12.0) (31.0-53.0) mm and left ventricular ejection fraction (LVEF) increased from 30% ± 15% (18%-47%) to 44% ± 18% (33%-65%). During 2-14 months' follow up, LVEF increased progressively which became significantly higher than before (65% ± 8% vs. 30% ± 15%, t = 5.6, P < 0.05) . Cardiac chamber sizes and left ventricular systolic function for the other 8 patients maintain normal during follow up. Pacing status and sensitivity were satisfactory for all these patients during follow up.CONCLUSION:Implantation of left atrial and ventricular epicardial pacemaker might be considered for children diagnosed as complete atrioventricular block for whom endocardial pacemaker could not be implanted, due to its utmost protection for cardiac function with minimal injury and its ability to prevent or reverse pacemaker syndrome. Left atrial and left ventricular epicardium should be regarded as the first-choice and routine locations for epicardial pacing.

Objectives To evaluate the electrocardiographic characteristics of atrial tachycardia (AT) originating from atrial appendages in children and to investigate the effects of radiofrequency catheter ablation (RFCA) under guidance of CARTO system and appendectomy. Methods Of the 17 children with incessant AT receiving RFCA under CARTO system in our electro cardiac lab, five were diagnosed as AT originating from atrial appendages, age 8.1±4.6 (3.2∼12.8) years. Male:Female 1:4. AT appeared to be incessant in all of these five children, resisting to diverse antiarrhythmic drugs or only minimal effects were gained. Decreased cardiac systolic function were detected in four children (80%) whose left ventricular ejection fraction (LVEF) were 44%∼50%. Electroanatomical mapping under CARTO system revealed atrial appendage origin for these five children, RFCA by cooled tip catheter (Navi-Star THERMO COOL) were performed at the location of earliest AT origin. We investigated the P′ wave configurations in electrocardiogram of this AT type and effects of RFCA. Appendectomy was used for patients with AT recurrence after RFCA, and effects of this surgical procedure were evaluated. Results Increased focal automaticity was regarded as electro physiologic mechanism for AT originating from atrial appendages proved by CARTO mapping. Three from right atrial appendage (RAA) and two from left atrial appendage (LAA). P′ wave configuration for AT from RAA: (1) positive P′wave in I and aVL leads; (2) positive P′ wave in II, III and aVF leads; (3) negative and double-peak P′ wave in V1 lead. P′ wave configuration for AT from LAA: (1) negative P′ wave in I and aVL leads; (2) positive P9 wave in II, III and aVF leads (3) positive P′ wave in V1 lead (1 case) or bidirectional P′ wave with positive tendency (1 case). Immediate success rate for RFCA was 100%. three recurred (60%) during follow-up (5–14 months). Of these recurred, two originated from RAA (2/3, 66.7%) and one from LAA (1/2, 50%). Appendectomies were performed under general anaesthesia with beating heart, reversions to sinus rhythm were gained at time the appendages were incised. No recurrence was detected during 5–14 months9 follow-up. Conclusions (1) The electrocardiographic characteristics of AT originating from atrial appendages in children are unique. (2) RFCA under CARTO mapping system by cooled tip cather is safe and effective for AT originating from atrial appendages in children while recurrence rate is high. (3) Appendectomy for AT originating from atrial appendage is reliable, while the assurance of AT origin by CARTO mapping system should be gained preoperatively.

A 57-year-old man with a long history of frequent chest pain was admitted to our cardiac center. Four years ago, he underwent coronary artery bypass grafting (CABG) with the left internal mammary artery (LIMA) to the distal segment of the left anterior descending coronary artery (LAD) grafting for myocardial bridging (MB) in LAD despite having β-blockers and calcium channel blockers. However, the patient still had recurrent angina within 3 months after the operation. ECG showed negative T wave in leads V1 and V2. On echocardiography, left ventricular ejection fraction was 60% with normal regional wall motion. The repeat coronary angiography showed a MB in the proximal LAD about 3 cm in length with 90% systolic narrowing of the LAD, and the LIMA graft was totally occluded (Figure 1A through 1C). The …

无顶冠状静脉窦综合征(unroofed coronary sinus syndrome,UCSS)罕见.2001年10月至2008年4月,我们为7例完全型无顶冠状静脉窦综合征的先天性心脏病病儿(人)实施了手术治疗,畸形矫正满意,效果优良,无住院死亡,均治愈出院.

