Surgical palliation of cardiac malformations associated with right isomerism

Between 1985 and 1993, palliative surgery was performed on 13 pediatric patients who had complex cardiovascular anomalies associated with right isomerism. The patients included two neonates, ten infants, and one child who were divided into two groups according to whether or not a total anomalous pulmonary venous connection (TAPVC) was present. Group 1 consisted of six patients with TAPVC and group 2 consisted of seven patients without TAPVC. In group 1, the surgical procedures involved TAPVC repair alone in two patients, combined TAPVC repair with a modified Blalock-Taussig shunt in two, combined TAPVC repair with pulmonary artery banding in one, and a modified Blalock-Taussig shunt alone in one. There were five hospital deaths and one late death in this group: pulmonary venous obstruction in two patients, perioperative myocardial failure in the two neonates, and congestive heart failure caused by increased pulmonary blood flow in two patients. In group 2, all the patients underwent systemic-pulmonary artery shunts, and there was one hospital death and three late deaths, the causes of which were unknown in two patients, and shunt failure and pneumonia in one patient each. These results suggest that surgical palliation for right isomerism produces poor results in young infants with obstructed TAPVC. Thus, we conclude that TAPVC repair should be performed without delay if pulmonary venous obstruction has been diagnosed clinically. Resolving pulmonary venous obstruction without cardiopulmonary bypass (CPB) may be preferable for infants, considering their difficult management. The systemic-pulmonary artery shunt should be of the low-calibrated type, especially if common atrioventricular valve regurgitation exists. If infants survive the surgery, they must be carefully followed up for a long period due to the risk of sudden death or infection.     

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。     

Totally anomalous pulmonary venous connection directly to the superior caval vein.

OBJECTIVE: Totally anomalous pulmonary venous connection directly to the superior caval vein is unusual. It is frequently associated with major congenital heart defects such as the syndrome of right isomerism. While improved results have been reported recently for the isolated form, complex cases are still associated with a higher mortality. METHODS: We undertook surgical correction in nine patients with direct pulmonary venous connection to the superior caval vein investigated in our institution from 1991 to 1999. In four of these patients, the venous anomaly was an isolated finding, while five patients with isomeric right atrial appendages had associated cardiac malformations rendering them unsuitable for biventricular repair. In one patient with an isolated form, the venous drainage was obstructed. Two patients with isomerism had previously undergone construction of an aortopulmonary shunt. Other associated surgical procedures in the patients with right isomerism were establishment of bidirectional cavo-pulmonary anastomoses in four cases and banding of the pulmonary trunk in one. RESULTS: There were neither early nor late deaths. Reoperation was needed in one patient because of pulmonary venous stenosis. In the five patients with right isomerism, two later underwent successful creation of the Fontan circulation. CONCLUSION: It is unusual to find direct drainage of all the pulmonary veins to the superior caval vein. When seen, the venous pathway is only rarely obstructed. For this reason, when associated with right isomerism, an aortopulmonary shunt should be constructed as initial palliation, with later repair of the anomalous pulmonary venous drainage at the time of construction of a bidirectional cavo-pulmonary anastomosis. When using this policy, the surgical results can be as good for the complicated variant as for the isolated form.     

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.     

Right heart bypass operation of complex cardiac anomalies in 4 patients with viscero-atrial heterotaxic syndrome

Four patients with viscero-atrial heterotaxic syndrome underwent a right heart bypass operation. 2 patients had right isomerism, and two had left isomerism. 3 patients had common atrio-ventricular canal, three had double outlet right ventricle, one had mitral valve hypoplasia, and one had double inlet right ventricle. All patients had anomalous systemic or pulmonary venous drainage. Redirection of systemic venous drainage was accomplished by total cavo-pulmonary shunt (TCPS) (2 patients), atrial baffle (1 patient), and intraatrial conduit (1 patient). In one patient underwent TCPS, regurgitant common atrioventricular valve was replaced with a prosthetic valve. One who had total anomalous pulmonary venous drainage (type Ib) died after cardiac repair. The three survivors are in improved condition. The right heart bypass operation, like total cavopulmonary shunt or connection, simplifies the redirection of systemic venous drainage and offers improved results in the surgical treatment of complex cardiovascular anomalies with viscero-atrial heterotaxic syndrome.     

