完全性肺静脉异位引流的外科治疗

目的 报道完全性肺静脉异位引流(total anomalous pulmonary venous drainage)的外科治疗疗效和体会. 方法 27例患者中(心上型19例、心内型8例),18例采用全身麻醉体外循环心脏停搏下手术,9例采用心脏不停跳手术.心上型横切右心房和左心房后壁,纵行切开肺总静脉,切口3.5～5.0 cm,与左心房后壁吻合,用自体心包片修补房间隔缺损(ASD),停机后结扎垂直静脉;心内型切开ASD与冠状静脉窦口之间的残余房间隔壁,以扩大ASD,自体心包修补ASD,并将肺静脉异位连接口与冠状静脉窦一并隔入左心房. 结果 结性心律4例,呼吸衰竭3例,全身水肿1例,大量胸腔积液5例;早期死亡1例,死亡原因为低心排血量综合征.其余患者均痊愈出院,随访6个月～10年,恢复良好,无吻合口狭窄发生. 结论 完全性肺静脉异位引流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,该手术对早晚期病变疗效均良好.     

全肺静脉异位引流心内修复术后护理

全肺静脉异位引流,是指左、右肺静脉未能直接与左心房相连,而与右心房或体静脉相连接。致使全肺静脉的氧合血,直接流入右心房。其发病率占全部先天性心血管畸形的1～4％。我院自1975年9月～1986年9月,对20例全肺静脉异位引流患者,进行了手术治疗。术后护理得法、无一例死亡。效果满意。一、临床费料全肺静脉异位引流心内修复术20例。其中男性6例、女性14例。年龄4～23岁;心上型13例、心内型7例。有紫绀症状者17例。二、护理要点 (一) 呼吸道的观察与护理全肺静脉异位引流病人,术前因肺静脉氧合血直接或间接的流到右心房,X线照片均示肺静脉充血,加之心上型和心下型手术需将肺静脉总干与左心房作一大的侧侧吻合,并闭合房间隔缺损和结扎垂直静脉。心内型需用人工补     

新生儿梗阻型完全性肺静脉异位引流的治疗

目的 评估新生儿完全性肺静脉异位引流( TAPVC)不同类型矫治手术方法和预后.方法 1999年至2011年,共收治68例新生儿梗阻型TAPVC急诊手术治疗,平均年龄16天,其中心上型21例,心内型8例,心下型36例和混合型3例.心上型和心下型TAPVC是将肺静脉共汇与左心房后壁作侧侧吻合,心内型TAPVC在心房内将扩大的冠状窦去顶将异位的肺静脉隔入左心房.结果 术后早期死亡2例,占2.9％.随访6个月至3年,经超声心动图随访,肺静脉吻合口均无明显狭窄,血液流速1.10 ～ 1.42 m/s.结论 早期的梗阻型TAPVC的纠治中左心房后壁与肺静脉共汇的侧侧吻合远期效果良好,肺静脉的梗阻情况需要远期进一步随访.     

心上型完全性肺静脉异位连接的手术方法与效果分析

目的总结心上型完全性肺静脉异位连接（S-TAPVC）矫治手术的经验,探讨不同手术方法的治疗效果。方法1985年5月至2007年12月,我们共完成86例S—TAPVC矫治手术,其中男49例,女37例;年龄7个月～35岁,平均年龄9．6岁;体重4．9～68．0kg,平均体重23．8kg。根据采取的不同手术方法将患者分为3组：组Ⅰ为经心房内切口吻合,20例;组Ⅱ为经左、右心房横切15吻合,49例;组Ⅲ为经左心房顶切口吻合,17例。肺静脉共干和左心房切口均采用间断连续吻合,用自体心包修复房间隔缺损（ASD）。术后肺静脉压≤15mm Hg则完全结扎垂直静脉,〉15mm Hg的完全开放或部分结扎垂直静脉。结果本组86例S—TAPVC矫治术,无早期死亡。术后3组左心房压分别为9．3±3．2mm Hg,9．9±2．9mm Hg和11．6±3．8mm Hg,开放或部分结扎垂直静脉例数分别为0例（0％）、7例（14．3％）和2例（11．8%）,发生心律失常例数分别为5例（25．0％）、15例（30．6％）和1例（5．9％）,严重低心排血量综合征分别为1例、0例和1例,二次开胸止血分别为2例、0例和0例。组Ⅲ术后早期心律失常发生率明显低于组Ⅱ（P=0．042）。结论S—TAPVC手术效果满意。经左心房顶切口吻合可以防止术后心律失常,特别适用于婴幼儿;经左、右心房横切口吻合手术视野显露良好,可以保证吻合口的大小,适合于大龄儿童或成年人。     

