Surgical treatment of total anomalous pulmonary venous connection Darling type Ib using pedicled right atrial flap]

A one-month-old baby with total anomalous pulmonary venous connection (TAPVC) type Ib underwent a total correction with a pedicled right atrial (RA) flap, which was made by incising the RA wall in a quadrangular configuration. The common pulmonary vein (PV) was cut back into the left atrium (LA). Then the RA flap was sutured along the limbus of PV recess and atrial septal defect (ASD) to create a new pulmonary venous channel. The defect in the RA wall was directly closed without any prosthetic patch. Absorbable sutures (# 6-0 PDS) were used throughout. Postoperative course was uneventful and echocardiogram showed widely opened PV channel draining into the LA. We think that this procedure could be applied in various types of total and partial anomalous pulmonary venous connection, avoiding pulmonary venous obstruction on the assumption that the RA flap should grow.     

Surgical repair of inferior sinus venosus atrial septal defect.

A 9-year-old girl presented for cardiac evaluation with symptoms of dyspena, fatigue, and cyanosis with exercise. Cardiac catheterization demonstrated an atrial septal defect; an anomalous right superior pulmonary vein was suspected but not confirmed. Operation disclosed anomalous drainage of the right superior and inferior pulmonary veins into the right atrium, an intact fossa ovalis, and an inferior sinus venosus defect. Repair was accomplished by detaching the posterior edge of the atrial septum and suturing it to the right of the pulmonary veins, so that the defect was closed and all the pulmonary venous blood was directed to the left atrium.     

Surgical repair of an unusual type of supra-cardiac total anomalous pulmonary venous connection to the superior vena cava

Anomalies of the pulmonary venous drainage vary widely in their anatomic spectrum and clinical presentation. We describe an unusual case of supra-cardiac total anomalous pulmonary venous connection (TAPVC), where the pulmonary veins drained directly in the posterior aspect of proximal right superior vena cava (SVC) through separate ostia. The veins were re-routed with a patch to the left atrium via the secundum atrial septal defect (ASD). The continuity between distal SVC and right atrium was re-established by re-implanting the SVC to the right atrial appendage (Warden Procedure).     

Bilateral partial anomalous pulmonary venous connection; report of a case

We have experienced a case of bilateral partial anomalous pulmonary venous connection with a fossa ovalis type of atrial septal defect and pulmonary stenosis. The right upper pulmonary vein returned to the superior vena cava and the left upper pulmonary vein returned to the left innominate vein via the vertical vein. The atrial septal defect was enlarged and the right upper pulmonary vein was baffled into the left atrium with an equine pericardial patch. The left upper pulmonary vein was divided and anastomosed to the left atrial appendage. Pulmonary commissurotomy was also done for concomitant pulmonary stenosis. Postoperative course of the patient was excellent with constantly normal sinus rhythm. Angiography 2 weeks after operation showed no evidence of pulmonary venous obstructions on both sides.     

A case report of total anomalous pulmonary venous drainage corrected with Vargas' method

A 50-day-old infant with Darling's type Ib of total anomalous pulmonary venous drainage (TAPVD) was operated on with the Vargas' method. Pulmonary veins drained separately into the right superior vena cava (SVC). A J-shaped right atriotomy was performed according to the Vargas' method. The posterior flap was sutured to the anterior border of a previously enlarged atrial septal defect, directing the pulmonary blood flow toward the left atrium. The right SVC was divided just above the site of drainage of pulmonary veins, the proximal end of the right SVC was closed, and the anastomosis between the distal end of the right SVC and the previously opened right atrial appendage was performed. However, pulmonary hypertension remained because of the restrictive orifices of pulmonary venous drainage into SVC, and then side-to-side anastomoses between pulmonary veins and left atrium had to be added. Eight months after the operation pulmonary hypertension progressed markedly, because the orifices of the anastomoses became severely stenotic. Re-operation was performed to create a large anastomosis between pulmonary veins and left atrium. The indication and the long-term prognosis of the Vargas' method were discussed.     

Cor triatriatum associated with large atrial septal defect and partial anomalous pulmonary venous return. Case report

A rare variant of cor triatriatum is presented with a large true atrial septal defect and partial anomalous pulmonary venous return into the right atrium. The correct diagnosis was made at the operation and abnormal left atrial septum was excised completely and a new interatrial septum was created with pericardial patch in such a position that the abnormally drained right upper pulmonary vein was left in the left atrium. It was thought to use the abnormal left atrial septum to close the atrial septal defect by excising only the right lateral border of this abnormal septum and resuturing it to the right atrial wall to close the true atrial septal defect. This thought could not be realized because of the small size of this abnormal septum and large size of the atrial septal defect. This technique can be realized in small or medium sized atrial septal defects associated with cor triatriatum.     