目的 总结分析瓣膜病合并重度肺动脉高压患者行机械瓣膜置换术的围术期治疗经验.方法 分析瓣膜病合并重度肺动脉高压(肺动脉收缩压>70 mmHg)的机械瓣膜置换术患者41例,其中二尖瓣置换术(MVR)21例(风心病19例,脱垂2例),主动脉瓣置换术(AVR)4例(风心病3例,二叶瓣畸形1例),双瓣置换术(MVR+AVR)16例(均为风心病),同时行De Vega三尖瓣成形术27例,冠状动脉旁路移植术3例,左房血栓清除术6例.回顾术前治疗措施、术中注意事项、术后治疗方法,通过超声心动图评价恢复情况.结果 41例患者中死亡2例(4.9%),围术期发生肾功能不全2例(4.9%).术后随访12～66个月,心功能均恢复至I～II级.肺动脉压力术后1周显著下降(91 mmHg±19 mmHg vs 37 mmHg±10 mmHg),3个月后进一步下降至(35±13)mmHg.结论 瓣膜置换术可显著降低瓣膜病患者的肺动脉压力,进而改善其心功能.在术前准备周密、手术方案合理、积极预防和处理好术后并发症的前提下,重度肺动脉高压并不显著增加机械瓣膜置换手术的病死率.

Objective To study the diagnosis accuracy and features of echocardiography on aortic coarctation combined with hypoplastic aortic arch. Methods The echocardiographic characteristics of seven patients who were diagnosed as aortic coarctation combined with hypoplastic aortic arch were analyzed and compared with results of cardiac catheterization, CT scan and operation. The diagnostic features of echocardiography were summarized. Results Besides the echocardiographic characteristics of aortic coarctation,other specific echocardiographic characteristics of hypoplastic aortic arch were:distinct stenoses of transverse and decsending part of aortic arch were visualized in supra-sternal long-axis view; the range of hypoplastic part started from innominate artery beginning or left common carotid artery beginning; the ratio of diameter of hypoplastic aortic arch over that of descending aorta at diaphragm was less than 0. 5; the blood flow speed detected by Doppler at the site of aortic coarctation was in the normal range. The echocardiographic characteristics of the aortic coarctation combined with hypoplastic aortic arch were correspondent to the results of cardiac catheterization, CT scan and operation results. Conclusions There is high accuracy of echocardiography in diagnosis of aortic coarctation combined with hypoplastic aortic arch. Hypoplastic aortic arch should be paid more attention in diagnosis of aortic coarctation.

Objective To study the value of transesophageal echocardiography(TEE) in the operation of Ebstein's anomaly(EA).Methods The TEE characteristics of 69 patients with Ebstein's anomaly were retrospectivly analized.Results TEE could display the anatomic features of tricusipid valves(TV) clearly in all patients.Six cases were found having downward displacement of anterior leaflet before cardiopulmonary bypass,6 were found having hypoplasia of posterior leaflet, and posterior leaflets were absent in 3 cases.Ten cases were found middle regurgitation of TV by TEE after surgical correction during the operation, which had been improved satisfatorily by second time TV plasty.Conclusions TEE is useful in accurate diagnosis of the EA,and evaluation of operative effects during the operation, thus could help increase success rate of operation.

目的总结再次心脏手术的危险因素和有效治疗方法。方法回顾2004年4月—2007年3月84例再次实施心脏外科手术的临床资料,总结不同病种病人再次手术的情况及经验。结果 83例病人手术成功。术中最危险并发症严重大出血4例,均止血成功;死亡1例,死亡率1.2%。死亡原因:术中因低心排血量综合征无法停止辅助循环。结论把握恰当的再次手术时机,术前的精心准备,术中的精细操作,能有效减少或避免再次心脏手术开胸过程中严重的大出血,提高了再次心脏手术的安全性及成功率。

OBJECTIVE:To analyze the surgical strategy and result of one-stage repair for congenital aortic arch disease associated with other cardiac anomalies.METHODS:Between April 1993 and November 2009, 25 consecutive patients aged 26 d to 6.5 years underwent one-stage repair for congenital aortic arch disease with other cardiac anomalies. Among them, 6 patients had coarctation of aorta, 6 patients had interrupted aortic arch, and 13 cases had hypoplasia of aortic arch. The surgical techniques include excision of the anterior wall of pulmonary artery, resection of patent ductus arteriosus tissue, aortic arch reconstruction with autologous pulmonary artery wall, reconstruction of the pulmonary artery and repair of the associated defects.RESULTS:Twenty-four patients survived and recovered uneventfully. One patient died of pulmonary hypertension crisis in hospital. The reconstruction of the aorta and the correction of the intracardiac anomalies were proved by postoperative echocardiography and CT scan. There were no neurological or other complications. The follow-up showed that all patients developed normally and there were no restenosis of the aorta arch.CONCLUSIONS:With the benefits of growth potential and less tension, autologous pulmonary artery tissue is an optimal choice in aortic arch reconstruction. One-stage repair of congenital aortic arch disease associated with other cardiac anomalies can achieve good results.

