Correction of total anomalous pulmonary venous connection of the cardiac type

Surgical treatment of the cardiac type of total anomalous pulmonary venous connection requires special techniques. The treatment and outcome in 17 consecutive patients who had undergone primary repairs of the cardiac type between 1965 and 1996 were reviewed. The median age was 3 months and median weight 4.2 kg. The connection was the coronary sinus in ten patients, and the right atrium in six. Interatrial communication was routinely augmented. The right atrial cavity was partitioned using a patch to direct the anomalous pulmonary veins into the left atrium through the atrial septal defect in the first 13 patients. In the last four patients, the free wall flap of the right atrium was developed as a neoseptum. There were three early postoperative deaths during the early period of conventional repair. Two patients developed residual or recurrent diffuse obstruction in the individual lobar veins; reoperation to relieve the obstruction was attempted but unsuccessful. One sudden death occurred in a patient with occasional heart block. Ten survivors have been asymptomatic during follow-up, but two incomplete heart blocks and one atrial flutter were noted among patients who underwent conventional repair. The right atrial wall flap technique was not associated with any mortality or morbidity, such as arrhythmia and recurrent pulmonary vein stenosis during 12 to 63 months of postoperative obstruction. In conclusions, a flap technique using the right atrial wall appears to be a promising method to decrease the incidence of supraventricular arrhythmias and pulmonary vein drainage obstruction following repair of the cardiac type.     

右房异构单心室伴完全性肺静脉异位引流的外科治疗

目的 介绍右房异构、单心室伴完全性肺静脉异位引流（TAPVC）的外科治疗经验和TAPVC在右房异构纠治手术中意义。方法 1999年6月于2000年3月手术治疗右房异构、单心室伴TAPVC5例。其中4例为心上型,1例为混合型。所有病儿均施行一侧或双侧的双向腔肺血管吻合术（BCPC）,4例同时进行TAPVC纠治术。结果 手术死亡1例,原因为 诊断不明确、体外循环时间过长、术后严重低心排和低氧血症。生存4例术后均无残余解剖梗阻,术后血流动力学稳定,血氧饱和度明显增高,随访结果满意。结论 术前明确TAPVC的诊断对指导手术具有重要意义;右房异构、单心室伴TAPVC在进行分流术时应同时施行TAPVC纠治术。     

Hydatid cyst in the right ventricular outflow tract: Successful surgical outcome of a rare entity

Echinococcosis is endemic in India but heart is an uncommon site of presentation of hydatid cyst. We report a case of a young female with hydatid cyst of right ventricular outflow tract who was operated through median sternotomy under cardiopulmonary bypass and entire cyst was successfully removed. The preoperative diagnosis was confirmed by histopathology of the excised specimen.     

心上法矫治小儿完全性心上型肺静脉畸形引流(附11例报告)

目的介绍心上法矫治心上型完全性肺静脉畸形引流(TAPVR).方法 1998年6月至2001年8月,采用心上法矫治心上型TAPVR 11例,其中男7例,女4例;年龄5月龄～15岁,平均(5.33±4.98)岁;体重6.4～33.0kg,平均(15.09±8.78)kg.在全麻、低温体外循环下行矫治术,正中切口纵劈胸骨入胸,经横窦,上腔静脉与升主动脉之间显露共同肺静脉干及左心房,将其侧侧吻合.结果术后早期(30 d)无死亡,随访4～36个月,无远期死亡及心律失常发生.结论心上法矫治心上型TAPVR可获得良好的显露和足够大的吻合口,手术对心脏的损伤小,可降低术后心律失常的发生率.     

心上型完全性肺静脉异位引流外科治疗24例临床分析

目的总结心上型完全性肺静脉异位引流的外科治疗经验。方法1995年7月至2004年6月,手术治疗心上型完全性肺静脉异位引流24例,其中男11例,女13例;年龄40d～35岁,其中6周岁以下23例,体重3．8～54．0kg。经右心房、房间隔切开左心房后壁和肺静脉共干吻合10例;横行切开双心房与肺静脉共干吻合8例;2例分别用人造血管补片和自体心包补片将垂直静脉入口和扩大的房间隔切口一并隔人左心房;1例行左心房后壁和肺静脉共干吻合的同时用自体心包补片将肺静脉共干人口和扩大的房间隔切口一并隔入左心房;3例行左心房顶和肺静脉共干吻合。术后呼吸机辅助呼吸,多巴胺、硝普钠辅助循环2～5d。结果手术死亡1例,死于低心排出量综合征;术后心包填塞1例,再次手术止血后治愈;心律失常6例,均治愈;急性肺水肿1例,二次手术重新开放垂直静脉延迟闭合治愈。术后随访3个月至7年,平均2．5年。术后2周严重室上性心律失常1例,治愈;远期因心包补片挛缩致肺静脉严重回流障碍二次手术1例,治愈;其余患者均未见远期并发症。结论心上型完全性肺静脉异位引流可采用不同的手术方法,正确的手术方法和对并发症的及时处理可以获得良好的治疗效果,单纯应用右心房内补片应选用合适的修补材料,左心房顶和肺静脉共干吻合法暴露好、操作简单、心律失常发生率低。     