完全性肺静脉异位连接的外科治疗

目的　总结 18例完全性肺静脉异位连接的外科治疗经验。　方法　全组均在全身麻醉中度低温体外循环下进行手术 ,11例心上型患者除早期 1例经后径法矫治外 ,其他 10例经右心房切口径路矫治 ;7例心内型采用自体心包片将冠状静脉窦口经扩大的房间隔缺损隔入左心房 ,后 5例用 5 - 0 Prolene线连续缝合房间隔粗糙面后再矫治。结果　无手术死亡 ,2例心内型患者术后 6个月和 8个月再次手术 ,发生心律失常 7例 ,一过性肺水肿 2例 ,经治疗均痊愈。 16例随访 4个月～ 5年 ,心功能正常。　结论　提高手术成功率和远期疗效的关键为 :术中吻合口要足够大 ,术后及时处理心律失常、肺水肿和低心排血量 ,心内型矫治时消除房间隔粗糙面可防止术后肺静脉梗阻。     

部分型肺静脉异位引流至上腔静脉手术矫治方法的改良

目的 为防止部分型肺静脉异位引流至上腔静脉手术矫治后的相关并发症，对其手术方法进行改良。方法 4例患者接受了手术矫治。经右心房至上腔静脉的联合切口，利用上腔一右心房片修补房间隔缺损，同时将右上肺静脉的血流经房间隔缺损引流至左心房，再以一自体心包片加宽上腔静脉同时缝合右心房壁。结果 4例患者全部顺利恢复出院。术后早期超声心动图检查未见肺静脉或腔静脉的梗阻，亦无残余分流。术后随诊患者仍维持窦性心律。结论 该方法操作简单，显露良好，避免了残余分流及肺静脉或腔静脉梗阻的发生，同时避免了窦房结的直接损伤。     

静脉窦型房间隔缺损的外科治疗

目的 总结静脉窦型房间隔缺损（SVASD）外科治疗的方法和经验。方法 32例SVASD患者均伴有右侧部分或全部的肺静脉异位引流。上腔SVASD25例，其中22例采用双片心包补片修补，3例将异位引流的肺静脉改道、上腔静脉与右心耳吻合（Warden法）进行手术纠治。7例下腔SVASD患者中，3例为弯刀综合征，关闭房间隔缺损（ASD）的同时将异位引流的肺静脉离断后与左心房后壁吻合；其余4例采用单片心包补片修补ASD。结果 手术效果满意，无手术死亡。随访28例，心电图检查均为窦性心律；心脏超声心动图检查提示：心内无残余分流、右肺静脉及腔静脉回流无梗阻。结论 SVASD特殊的解剖结构决定了其多伴右侧肺静脉异位引流，纠治上腔SVASD时应注意避免损伤窦房结，防止上腔静脉回流梗阻；但弯刀综合征多伴右肺发育不良，心功能情况还须长期随访。     