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.     

Right and left isomerism: the cardiac surgeon's view

We have treated by palliative or corrective surgery 12 patients with the complex cardiovascular anomalies associated with right isomerism (asplenia syndrome) or left isomerism (polysplenia syndrome). A systemic-pulmonary artery shunt was performed in 4 patients, with 2 early deaths. Anatomical intracardiac repair was successfully done in 2 patients, while an orthoterminal repair using a modified Fontan procedure was attempted in 5 patients, only 1 of whom survived. A 1-year-old girl with diffuse pulmonary arteriovenous fistulas associated with left isomerism was not considered a good candidate for operation after an exploratory sternotomy. The surgical options and technical problems related to the complex intracardiac morphology of these developmental syndromes are presented.     

Simultaneous complex single ventricle palliation and tracheoplasty for heterotaxy syndrome

Although isomerism of the bronchial tree is an integral part of hetrotaxy syndrome, the association of congenital tracheal stenosis is rare in this group of disorders, and it has not yet been thoroughly described in the literature. This condition is potentially life-threatening and precludes single ventricle palliation. This report presents the case of a 5-month old infant with symptomatic congenital tracheal stenosis, functionally univentricular heart and extracardiac total anomalous pulmonary venous connection (TAPVC). The condition was successfully treated with bidirectional Glenn anastomosis, central pulmonary artery plasty, repair of TAPVC and slide tracheoplasty.     

Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes.

BACKGROUND: The historic outcome of the Fontan procedure in children with single ventricle and heterotaxy syndrome has been poor, and in the current era it has been incompletely described. METHODS: From January 1993 through April 2000, 30 patients (age range, 5.3 +/- 3.6 years) had total cavopulmonary connection for single ventricle and isomerism and heterotaxy syndrome. Right atrial isomerism and heterotaxy (n = 19) or left atrial isomerism and heterotaxy (n = 11) were associated with a morphologic right ventricle (n = 26), a common atrioventricular valve (n = 25), pulmonary atresia (n = 9) or stenosis (n = 17), anomalous pulmonary venous drainage (partial, n = 7; total, n = 11; obstructed, n = 4), and interrupted inferior vena cava (n = 13). A previous systemic to pulmonary artery shunt had been performed in 21 patients, and 3 patients had previous pulmonary artery banding. The Fontan procedure was staged in all but 3 patients at a median age of 12 months (range, 3 to 114 months). Before the Fontan procedure, atrial arrhythmia was present in 7 patients (23%). RESULTS: An extracardiac conduit was constructed in 22 patients (median diameter, 20 mm; range, 16 to 27 mm), and a lateral tunnel was constructed in 4 patients. Associated procedures included pulmonary artery augmentation (n = 17), repair of anomalous pulmonary venous drainage (n = 7), and repair of a regurgitant atrioventricular valve (n = 2). The mean cardiopulmonary bypass time was 134 +/- 57 minutes. Cardioplegic cardiac arrest was used in 12 patients for a mean duration of 62 +/- 28 minutes. There were four hospital deaths (13%), three that occurred in children having concomitant repair of anomalous pulmonary venous drainage (previously undetected in one). Thirteen children (41%) developed early postoperative atrial arrhythmias, 11 of the 13 children required temporary pacing. There was one late death. Follow-up (3.4 +/- 2.7 years) was available on 24 of 25 survivors. All but 2 patients were in sinus rhythm. CONCLUSIONS: Early and midterm outcomes after the Fontan procedure in this patient group improved and may be further ameliorated by detecting and repairing associated obstruction to pulmonary venous flow and performing atrioventricular valvuloplasty before the Fontan procedure.     

