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Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients.A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates.A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029).Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort.

Objective Conventional procedures for partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) still have serious complications, such as late SVC and/or pulmonary venous obstruction and supraventricular arrhythmia. We aimed to introduce our newly developed surgical technique with minimum right atriotomy and double-barreled arrangement of systemic and pulmonary venous channels (double-decker technique). Methods From 1998 to 2018, 21 consecutive patients with PAPVR to the SVC underwent this new procedure. The patients' median age and body weight were 4.4 years and 16.5 kg, respectively. Two female patients underwent lateral thoracotomy. Postoperative hemodynamics of both venous channels were assessed using time-resolved 3-dimensional magnetic resonance phase contrast imaging in 6 patients. Results The median follow-up period was 11.0 years. There was no early mortality and late death. No patient required reoperation and/or intervention. All patients maintained normal sinus rhythm, and supraventricular arrhythmia did not occur. The median blood flow velocity of the neo-SVC and neopulmonary venous channel was 0.40 and 0.30 m/s, respectively. Using time-resolved 3-dimensional magnetic resonance phase contrast imaging, the straight and nonrestrictive flow and low wall shear stress were visualized in both venous channels. Conclusions Our newly developed double-decker technique is a useful alternative surgical procedure for PAPVR to the SVC. Late complications can be completely avoided using this method. Growth potential of both channels is also maintained.

ObjectivesSinus node dysfunction is known as a major complication after repair of partial anomalous pulmonary venous connection. We retrospectively analyzed the results of the atrial wall flap technique compared with the results of patch repair or direct suturing in the intra-atrial tunnel technique.MethodsBetween 1991 and 2007, 23 patients (mean age, 6 years; range, 5 months–17 years) with partial anomalous pulmonary venous connection underwent surgical intervention. The right anomalous pulmonary veins drained to either the right atrium or superior vena cava in 8 and 15 patients, respectively. Patients were divided into 2 groups: group F (n = 14), who had repair with an atrial flap, and group N (n = 9), who had repair without an atrial flap. All patients had normal sinus rhythm preoperatively.ResultsNo patients had signs of superior vena cava or pulmonary venous obstruction within a mean follow-up of 4.8 years. One patient in group F required pacemaker implantation. In the early postoperative period, sinus node dysfunction developed in 93% of group F and 44% of group N patients (P < .01) and was prolonged until discharge in 57% of group F and 0% of group N patients (P < .01). At the most recent clinical visit, sinus node dysfunction was identified in 50% of group F patients, whereas all patients in group N had normal sinus rhythm (P < .02).ConclusionsThe atrial flap technique, which requires incision or suture crossing the crista terminalis, could cause sinus node dysfunction, whereas the intra-atrial rerouting method with a patch or direct suture maintains normal sinus node function postoperatively.

Background: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. Methods: Thirty-one patients (19.9 ± 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. Results: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. Conclusions: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues. (J Thorac Cardiovasc Surg 2000;119:340-6)

Ojective: The aim of this report is to review the surgical experience of a single institution with a relatively large series of patients with mixed total pulmonary venous drainage. Patient population: Between January 1, 1971, and December 31, 1994, 232 patients with total pulmonary venous drainage underwent surgical correction. Twenty of these patients (8.6%) had mixed type total pulmonary venous drainage. Ages at operation ranged from 1 day to 46 months, with a median of 2.3 months. Results: Both cardiac catheterization and echocardiography were performed before operation in 12 patients. Four patients underwent only cardiac catheterization, and another four patients underwent only echocardiography. The sensitivity and specificity for catheterization were 94% and 99%, respectively; they were 31% and 100%, respectively, for echocardiography. Severe pulmonary venous obstruction was present in three patients, all of whom underwent emergency operation. Three patients (15%), all of whom had preoperative pulmonary venous obstruction, died after operation. There were two late deaths, one of pulmonary vein stenosis and the other of probable pulmonary hypertension. The actuarial survival at 10 years was 73% for all patients; patients who survived the initial operation had a 10-year survival of 87%. Conclusion: The diagnosis of mixed total pulmonary venous drainage can be difficult to establish by echocardiography or at the time of operation. For patients in stable condition, cardiac catheterization may be considered if fewer than three pulmonary veins are identified by echocardiography. Pulmonary venous obstruction is relatively infrequent in this group of patients but when present impacts patient survival significantly. The long-term results with this lesion are excellent. (J Thorac Cardiovasc Surg 1996;112:1581-8)

Background: Thirty-two patients with scimitar syndrome were seen in the period between 1975 and 1995. There were 11 male and 21 female patients. Median age at diagnosis was 7 months (mean 7.7 years, range 1 day to 70 years). Patients in whom the diagnosis was made during the first year of life (infantile group, n = 19) had more severe symptoms and had a higher incidence of heart failure (11/19 vs 0/13) and of pulmonary hypertension (11/19 vs 1/13) than did the patients in whom the diagnosis was made after age 1 year (adult group, n = 13). In 17 patients the anomalous pulmonary venous drainage was repaired by baffling the vein to the left atrium. The median age at this operation was 5.8 years (mean 14.8 years, range 6 months to 70 years). Results: No deaths occurred in this surgical group during a mean follow-up period of 8.9 years (range 1.6 to 17 years). Eight patients (47%), however, had evidence of pulmonary venous stenosis after repair, and two required reoperation for pulmonary venous obstruction. All six children in the infantile group had postoperative pulmonary venous stenosis, compared with two of 11 older patients. Postoperative quantitative pulmonary perfusion scans performed in 15 patients demonstrated reduced flow to the right lung (24%, range 0% to 59%). Conclusion: We conclude that age at detection of scimitar syndrome is important in predicting outcome. Surgical repair seldom results in normal blood flow to the right lung but abolishes left-to-right shunt. Postoperative pulmonary venous obstruction is prevalent, especially in the infants. (J THORAC CARDIOVASC SURG 1996;112:1161-9)

