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先天性心脏病外科治疗中国专家共识（五）：再次开胸手术

法洛四联症合并完全性房室隔缺损的外科治疗

目的 探讨法洛四联症合并完全性房室隔缺损外科治疗经验。 方法 回顾性分析9例患者的临床资料，其中完全性房室隔缺损A型3例，C型6例；均有右心室流出道漏斗部狭窄，6例伴明显的肺动脉瓣及瓣环狭窄。术前共同瓣轻度反流7例，中度反流2例。 1例行中央体肺分流术，1例行心外管道全腔静脉-肺动脉吻合术，其余7例均行一期根治手术，其中跨肺动脉瓣环5例。 结果 9例患者死亡3例；1例婴儿术后2d脱机，后因低氧再次插管呼吸机辅助呼吸40d脱机，5例在术后1d脱机，呼吸循环功能良好，顺利康复出院。随访１个月～4年，4例有轻度二尖瓣反流，无残留心脏畸形。 结论 法洛四联症合并完全性房室隔缺损手术的关键在于确切修补室间隔缺损、完善修复二尖瓣、防止左心室流出道狭窄及Ⅲ°房室传导阻滞。

Surgical Treatment of Complicated Congenital Heart Diseases with the Modified Fontan Operation

Objective To investigate the operative characteristic and results of the modified Fontan procedure, and improve the application of Fontan procedure in the clinic. Methods From Sep. 1992 to June 2006, 77 cases (aged 2.5 years to 20.0 years) with a wide range of complicated congenital heart diseases underwent the modified Fontan procedure.Right atriumpulmonary artery connection were performed in 21 cases, intraatrium fenestrated baffle or conduit total cavopulmonary connection were performed in 28 cases, extracardiac conduit total cavopulmonary connection were performed in 24 cases, atrium wall lateral tunnel total cavopulmonary anastomosis were performed in 2 cases, extracardiac pericardialtube total cavopulmonary anastomosis was performed in 1 case, and extracardiac direct total cavopulmonary connection was performed in 1 case. Results In early postoperative period, there were 1 case of successful reoperation and 5 death, the cause of death were heart failure (3 cases), arrhythmia (1 case) and cerebral hemorrhage (1 case). The early survival rate was 93.5%(72/77), with 92.0% free from failure. The eject fractions of left ventricle (LVEF) after operation were increased than those before operation （68.5%±4.0% vs.62.0%±4.5%,P=0.032） and left ventricular enddiastolic diameter (LVEDD) were decreased than those before operation （52.5±7.8mm vs.62.5±11.0mm, P=0.013）. A total of 63 cases (87.5%) were followed up from 1 to 15 years after operation. In late postoperative period, there were 4 cases of death, and 1 of successful re-operation. Late survival rate was 88.3%, with 86.0% free from failure. Conclusion In the treatment for complicated congenital heart diseases, the modified Fontan procedure could result in good early and longterm outcome, and the choices for surgical procedure are various.

小儿先天性心瓣膜病的外科治疗

目的 探讨小儿先天性心瓣膜病手术治疗的方法,并总结其经验.方法 45例先天性心瓣膜病患儿实施主动脉瓣修复成形术4例,二尖瓣综合成形术31例,二尖瓣置换术、三尖瓣置换术和主动脉瓣环扩大瓣膜置换术各1例,三尖瓣综合成形术9例次.外科技术包括瓣叶或交界折叠(悬吊)成形,瓣环环缩,置Carpentier环,升主动脉加宽换瓣,瓣叶楔形切除及瓣下结构畸形修复等,同期矫治其它心内畸形.结果 1例术后死于重度低心排血量综合征,其余44例顺利恢复.二维超声心动图示5例有轻度二尖瓣反流,1例有轻度三尖瓣反流,随访5个月～8年,效果稳定.结论 采用综合成形技术,保留自身瓣膜是小儿先天性心瓣膜病外科治疗的首选方法,具有效果确切,术后心功能好,并发症少,无需长期抗凝及不影响生长发育等优点.

Surgical Treatment for Anomalous Origin of One Pulmonary Artery in Infants and Children

Abstract：　Objective　To summarize our experience of surgical treatment for anomalous origin of one pulmonary artery in infants and children.　Methods　From March 2005 to May 2010，11 patients with anomalous origin of one pulmonary artery and other concomitant congenital cardiovascular malformations underwent surgical repair in Xijing Hospital of Fourth Military Medical University．The mean age of the patients was 11.5 months with a range from 2 months to 36 months．Their mean body weight was 7.1 kg with a range from 4 to 13 kg. Seven patients had anomalous origin of the right pulmonary artery from the ascending aorta, and four patients had anomalous origin of the left pulmonary artery from the ascending aorta. All the eleven patients had other concomitant intracardiac anomalies or vascular malformations as well as pulmonary hypertension, and underwent one stage surgical repair via median sternotomy under hypothermia and cardiopulmonary bypass.　Results　Their operation time was 169 - 293 （231±55） min, cardiopulmonary bypass time was 87-210 （138±47） min, and aortic-clamping time was 45-133 （86±28） min. There was one postoperative death who had low cardiac output syndrome after repair for tetralogy of Fallot and anomalous origin of the right pulmonary artery. The overall postoperative mortality was 9.1％. Postoperative echocardiography of all the surviving patients showed their left and right pulmonary artery origined from the right ventricle and pulmonary artery with satisfactory malformation correction but no residual shunt and pulmonary stenosis . All the surviving ten patients were followed up with a follow-up rate of 100% and mean follow-up time of 13.5 months with a range from 3 to 32 months. Their echocardiography during follow-up showed that there was no pulmonary stenosis in all the patients, and pulmonary blood pressure significantly decreased in 9 patients.　Conclusion　Patients with anomalous origin of one pulmonary artery should undergo surgical repair as early as possible with satisfactory short-term outcomes in infants and children. For elder patients with irreversible pulmonary hypertension, the choice of surgical treatment should be more cautious. During the surgery, the anomalous pulmonary artery and ascending aorta should be dissociated fully, and transection of the ascending aorta is helpful to get a satisfactory operating field view for the surgeon. Repairing aortic defect with autologous pulmonary arterial patch can effectively avoid the occurrence of postoperative aortic aneurysm.