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.     

Congenital superior vena cava (SVC) stenosis and obstructed supracardiac total anomalous pulmonary venous connection (TAPVC)—A surgical perspective

Congenital superior vena cava (SVC) stenosis is a very rare anomaly, especially in pediatric population. Coexistence with obstructed supracardiac total anomalous pulmonary venous connection (TAPVC) has never been reported. Clinical examination should prompt detailed and focused evaluation for this treatable etiology. Pericardial patch augmentation can cure SVC stenosis, and may allow for growth potential as well. We describe a case of congenital SVC stenosis in a case of obstructed supracardiac TAPVC in a 3-month-old infant, managed successfully.     

Outcome of primary repair of infracardiac total anomalous pulmonary venous connection using a right-sided approach to the left atrium

Abstract Objective: Pulmonary venous obstruction (PVO), the major postoperative complication in patients with infracardiac total anomalous venous connection (TAPVC), compromises the surgical outcomes of TAPVC repair. Here, we report our experience using a right‐sided approach to the left atrium to repair this anomaly variant. Method: Eleven patients with infracardiac TAPVC underwent this surgical modification from September 2005 to December 2009. After a medium sternotomy, bicaval venous cannulation was performed for cardiopulmonary bypass (CPB). By adequate exposure of the surgical field, incision of the left atrium was located and anastomosed to the corresponding incision in the pulmonary venous confluence through the right side. Medical records consisting of preoperative and postoperative data were retrospectively reviewed to analyze the efficiency of this strategy. Results: Total correction was achieved in all 11 patients and there were no operative deaths. Postoperative low cardiac output persisted in six patients (54.5%), including five patients with preoperative PVO. No late deaths were noted in a mean follow‐up of 26.7 months (range, 1 to 51 months). Both residual atrial shunt and severe anastomotic obstruction were excluded by transthoracic echocardiography. Moderate residual obstruction was confirmed in one patient. At the end of the follow‐up, all patients had normal biventricular function and were in New York Heart Association (NYHA) Functional Class I. Conclusion: The right‐sided approach for repair of infracardiac TAPVC contributes to produce favorable outcomes. This modified technique is useful for enhancing exposure during surgical repair and providing adequate patent anastomosis. (J Card Surg 2011;26:102‐106)     

Atrial isomerism: a surgical experience.

Most publications on atrial isomerism are autopsy or case reports. The authors review 41 consecutive children operated on from 1980 through to 1996 with emphasis on associated cardiac anomalies, surgical procedures and outcome. Left atrial isomerism was present in 23 patients. Interruption of the inferior vena cava (56%), atrio-ventricular septal defect (47%), common atrium (38%) and cor triatriatum sinistrum (30%) were the most common diagnoses. Biventricular repair was achieved in 17 children and total cavo-pulmonary connection in two. Three underwent staged palliation: modified Blalock-Taussig shunt for two and bidirectional Glenn anastomosis for one. The remainder received a cardiac pacemaker. One patient died early after repair. Two underwent reoperation to correct a regurgitant left atrio-ventricular valve: one of these, in another hospital, had peroperative death. Three died later. Actuarial survival rate after repair and total cavo-pulmonary connection that was stabilized after 2 years was 84%. In the 18 children with right atrial isomerism, pulmonary atresia or stenosis predominated (89%) with discordant ventriculo-arterial connection (72%), atrio-ventricular septal defect (72%), 'single' ventricle (55%) and extracardiac total anomalous pulmonary venous drainage (50%). Biventricular repair was achieved in two patients and complete Fontan circulation in eight. The other eight underwent various staged palliative procedures and correction of extracardiac total anomalous pulmonary venous drainage. Five patients died postoperatively: two in our unit after modified Blalock-Taussig shunt and total cavo-pulmonary connection, three in other hospitals after repair (n = 1) and Fontan (n = 2). Five died later. One was lost for review. Survival after repair and Fontan stabilized after 6 months at 49%. In conclusion, the cardiovascular malformations associated with left atrial isomerism can often be successfully corrected. Those accompanying right atrial isomerism usually preclude a biventricular repair, require staged palliation and carry a poor prognosis.     