Asplenia syndrome with total anomalous pulmonary venous connection

Three patients of asplenia syndrome with total anomalous pulmonary venous connection (TAPVC) were reported. Case 1 with exceeding pulmonary blood flow, underwent TAPVC repair and pulmonary artery banding as a first palliation before bi-directional Glenn shunt. Case 2 did not require any surgery to control the pulmonary blood flow before the simultaneous procedure of TAPVC repair and bi-directional Glenn shunt. Case 3 with decreased pulmonary blood flow underwent a complicated course with 3 times of Blalock-Taussig shunts and the repair of TAPVC to prepare for bi-directional Glenn shunt. Simultaneous repair of TAPVC with the procedure which aimed to control the pulmonary blood flow at the first palliation surgery will simplify the control of pulmonary blood flow and prepare good condition of the lung for the Fontan operation in the future.     

应用无内膜接触缝合技术治疗心上型全肺静脉异位引流

目的 探讨无内膜接触缝合技术应用于初次心上型全肺静脉异位引流(TAPVC)的外科矫治,以顶防术后肺静脉梗阻发生的可行性.方法 自2007年12月至2008年12月,25例TAPVC病儿接受体外循环下畸形矫治手术,其中心上型TAPVC 9例手术中5例采用无内膜接触缝合技术.男2例,女3例;年龄为2个月～13岁;体重为4.5～21.0 kg,平均(7.9±6.4)kg.手术选择心包斜窦入路,沿共同静脉长轴横向剖开,并将此切口上延至垂直静脉的心包返折处,使用7-0 PDS缝线将左心房后壁切口与共同静脉切口周边的心包组织吻合,通过"控制性出血技术"将肺静脉回流的血液引流进入左心系统;部分结扎垂直静脉.结果 5例采用无内膜接触缝合技术进行外科矫治的病儿全部生存.1例病儿因术前并发双侧肺实变,术后机械辅助呼吸超过7天其他病儿均顺利康复,无并发症.术后行心脏超声随访,随访1～13个月,中位数为7个月,常规随访时间点分别设在出院前、术后3个月、半年及1年.至目前为止,5例病儿肺静脉回流通畅,血流速度0.65～0.85 m/s.结论 在治疗心上型全肺静脉异位引流方面,无内膜接触缝合是一项切实可行、易于操作的外科技术,可以预防术后早期肺静脉梗阻的发生;但其大宗病例的远期疗效,尚有待进一步的研究和探索.     

心脏右侧径路纠治心下型肺静脉异位回流

目的 评估经心脏右侧径路纠治心下型完全性肺静脉异位回流方法的价值.方法 2005年9月至2007年12月,采用经心脏右侧进路方法纠治心下型完全性肺静脉异位回流7例.平均年龄(70.57 -44.67)天;平均体重(4.07±0.87)kg.其中3例肺静脉回流梗阻,2例卵圆孔未闭较小.结果 无手术死亡.术后并发症包括:低心排5例,发生肺动脉高压危象3例,呼吸机辅助7天以上2例,肺部感染2例.术后随访6个月至2年,超声检查心房内无残余分流,吻合口无明显梗阻,流速1.20～1.47m/s;心功能:射血分数0.70～0.79,短轴缩短率0.32～0.44;1例出现肺静脉回流的流速增快,其右上肺静脉2.60m/s,右下肺静脉2.12m/s,继续随访中.结论 采用经心脏右侧径路方法纠治心下型完全性肺静脉异位回流,取得了较好效果.手术成功取决于左房与垂直静脉的吻合口大小,保证肺静脉回流无梗阻.

Abstract：

Objective Infracardiac total anomalous pulmonary venous connection, a rare congenital cardiac defect, is associated with high mortality. A modification was designed for the procedure to reduce the post-operative obstruction in the pulmonary venous. Methods From September 2005 to December 2007, seven patients with infracardiac total anomalous pulmonary venous connection were treated with repair surgery through right side approach. The patients' age at operation was (70.57 ±44.67) days , the weight was (4.07 ±0.87) kg. Three patients had pulmonary venous obstruction, and 2 with small PFO. A modified right - side approach for repairing this defect was used. Results No death occurred after the operation. The postoperative complications included low cardiac output in 5 patients (71.43%), pulmonary hypertension crisis in 3patients ( 42.86% ) , mechanical ventilation for more than 7 days, which happened in 2 patients (28.58%) and pulmonary infection. All of the patients received follow-up. No residual shunt and pulmonary venous return obstruction were identified on echocardiogram(with a velocity from 1.2 m/s to 1.47 m/s). The heart function of patients was within the normal range(EF 0.70 -0.79, FS 0.32 -0.44). Conclusion The modified surgical procedure for the correction of infracardiac total anomalous pulmonary venous connection by right side approach was associated with favorite clinical outcomes, The post-operative outcomes depended on the size of anastomosis between the common vein and left atrium and the patency of the pulmonary venous return. Adequate size of anastomosis and maintenance of the spatial structures in adjacent regions were helpful in decreasing the adverse effect of postoperative obstruction.     