OBJECTIVE:To review the experience of staged total cavopulmonary connection (TCPC) in complex congenital heart diseases.METHODS:From June 1998 to March 2008, 22 patients underwent staged TCPC for complex congenital heart diseases. Among them, 9 were univentricular and pulmonary artery valve stenosis; 3 were univentricular and pulmonary artery atresia; 1 was transposition of great arteries, crisscross heart and pulmonary artery valve stenosis; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery atresia and atrioventricular valvular regurgitation; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery valve stenosis and atrioventricular valvular regurgitation after Glenn procedure; 1 was mirror image dextrocardia, single ventricle, pulmonary artery atresia, major aortopulmonary collateral arteries (MAPCAs) and right pulmonary arteriovenous fistula after Glenn procedure; 4 were tricuspid atresia and pulmonary artery valve stenosis; 1 was tricuspid atresia and pulmonary atresia; 1 was mirror image dextrocardia, double-outlet of right ventricle, left ventricular hypoplasia, pulmonary artery valve stenosis, tricuspid incompetence, and MAPCAs. Among them, 5 patients received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC. Seventeen patients received bidirectional Glenn procedure, the mean age was (5.9+/-4.4) years old. Pulmonary artery pressure pre-Glenn procedure was 17 to 20 mm Hg (1 mm Hg=0.133 kPa). Atrioventricular valve incompetence in 3 patients. Nakata index was less than 200 mm2/m2 in 4 patients before the first stage operation. The age of TCPC procedure was (9.6+/-4.9) years old, the interval time was (3.7+/-1.2) years.RESULTS:There was one in-hospital death, the mortality was 4.5%. The patient with univentricular and pulmonary atresia, received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC and died of pneumorrhagia. Other patients were recovered well, postoperative central venous pressure was 12 to 18 mm Hg, percutaneous oxygen saturation was 90% to 96%. The cardiac function were in NYHA class I to II.CONCLUSIONS:The staged TCPC was a good procedure in high-risk Fontan candidates. The results were satisfactory for those patients. This staged strategy may extend the operative indications for the Fontan procedure.

OBJECTIVE:To retrospectively analyze the experiences, indications, technique, and results of coronary artery bypass grafting (CABG) in patients over 70 years old.METHODS:Ninety-one patients received coronary artery bypass grafting from March 2004 to March 2008. Ages ranged from 70 to 83 years old, 22 patients over 75 years old. Conventional CABG (CCABG) in 72 patients, off-pump CABG (OPCAB) in 19 patients. Clinical data has no significant differences in two groups. The rate of using left internal mammary artery was 96.7%. The number of grafts in CCABG and OPCAB group were 2 to 5 (3.5 +/- 0.8) and 1 to 4 (2.9 +/- 0.7) respectively.RESULTS:In-hospital death in 2 cases, both were from chronic obstructive pulmonary disease and pulmonary infection. Cerebral infarction in 1 case and pulmonary infection in 2 cases in CCABG group, but no significant difference between two groups, and no difference in intubation, ICU stay, respiratory failure, renal function failure. But number of grafts in CCABG was significantly more than that in OPCAB (P < 0.01). Postoperative follow-up was 3 to 36 months, 1 case with recurrent angina in OPCAB.CONCLUSIONS:According to the characteristic of coronary artery disease in elderly, fully revascularization and improving myocardial blood supply, patients over 70 years old with CABG can obtain the same efficacy as younger patients. There were not significant difference between CCABG and OPCAB.

Objective To summarize the experiences of reoperations on Ebstein's anomaly. Methods Clinical data of 17 cases of Ebstein's anomaly (6 males and 11 females, aged from 3 to 54 years old, averaged 23) were analyzed retrospectively. All the patients felt short breath and palpitation after exertion, and cyanosis was found in two patients. In the first operation, downwards displaced tricuspid valve leaflet was suspended and atrialized right ventricle was replicated in 14 cases, tricuspid anuloplasty and atrial septal defect were repaired in 2 cases, and tricuspid valve was replaced in 1 case. The interval time between two operations was 1-20 years in an average of 8 years. Before second operation, the cardiac function was NYHA Ⅱ in 6 cases, NYHA Ⅲ in 11 cases. Eight cases received Ebstein's anomaly anatomic correction, including transplanting downward displaced tricuspid valve and excising atrialized right ventricle. Seven cases received tricuspid valve replacement. Two cases received tricuspid valveplasty. All of operations were performed on arrested heart with moderate hypothermic cardiopulmonary bypass. The time of cardiopulmonary bypass was 70-287 (89.3±11.1)min, the time of aorta clamping was 70-287 (64.0±8.6)min. The transesophageal echocardiography was routinely performed before the operations and after the hearts rebeating. Results No case died in hospital, and all the patients were followed up for 1-3 years. Transesophageal echocardiography showed the tricuspid valve was closed well (non-reflow in 9 cases, mild reflow in 1 case) in 10 cases which received Ebstein's anatomic correction and tricuspid valveplasty. The cardiac function states of patients were significantly improved (NYHA Ⅰ-Ⅱ). Conclusion Most of patients with reoperations on Ebstein's anomaly can received anatomic correction. Tricuspid valve replacement is available if the pathology of tricuspid valve is severe.