Tetralogy of Fallot: selective staged management

One hundred twenty-four patients with tetralogy of Fallot have undergone either primary total repair (61), shunt and later repair (30), or an initial shunt (33). The mean ratio of pulmonary anulus to descending thoracic aorta increased from 0.80 +/- 0.25 before the shunt to 1.22 +/- 0.26 before the repair (p less than 0.0001). The mean ratio in the primary repair group was 1.23 +/- 0.25. A transannular patch was necessary in only six of 91 patients (6.6%). Postrepair right ventricular/left ventricular pressure ratio averaged 0.50 +/- 0.11 in the shunt plus repair group and 0.43 +/- 0.12 in the primary repair group. Only four patients had a right ventricular/left ventricular pressure ratio less than 0.65. A significant inverse linear relationship existed between this ratio and the pulmonary anulus size measured at operation and normalized for the patient's height (p less than 0.01). Postoperative complications occurred in 21% of patients after a shunt and 20% of patients after open heart repair. The early mortality was 0.8% (1/124). An initial shunt in patients with a small pulmonary anulus can result in an increased anulus size and better hemodynamic result with frequent avoidance of a transannular patch. Staged repair may result in improved overall mortality rates.     

Staged operation for right isomerism with total anomalous pulmonary venous return: report of a case

A male infant with right isomerism, single ventricle, severe pulmonary stenosis (PS), and total anomalous pulmonary venous return (TAPVR) underwent successful staged Fontan operation. Pulmonary vein drained into right superior vena cava without a stenotic lesion. Common pulmonary venous chamber was also connected with the atrium by a thin bridging vein. Blalock-Taussig shunt was performed at 2-month-old. Because of progressive pulmonary congestion, an anastomosis between common pulmonary venous chamber and the atrium followed 4 days after the first shunt. Bidirectional cavopulmonary shunt was performed 7 months after the second operation. Because of progressive atrioventricular valve regurgitation, repeated bandings of the shunt were required to regulate the pulmonary flow. Total cavopulmonary connection was completed 9 months after the BCPS. Staged operation is a useful strategy to acquire an appropriate pulmonary blood flow for the isomeric heart with TAPVR and severe PS.     

Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions.

OBJECTIVES: To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique. METHODS: Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.2%) presented associated anomalies other than PDA. Twenty-eight patients (31.5%) were emergencies, due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included the double-patch technique with left atrial enlargement in 29 patients and side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium in 29 patients. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Total follow-up was 727.16 patient-years (mean 8.55 years, 98.8% complete). RESULTS: Early mortality was 7.86% (7/89). Ten patients (11.2%) underwent reintervention, including reoperation (eight), balloon dilation (one) and intraoperative stents placement (one), for anastomotic (four) or diffuse PV stenosis (six), with four late deaths. Kaplan-Meier survival is 87.3+/-0.036 SE% at 18.07 years with no difference according to anatomic type or surgical technique. Freedom from PV reintervention for operative survivors is 86.7+/-0.052 SE% at 18.07 years. Cox proportional hazard indicates associated anomalies (P=0.008) and reoperation for intrinsic PV stenosis (P=0.034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P=0.022), while the double-patch technique increased the risk of late arrhythmias (P=0.005). CONCLUSIONS: Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse outcome.     