心上型完全性肺静脉异位引流的单中心治疗结果

目的 分析心上型完全性肺静脉异位引流的手术治疗结果.方法 回顾性分析2014～2019年在本中心行外科手术治疗的98例心上型完全性肺静脉异位引流患者的临床资料,其中男64例、女34例,中位手术年龄3.0(1.5,7.0)个月,中位体重5.0 (4.0,6.0) kg.术前肺静脉梗阻23例(23.5％).传统手术技术治疗...     

Improving outcomes in functional single ventricle and total anomalous pulmonary venous connection

Background

We have previously reported that the outcome of infants with functional single ventricle and total anomalous pulmonary venous connection is poor relative to that of other single ventricle patients. Younger age at initial operation and obstructed total anomalous pulmonary venous connection were found to be risk factors for mortality. A review of our recent experience was undertaken to determine whether results in these patients are improving.

Methods

Medical records of 18 patients admitted after 1997 were reviewed (group B) and compared with the previous group of 73 patients admitted between 1984 and 1997 (group A). Data were analyzed using a Cox proportional hazards model.

Results

Median age at first operation was the same for both groups. The incidence of obstructed total anomalous pulmonary venous connection was not significantly different between groups (29% versus 33%, p = 0.70). Early survival is significantly improved for group B compared with group A (p = 0.015). Only group and younger age at initial operation were found to be risk factors for mortality. In the current group, 5 patients have undergone superior cavopulmonary connection with one death, 3 have undergone Fontan completion with no deaths, and heart or heart-lung transplantation was performed in 5 patients with two deaths.

Conclusions

Early survival in patients with single ventricle and total anomalous pulmonary venous connection has improved significantly in recent years, but intermediate survival is still approximately 50%. Selective management using staged reconstructive surgery and transplantation may result in improved survival. Further study may identify risk factors for mortality and improve selection of appropriate patients for each therapy.     

Modified repair of mixed total anomalous pulmonary venous connection

Mixed total anomalous pulmonary venous connection (TAPVC) is a rare condition, accounting for only 5% of diagnosed TAPVC. It is associated with a poor prognosis unless surgically corrected by connecting the pulmonary venous sinus to the left atrium and optionally dividing the abnormal connection to the systemic venous system. We report a modified technique of complete intracardiac repair of mixed supracardiac and cardiac TAPVC in a 3-year-old child without pulmonary venous sinus-to-left atrium anastomosis.     

混合型完全性肺静脉异位引流的外科治疗

目的总结手术治疗混合型完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的经验。方法 2006~2018年,我院共完成51例混合型TAVPC患者(排除合并单心室、法洛四联症等患者)的外科治疗,其中男35例、女16例,中位年龄102.0(59.0,181.0)d,中位体重5.0(4.1,6.4)kg。根据解剖形态将患者分为3类:3+1型(38例,3支肺静脉回流入同一个部位,而另一支肺静脉回流另一侧部位);2+2型(9例,两侧的肺静脉分别回流入不同的位置);怪异型(4例,无法归入以上两类的怪异解剖类型)。结果无患者院内死亡,中位随访时间41.0(18.0,86.5)个月。术后发生肺静脉梗阻10例。Kaplan-Meier生存曲线示3种类型术后肺静脉梗阻率差异无统计学意义(P=0.239)。Cox分析发现术前肺静脉梗阻与术后肺静脉梗阻明显相关(P=0.024)。结论混合型TAPVC解剖形态多变,需要个体化手术方法。     

Repair of total anomalous pulmonary venous connection in neonates

Aim To study the results of repair of Total Anomalous Pulmonary Venous Connection (TAPVC) in neonates Materials and Methods Retrospective study of 27 neonates operated for TAPVC between January 2001 and October 2003. 27 neonates underwent TAPVC repair during the 2-year period. 21 were males and 6 were females. Results Total hospital stay ranged from 10 days to 75 days. 9 supracardiac, 13 infracardiac, 3 cardiac and 2 mixed type comprised the group. Obstruction was seen in 24 patients. All the patients had severe pulmonary artery hypertension. Vertical vein was ligated in almost all cases either at the time of surgery or during the closure of sternum. Delayed sternum was closed in all cases but 7. Of 3 deaths, one died after permanent pacemaker implantation (about 1 month after the initial surgery of repair of cardiac TAPVC), one died due to pre-operative vascular access related accident and a third died due to post-operative low cardiac output. All survivors were thriving well at last follow-up. Conclusion Repair of TAPVC in the neonatal age has been found to be rewarding with significant improvement in the well being of the child. Judicious use of pulmonary vasodilators like nitric oxide, Sildenafil and phenoxybenzamine and delayed sternal closure has improved the results in this study group. Presented at the 50^th Annual Meeting of IACTS, New Delhi, Feb., 2004.