先天性左心室流出道梗阻的手术治疗

目的分析我们中心近年来先天性左心室流出道梗阻患儿的外科治疗经验，总结手术方式及治疗效果。方法回顾性分析 2011 年 11 月至 2017 年 12 月我院心血管外科共收治先天性左心室流出道梗阻患儿 58 例的临床资料。男 37 例、女 21 例，年龄 2～16 个月（8.49 ± 3.21）岁，体重 2.70～56.00（31.00±6.72）kg。所有患儿均在全身麻醉低温体外循环下行外科矫治术。结果所有患儿顺利出院，随访 6～72 个月。患儿术后心功能良好，行主动脉瓣置换的患儿人工瓣功能正常，无瓣周漏、传导障碍及明显的出凝血并发症。结论先天性左心室流出道梗阻的外科矫治效果良好。选择合适的手术方式、完全有效的解除流出道梗阻及坚持长期随访等，往往能够取得满意的外科治疗效果，促进患儿术后康复。

Surgical Managemen t for Coarctation of Aorta Associated with Heart Anomalies in Infan ts

Corresponding author: Y I Ding -hua, E -mail: yidh@fmmu. edu. cnAbstract: 　Objective　To investigate the optimal surgical approaches for coarctation of aorta (CoA ) associated with heart anomalies (CoA -HA ) in infants through analyzing the immediate and long-term outcome post-operation. Methods　From May 1998 to November 2006, 29 patients with CoA -HA were admitted to this institute. Subclavian flap angioplasty was performed in three patients, excision of coarctation and end-to-side anastomoses in six pat ients,and end-to-end anastomoses in the remaining 20 pat ients. Their clinical data were retrospect ively reviewed and the long-term follow -up results obtained through telephone o r letters. 　Results　Three patients died of operations, of whom one died of low cardiac output syndrome, one died of sepsis and one died of multiple organs failure. In two months’to eight years’follow -up , no later death and severe neurological complications were found. Two patients suffered from recurrent coarctation, but had no sense of symptoms and free from re-operation. In all pat ients neither systemic hypertension, nor arterial aneurysm and aortic valve regurgitation were found. 　Conclusions　Mortality of surgical corrections is accepted fo r CoA -HA in infants. Preoperative heart dysfunction and prolonged mechanical ventilation are the high risks of operative death. Recurrent coarctation is the major later comp lication.

Analysis of 85 infants of minimal median sternotomy for cardiac surgery under cardiopulmonary bypass

目的 探讨胸部正中小切口在婴幼儿先天性心脏病手术治疗中的可行性及效果。 方法 将我院 2016 年 5 月至 2016 年 10 月 170 例行手术治疗的常见先天性心脏病婴幼儿患者分为两组：常规组，85 例，男42例、女43例，年龄（6.9±2.1）个月，采用常规胸部正中切口；小切口组，85 例采用胸部正中小切口，男43例、女42例，年龄（6.4±1.8）个月。小切口手术切口于平第 3 肋间切开，止于剑突起始处上 0.5 cm，刚好放入小胸骨撑开器为好。 结果 两组患儿体外循环时间差异无统计学意义（P>0.05）。小切口组手术时间略长（P<0.05）。两组预后没有差别，但是小切口组伤口长度显著缩短[（7.8±0.8） cmvs. （4.0±0.5）cm，P<0.05]。 结论 正中小切口基本具有胸骨正中切口的优点，可显露心脏各部位，满足绝大部分心脏探查和手术操作需要，必要时仍可向上延长切口使心内操作不受限制等优点，故认为正中小切口在婴幼儿心脏手术中具有良好的安全性和美观性。

Diagnosis and treatment of aortopulmonary window associated with severe pulmonary hypertension

ObjectiveTo study the diagnosis and treatment of aortopulmonary window (APW) associated with severe pulmonary hypertension.MethodsThe clinical data of 23 patients with APW undergoing surgical treatment in The First Affiliated Hospital of Air Force Medical University from 2010 to 2018 were retrospectively reviewed. There were 9 male and 14 female patients. The age was 3-132 (4.63±2.14) months. The weight was 3.3-35.0 (17.3±3.6) kg.ResultsWindows were situated in the proximal of semilunar valve (type Ⅰ) in 8 patients, and distal of the aorta (type Ⅱ) in 14 patients, from proximal to distal (type Ⅲ) in only 1 patient. Eleven patients were isolated APW, the others were combined with cardiac defects. The mean pulmonary artery pressure was 68.4±7.5 mm Hg. All patients underwent surgical correction under general anesthesia and hypothermia cardiopulmonary bypass. All patients were discharged uneventfully, with an average follow-up time of 4 years. The patients showed good outcomes and no residual shunt after surgery, and the pulmonary artery pressure decreased to normal.ConclusionAPW is an uncommon congenital cardiac anomaly. The clinical presentation is an excessive left-to-right shunt, and most patients present early in life. Development of pulmonary hypertension and pulmonary vascular resistance is usually rapid. Operative treatment is indicated as soon as the diagnosis is established, regardless of the patient’s age, and most patients after surgery have a good long-term outcome.