38例完全性肺静脉异位引流的外科治疗

目的：总结３８例完全性肺静脉异位引流的外科治疗经验。资料和方法：３８例中男１７例,妇女１例。年龄２个月￣２２岁,３岁以内婴幼儿１２例。体重４．５￣３６．０ｋｇ。心上型２０例、心内型１５例、混合型３例。全组均在中度低温体外循环下将完全性异位引流的肺静脉直接或通过房间隔缺损隔入左房。结果：手术死亡３例（７．９％）,其中２例术后严重心律失常、１例多次缝合止血致吻合口狭小。术后并发心律失常１２例、呼吸道感     

Annuloplastic reconstruction for common atrioventricular valvular regurgitation in right isomerism

Two patients who had common atrioventricular valvular regurgitation associated with right isomerism, univentricular heart of the right ventricular type, transposition of the great arteries, pulmonary stenosis, and both systemic and pulmonary venous anomalies underwent common atrioventricular valvular annuloplasty. They also underwent bilateral, bidirectional cavopulmonary shunt. A Carpentier's ring was used in patient 1 and a polytetrafluoroethylene tube was used in patient 2 to reduce the diameter of the common atrioventricular annuli. Postoperative catheterization confirmed complete elimination of regurgitation of the common atrioventricular valve in both patients.     

Bidirectional glenn operation in infancy

The ideal age for bidirectional Glenn shunt (BDGS) as the first stage of staged Fontan is still not clear. Because of the concerns regarding relatively high pulmonary vascular resistance during infancy, many centres would bridge through a systemic to pulmonary artery shunt in this age group. Patients and Methods We did a retrospective analysis of 28 infants who had undergone bidirectional Glenn shunt at our institute from February 200. Results The mean age was 5 months (2.5–11) and the mean weight was 6.5 Kg (3.4–8.7). Boys dominated the group (25∶3). 7 infants had previous procedures. In 3 patients, BDGS was done as a salvage procedure. Formal Cardiopulmonary bypass (CPB) was used in all but 4 patients, in whom a right heart bypass was used. Superior Vena Cava (SVC) or innominate vein was cannulated in 12 patients and the rest were managed with temporary occlusion of SVC under deep hypothermic low flow bypass. 9 infants had bilateral BDGS. The main pulmonary artery was interrupted in 12 and atrial septectomy was done in 10 patients. Additional procedures with BDGS included Patent Ductus Arterious (PDA) interruption, Blalock Taussig (BT) shunt interruption, Left pulmonary arterioplasty, Stansel procedure and redo TAPVC repair. The mean SVC pressure post operatively was 14 (10–24) and only 2 patients needed pulmonary vasodilators in the post-oprative period. There is only one mortality in this series and the duration of chest tube drainage and Intensive Care Unit (ICU) stay is comparable with the older age group. Conclusion BDGS can be performed safely in infants more than 2 months of age electively or as a salvage procedure. It helps to avoid one step in the form of aortopulmonary shunt and hence the ventricular volume overload associated with it. Further studies are required to establish the growth potential of pulmonary arteries following an early BDGS. Presented at the 50th annual meeting of IACTS, New Delhi, Feb. 2004.     

Repair of subdiaphragmatic total anomalous pulmonary venous connection without cardiopulmonary bypass

Two neonates with asplenia syndrome, subdiaphragmatic total anomalous pulmonary venous connection, and pulmonary stenosis underwent a palliative operation without cardiopulmonary bypass. With the use of a side-biting clamp, and anastomosis was created between the pulmonary venous confluence and the right atrium. (Since children with asplenia syndrome have common mixing lesions, pulmonary venous drainage to the right atrium is not physiologically detrimental.) The descending vertical vein was ligated. A systemic-pulmonary shunt was performed. Both infants were discharged from the hospital less than 1 week after the operation. Both infants subsequently died, at 4 months and at 16 months of age. At autopsy, pulmonary venous drainage was unobstructed, with a surgical orifice 86% to 90% of the aortic anulus. We conclude that, in infants with asplenia syndrome and obstructed total anomalous pulmonary venous drainage, relief of pulmonary venous obstruction can be accomplished without cardiopulmonary bypass.