The Operative Problem of Small Left Atrium in Total Anomalous Pulmonary Venous Connection: Report of 5 Patients

A moderately small left atrium is a common finding in total anomalous pulmonary venous connection (TAPVC). In most patients in whom the common pulmonary vein is anastomosed to the left atrium, the small size of this chamber does not interfere with good operative results. Recently a patient was encountered in whom the left atrium measured less than 2 cm in its greatest dimension. This atrium was too small to take an anastomosis with the common pulmonary vein. Therefore the common pulmonary vein was anastomosed primarily to the right atrium, the floor of the fossa ovalis was excised, and a pericardial conduit was constructed. Subsequently, 4 additional patients have been similarly treated. The problem of small left atrium in TAPVC is discussed.     

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。     

Polysplenia with anomalous pulmonary venous drainage to right atrium caused by atrial septal displacement; report of a case

A 3-year-old girl with polysplenia, atrial septal defect (ASD), and partial anomalous pulmonary venous connection (PAPVC) was admitted to our unit to have total correction. Preoperative three-dimensional computed tomography (3D-CT) and transesophageal echo (TEE) showed that the pulmonary veins were connected with what normally constitutes the posterior wall of the left atrium, but right pulmonary veins became incorporated into the right atrium because of the atrial septal displacement. In the procedure, intracardiac inspection revealed absence of septum secondum and leftward deviation of septum primum. Deviated septum primum was incised at the posterior edge and shifted rightward to incorporate right pulmonary veins into the left atrium. ASD was closed using autologous pericardial patch. This type of PAPVC was rare and preoperative 3D-CT and TEE was quite useful in evaluating unique anatomical characteristics.     

Total cavo-pulmonary connection without foreign material for asplenic heart associated with partial anomalous pulmonary venous connection

The presented case was a 3-year-old boy diagnosed with asplenia (SLL), double outlet right ventricle, pulmonary stenosis, atrioventricular septal defect, hypoplastic left ventricle and partial anomalous pulmonary venous connection to the superior vena cava. Partial anomalous pulmonary venous connection was repaired by translocation of pulmonary artery to avoid pulmonary venous obstruction when Glenn anastomosis was performed. Total cavo-pulmonary connection was established by re-routing the inferior vena cava to pulmonary artery using the atrial septal remnant and the left atrium free wall flap.     

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.     

Sinus Venosus Atrial Septal Defect Associated with Partial Anomalous Pulmonary Venous Drainage: Surgical Repair

This report describes a new technique for the repair of sinus venosus atrial septal defect associated with partial anomalous pulmonary venous drainage. A right atrial wall flap is used both to deflect the anomalous venous blood into the left atrium and to close the atrial septal defect. Then an atrioplasty is performed. This method does not employ any foreign materials, avoids injury to the sinoatrial node and internodal tracts, and minimizes the risk of obstruction of the ostia of the anomalous pulmonary veins and superior vena cava.     

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.     

Repair of two cases of partial anomalous pulmonary venous return by draining total right pulmonary venous blood to the left atrium through dilated atrial septal defect]

Two cases of partial anomalous pulmonary venous return were reported in whom right pulmonary veins were totally drained to the coronary sinus and to the right atrium respectively. Re-routing of pulmonary veins was done by dilated atrial septal defect and making intra-atrial tunnel using equine pericardial patch. Postoperative courses were uneventful. MRI was useful to identify the pathway from right pulmonary veins to the left atrium.     

Surgical repair of coronary sinus type partial anomalous pulmonary venous drainage with intact atrial septum

We experienced a rare form of PAPVD without atrial septal defect. The patient was a 33-year-old male and he was referred to our institute because of mild right pulmonary congestion detected by a routine chest X-P. Enlarged coronary sinus, right atrium and right ventricle were documented by UCG. The Qp/Qs was 1.9 and pulmonary artery pressure was 38/7 mmHg (mean: 17 mmHg) by cardiac catheterization. Selective pulmonary angiogram showed that all right pulmonary veins drained into the coronary sinus without evidence of an atrial septal defect. Enhanced chest CT clearly demonstrated the connection between the right pulmonary vein and the coronary sinus. Intracardiac repair without atrial baffle was carried out under hypothermic cardiopulmonary bypass. Under cardiac arrest with cardioplegia, the common wall between the right pulmonary vein and the left atrium was incised and the connection between the right pulmonary vein and the left atrium was established. The flap made by this incision was brough posterior to close the right pulmonary vein opening to the coronary sinus. The postoperative course was uneventful and the minimum diameter of the right pulmonary vein was found to be 15.5 mm by a postoperative pulmonary artery angiogram. This operative method without an atrial baffle could be an alternative procedure for coronary sinus type PAPVD.     

Partial anomalous pulmonary venous connection of singular form.