肺静脉畸形引流的外科治疗

目的探讨肺静脉异位引流（APVC）的外科治疗经验。方法全组患者共10例，其中完全型3例（心上型），部分型7例（4例引流至右心房，3例引流至上腔静脉），男6例，女4例，全组患者均在中度低温体外循环心内直视下采用右心房切口，通过吻合和补片法将异位的肺静脉隔人左心房，同时纠正合并的心内畸形。结果手术死亡1例（10％），其余9例患者均痊愈出院。结论本病确诊后应尽早手术，从而防止肺血管阻塞性病变的进展，手术成功的关键是肺静脉回流是否通畅，随着手术技术的完善以及术后监护水平的提高，肺静脉异位引流的外科治疗可以取得满意的疗效。     

17例心下型完全性肺静脉异位引流的外科治疗北大核心CSCD

目的 评估新生儿、小婴儿心下型完全性肺静脉异位引流的纠治方法及效果.方法 2000年7月至2009年6月,共纠治心下型完全性肺静脉异位引流17例.病婴出生15天～3.7月龄,其中1月龄以下9例,占52.9%;体重（4.10±0.95）kg,其中5 kg以下15例,占全组88.2%.4例低温低流量灌注下,经右心房切口,切开房间隔,左房与肺静脉吻合;13例深低温停循环灌注,采用心脏上翻法,左心房斜形切口与肺静脉无张力缝合,其中吻合口宽度保证在2.5～4.0 cm.结果 全组手术顺利,无手术死亡.6例术后延迟关胸,5例发生肺高压危象,2例出现暂时性Ⅲ度房室传导阻滞,2例急性肾功能衰竭,4例肺水肿,所有病婴均治愈出院.1例术后反复出现肺部感染,心脏超声发现吻合口流速明显增快（3.05 m/s）、严重肺动脉高压、三尖瓣重度反流,1年后失随访.16例随访1个月～9年,平均（51.8±35.0）个月.随访期均行心脏超声、X线胸片、心电图检查.多数病婴心功能良好,心影较术前明显缩小,肺充血消失,心电图示窦性节律,吻合口血流速度正常.结论 新生儿及婴幼儿心下型完全性肺静脉异位引流病情危重,早期诊断、早期手术效果良好,手术治疗的关键在于左心房与肺静脉吻合口应足够大,避免狭窄的发生.     

左心房内吻合治疗心下型肺静脉异位引流效果分析

目的探讨左心房内吻合术治疗心下型完全性肺静脉异位引流的效果。方法对32例心下型完全性肺静脉异位引流新生儿实施深低温体外循环下左心房内吻合术。结果本组手术顺利,23例患儿术后延迟关胸,无手术死亡病例。5例心脏复跳后心率较慢安装临时心脏起搏器,术后1~5 d心率恢复正常。除2例因低心排综合征于术后早期死亡,其余患儿均治愈出院。3例患儿术后反复出现肺部感染,2例出现气胸,给予抗感染,胸腔闭式引流均匀恢复。1例心脏超声提示发现主动脉弓有缩窄,再次手术解除主动脉弓缩窄,痊愈出院。28例随访3个月~5 a,行心脏超声、胸片检查。患儿心功能良好,吻合口血流速度正常。结论对心下型完全性肺静脉异位引流新生儿实施左房后壁心内吻合手术,吻合口充分,有利于提高术后恢复效果。     

完全型肺静脉异位引流的诊断和外科矫正

目的总结完全型肺静脉异位引流(TAPVD)的诊断和外科治疗经验.方法回顾近16年收治TAPVD17例,其中男10例,女7例;年龄2～37岁.心上型11例,心内型6例.心上型者经右、左心房联合切口矫正,心内型者切除肺静脉共干与左心房之间的组织,补片修补房缺.同时矫正合并畸形.结果超声检查误诊4例,其中3例为心内型,外院曾误诊为纵隔肿瘤1例.造影检查除3例心内型者均明确诊断.2例术中始发现合并的动脉导管未闭(PDA),1例心上型者再次手术矫正.1例术后死于呼吸衰竭.16例随访1.5～17.5年,心功能良好.结论超声心动检查是诊断本病的主要手段,但有时需行心血管造影,甚至经术中探查方可确诊.本病确诊后应尽早手术,术中应常规探查和处理PDA,及早切开肺静脉共干引流,同时完全矫正心内畸形.手术成功率较高,远期效果良好.     

Radical correction of paracardiac type IIb of total anomalous pulmonary venous connection using pedicled flaps of right atrium and atrial septum

Surgical results of total anomalous pulmonary venous connection (TAPVC) has been improved in recent years, however, late development of pulmonary venous obstruction was our concern in its total correction in early infancy. In the cardiac type of TAPVC, in which the pulmonary veins were connected to the right lateral wall, prosthetic patch is conventionally used in diversion of pulmonary venous flow into left atrium. It seemed favorable to repair this subset without using prosthesis. A 3-month-old female with TAPVCIIb according to Darling's classification underwent total correction on September 22th, 1988. Two pedicled flaps were developed using the right atrial wall and the atrial septum to create a pulmonary venous channel to divert arterial blood into left atrium and absorbable sutures were used throughout. Right atrium was entered through a vertical incision in its body and all the pulmonary veins were found in a recess in the lateral wall of the right atrium. Atrial septal defect in the cranial aspect of the fossa ovalis was enlarged by cutting the primum tissue along the right limbus and its caudal margin so as to form a pedicled flap attached to the left limbic tissue. Then the flap was sutured along the limbus to create a roof of the fossa ovalis. The second flap was made in the middle of the lateral atrial wall and was used to create a tunnel from the recess to the atrial septal defect. The defect in the right atrial wall was closed directly and no prosthetic patch was used. Postoperative course was uneventful and echocardiogram showed wide pulmonary venous channel draining into the left atrium.     

Total Anomalous Pulmonary Venous Connection with a Ductus Arteriosus Aneurysm in a Neonate: Report of a Case

We performed successful emergency surgery on a 1-day-old infant to repair a total anomalous pulmonary venous connection (TAPVC) and a ductus arteriosus aneurysm (DAA) diagnosed by two-dimensional echocardiography. We anastomosed the common pulmonary venous chamber to the left atrium and ligated the pulmonary end of the DAA. Echocardiography and cardiac catheterization done 3 months later showed that the DAA had regressed. To the best of our knowledge, this is the first report describing the successful repair of a supracardiac TAPVC with a DAA.     

Technical modifications for improved results in total anomalous pulmonary venous drainage.

To delineate factors that contribute to improved surgical outcome in patients with total anomalous pulmonary venous drainage, we reviewed the records of 52 consecutive patients. Venous drainage was supracardiac in 25 (48%), cardiac in 12 (23%), infracardiac in 10 (19%), and mixed in five (10%). Preoperative pulmonary venous obstruction was present in 18 patients (35%). Median age at the time of repair was 35 days and weight, 3.7 kg. Repair was performed with deep hypothermia, low-flow cardiopulmonary bypass, and occasional short periods of circulatory arrest. In patients with coronary sinus drainage, the veins were tunneled to the left atrium through an enlarged atrial septal defect, with a mortality of 8% (1/12) and no postoperative stenosis. The approach in patients with supracardiac, infracardiac, and mixed drainage varied with time. In 16 patients, the condition was managed by apical or right-sided exposure of the common vein, anastomosis of the common vein to the left atrium with continuous sutures, and primary closure of the atrial septal defect (type I repair). In the other 24 patients the common vein was approached from the right side through the right atrium and the interatrial septum. Common vein-left atrium anastomosis was performed with interrupted sutures and a piece of pericardium used to augment the anastomosis, prevent common vein distortion, and close the atrial septal defect (type II repair). Mortality in type I repair was 25% (4/16) and in type II repair, 4% (1/24). Follow-up was 7.86 +/- 4.0 years with no late deaths. Postoperative stenosis occurred in five of 14 (36%) patients who had type I repair versus two of 23 (9%) who had type II repair. Multivariate analysis showed that type I repair was a positive risk factor for hospital mortality (p = 0.05) and restenosis (p = 0.04). The technique of transatrial exposure of the common venous chamber, interrupted suturing of the common vein to the left atrium, and pericardial patch augmentation significantly improves survival and decreases risk of restenosis.     