We have recently experienced a rare case of partial anomalous pulmonary venous connection, in a 3-year-old female. The case was diagnosed preoperatively as anomalous left superior pulmonary venous connection with atrial septal defect. Surgery, however, revealed that only the right superior pulmonary vein returned to the left atrium, while the other three pulmonary veins returned to the coronary sinus or innominate vein. No associated atrial septal defect was found. An enlarged coronary sinus was observed. In the present case, three of the four pulmonary veins were found to have anomalous connections, and were hemodynamically close to being total anomalous pulmonary venous connection. We performed unique radical surgery using autologous tissue for multiple anomalous pulmonary venous connections and here report the results, with a short discussion of the literature.     

Extracardiac atrial pedicle conduit repair of partial anomalous pulmonary venous connection to the superior vena cava in children

Between June, 1982, and July, 1983, 6 children with partial anomalous pulmonary venous connection to the middle or high segment of the superior vena cava (SVC) underwent repair of the anomaly by division of the SVC proximal to the site of entry of the anomalous pulmonary veins. Continuity between the cephalad end of the SVC and the right atrium was established by direct anastomosis to the right atrial (RA) appendage or by creation of a pedicle conduit of RA appendage, RA free wall, and pericardium. The anomalous pulmonary veins remained in situ on the lower segment of SVC, blood being directed to the left atrium through an atrial septal defect by a pericardial patch placed within the right atrium well away from the sinoatrial node, anomalous pulmonary veins, and cavoatrial junction. All children have survived, remain in normal sinus rhythm, and have no evidence of vena caval or pulmonary venous obstruction. Follow-up cardiac catheterizations, angiocardiograms, and Holter recordings support the efficacy of this technique as an alternative in the management of anomalous pulmonary veins joining the SVC well above the cavoatrial junction.     

Correction of Anomalous Subdiaphragmatic Inferior Vena Caval Drainage of the Right Pulmonary Veins (Scimitar Syndrome)

Correction of anomalous infradiaphragmatic inferior vena caval drainage of the right pulmonary veins can be repaired under conventional cardiopulmonary bypass by placing a circumferential tape below the diaphragm between the hepatic veins and the scimitar vein entrance. During repair, creation of an atrial septal defect in the distorted septal-left atrial junction of scimitar syndrome is facilitated by first incising the anteromedial aspect of the fossa ovalis to better delineate the optimal posterolateral margin. A case history illustrates the technique.     

A case of anomalous right pulmonary venous drainage to high segment of superior vena cava with intact atrial septum--surgical repair by cavoatrioplasty avoiding injury to the sinoatrial node and its feeding artery

Surgical repair of anomalous right pulmonary venous drainage to the high segment of the superior vena cava (SVC) with intact atrial septum in a 5-year-old girl by cavoatrioplasty avoiding injury to the sinoatrial (SA) node and its feeding artery is described. The procedure was composed of #1: an oblique incision at the anterior wall of SVC above the anomalous right pulmonary veins (ARPVs) and closure of the lower end of the SVC, making the lower segment of SVC the ARPV channel, #2: a fan-shaped incision at the base of the right atrial (RA) appendage and suturing the cephalad flap over a created atrial septal defect, diverting the ARPV flow through the SVC into the left atrium, and #3: reconstruction of continuity between the cephalad end of the SVC and the RA by suturing the RA appendage flap over the lower segment of SVC. Pulmonary valvotomy was also done for concomitant pulmonary stenosis. Postoperative course of the patient was excellent, with constant normal sinus rhythm. Angiography one month after operation showed no stenosis of the SVC and pulmonary venous pathways. The method reported here was considered to have following advantages: It employs simple and short incisions, avoids injury to the SA node its feeding artery, and achieves wide and nonobstructive pathways of both SVC and pulmonary venous return without using foreign material.     

Late complications following surgical repair of total anomalous pulmonary venous return below the diaphragm.

Between May, 1975, and June, 1977, we surgically repaired an infracardiac total anomalous pulmonary venous return in 4 infants under deep hypothermic cardiac arrest. All patients had pulmonary hypertension and a patent ductus arteriosus. All survived operation and required positive end-expiratory pressure with mechanical ventilation. Late complications included patent ductus arteriosus not visualized at initial catheterization, breakdown of an oversewn atrial septal defect repair, and pulmonary venous obstruction despite an anastomosis diamter of 1.5 to 2.5 cm. At reoperation, there was scarring and contraction at the junction of the pulmonary veins and left atrium. One infant with these complications died post-operatively. In another patient, there was kinking of the left lower pulmonary vein at its juncture with the left atrium. In view of these complications we currently recommend the following: ligation of the ductus arteriosus regardless of catheterization findings; a right-sided approach to avoid pulmonary vein kinking; prosthetic patch closure of the atrial septal defect to avoid excessive tension on suture lines and to maintain maximum left atrial size; stellate anastomosis to provide the largest possible drainage channel; awareness that an unusually high positive end-expiratory pressure may be required postoperatively; and early recatheterization.