Modified septosuperior approach for the repair of supracardiac total anomalous pulmonary venous return in infants

An alternative technique for the repair of supracardiac total anomalous pulmonary venous return (TAPVR) is described. The pulmonary venous confluence (CPV) is identified below the right pulmonary artery between the aorta and superior vena cava. The atrial incisions are similar to a septosuperior approach of the mitral valve, modifying the incision on the roof of the left atrium to be parallel to the incision in the CPV. The common pulmonary vein is anastomosed to the roof of the left atrium. This approach offers optimal exposure for the repair without distorting the cardiac structures.     

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.     

Total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC) was repaired by operation in 20 infants during a 101/2-year period (1972 to 1983). Five patients died following operation. Factors that most affected mortality were the condition of the patient prior to repair, the year of operation, and the technique used for repair. Operative mortality before 1976 was significantly different from that after 1976 (57% [4/7] versus 8% [1/13], respectively; p less than 0.04). Prior to 1976, the several techniques used for anastomosis of the left atrium to the common pulmonary vein involved displacement of the heart from its anatomical position. After 1976, a standard approach using a right atriotomy for access was adopted for all such repairs. Intracardiac type of TAPVC was repaired by pericardial patch to direct blood flow through the atrial septal defect to the left atrium. Supracardiac and infracardiac types were repaired by enlarging the atrial septal defect so that a transverse incision through the back of the left atrium was exactly overlying the pulmonary vein posteriorly. A large anastomosis of the left atrium and common pulmonary vein was made with the heart in its natural anatomical position, which eliminates the possibility of distortion of the anastomosis. A pericardial patch was used to close the atrial septal defect. This experience suggests that the right transatrial approach of creating an anatomically correct anastomosis of the left atrium to the common pulmonary vein is an important factor in reducing operative mortality in patients with TAPVC.     

Total anomalous pulmonary venous connection to the portal and splenic vein associated with unilateral hypoplasia of pulmonary veins

Total anomalous pulmonary venous return (TAPVR) represents a rare congenital anomaly with wide anatomical and physiological variability. We report a case of a newborn with a challenging form of obstructed infracardiac TAPVR, in whom left and right pulmonary veins drained separately into the portal system. The right pulmonary venous sinus connected to the left branch of the portal vein, whereas the left venous sinus connected to the splenic vein. Surgical repair consisted of the creation of a common retrocardiac venous trunk which was anastomosed to the left atrium. The postoperative course was characterized by persisting congestion of the right lung. Two months later, right pulmonary vein hypoplasia was successfully enlarged with autologous pericardium.     

Modified anastomosis for repair of supracardiac total anomalous pulmonary venous connection in infants

Recurrent pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) is usually restricted to the anastomosis between the pulmonary venous confluence and the left atrium. We describe a modified technique for repair of supracardiac TAPVC in infants. An L-shaped incision of left atrium is utilized and the right-sided anastomosis is enlarged by using autologous pericardium to create a large and tension-free anastomosis.     

Novel repair for obstructed total anomalous pulmonary venous connection to coronary sinus

A 4-month-old infant underwent surgery for total anomalous pulmonary venous connection to the coronary sinus with obstructions both at the entry of the common chamber into the coronary sinus and at the patent foramen ovale. Using a novel technique, the right atrium was opened with a transverse incision. The coronary sinus was de-roofed. The coronary sinus-common chamber junction was split open into the common chamber. The incision extended up to the drainage of the right-sided pulmonary veins into the venous chamber. The common chamber was then anastomosed to left atrium thereby establishing an unobstructed pathway. The atrial septal defect was closed with a pericardial patch.