left venous valve remnant as the incomplete partition may explain the morphogenesis of the midline chamber. Because of marked asymmetry in the timing and sequence of events for pulmonary venous development, a process of differential pulmonary venous segregation, which supports the primary theory, may explain right pulmonary vein malincorporation into the midline chamber. Although appreciation of the developmental basis would not have altered the surgical approach, recognition of precise anatomic disposition of this unusual anomaly is essential for effective planning of the appropriate corrective surgical strategy. Awareness of the exact nature of the pulmonary venous drainage is pivotal to the prevention of pulmonary venous obstruction and its ensuing adverse hemodynamic effects. References

To facilitate the preoperative diagnosis and surgical management of visceral heterotaxy and asplenia, 72 postmortem cases were reviewed with particular attention focused on the systemic and pulmonary venous connections. The superior vena cava was bilateral in 51 cases (71%), but in 9 cases one of the superior venae cavae was partly or totally atretic. Patent bilateral superior venae cavae were found in 42 cases (58%) and the superior vena cava was unilateral in 21 (29%). Although the inferior vena cava was never interrupted, a prominent azygos vein was found in 6 cases (8%). Some hepatic veins drained separately from the inferior vena cava in 20 cases (28%). An intact coronary sinus was rare (2 cases, 3%). Anomalous pulmonary venous connection to a systemic vein was total in 42 (58%) of 72 and partial in 2 (3%) of 72, with obstruction in 24 (55%) of 44. Abnormal pulmonary artery branches (severe hypoplasia, localized stenosis, or discontinuity) were present in 21 (29%), and these obstructive arterial anomalies were associated with a significantly higher prevalence of anomalous pulmonary venous connection (p < 0.01) and of pulmonary venous obstruction (p < 0.01). Cardiac pulmonary venous connections were found in 28 (39%), with the pulmonary veins and the inferior vena cava entering the same atrium in 10 (36%) of 28. (J T HORAC CARDIOVASC SURG 1995; 110: 641-50)

Objective: Pulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures. Methods: We conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed. Results: Of 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7%(95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction. Conclusions: Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur. (J Thorac Cardiovasc Surg 2013;145:1255-62)

From January 1985 through January 1993, 41 patients less than 1 year of age underwent operative correction of isolated total anomalous pulmonary venous connection. There were 24 boys and 17 girls. The median age at operation was 13 days (range 1 to 282 days) and weight was 3.6 kg (2.5 to 5.2 kg). Locations of the connections were supracardiac in 19, cardiac in 9, infracardiac in 11, and mixed supracardiac and cardiac in 2. Obstruction of the pulmonary veins was severe in 24, mild in 3, and absent in 14. Preoperative stabilization included mechanical ventilation for 15 patients for a mean duration of 2 1/2 days and extracorporeal membrane oxygenation for 1 patient for 1 day. All operations were performed with deep hypothermia and circulatory arrest (mean arrest time 34 minutes). Supracardiac connections were repaired by performing a side-to-side anastomosis between the pulmonary venous confluence and the dome of the left atrium through a superior approach between the superior vena cava and the aorta. Coronary sinus connections were repaired by enlarging the atrial septal defect and the coronary sinus communication with the left atrium and closing the atrial defect with a large patch. Infracardiac repairs included elevation and rotation of the heart to the right and an elongated side-to-side anastomosis between the common venous confluence and the left atrium. One patient died 1 week postoperatively of persistent pulmonary hypertension. Another patient, who was supported by extracorporeal membrane oxygenation before the operation, died 3 months after the operation as a consequence of pulmonary lymphangiectasia. All other patients are alive and well with a mean follow-up of 26 months (range 3 to 77 months). One patient required two subsequent reoperations for persistent pulmonary venous obstruction, and another patient had superior vena cava obstruction necessitating reoperation. Operative treatment of total anomalous pulmonary venous connection in infants can be performed with low mortality and an infrequent need for reoperations.

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. (J T horac C ardiovasc S urg 1992;103:861-71)

From November 1973 to January 1988, 15 patients with cor triatriatum underwent surgical correction at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. Their ages ranged from 15 days to 48 years. Eight patients (53%) were younger than age 1 year. Evidence of congestive heart failure was present in five infants, whereas three infants, three children, and two adults initially had signs of pulmonary venous obstruction, and two children had a heart murmur only. Five patients had cor triatriatum alone; in ten cases there also was an atrial septal defect Associated anomalies in four patients included left superior vena cava, ventricular septal defect and left superior vena cava, partial anomalous pulmonary venous connection, and bilateral partial anomalous pulmonary venous connection. Cor triatriatum was repaired with the aid of cardiopulmonary bypass in all patients. Excision of the membrane was accomplished with a right atrial approach in 13 patients and a left atriotomy in one patient Both atria were opened in one case. All associated anomalies were simultaneously corrected. Three patients (20%) died early after operation. Among the 12 survivors, no late events have occurred, and all of them are presently in New York Heart Association functional class I. A recent echocardiogram shows absence of residual obstruction or shunt and good development of the left cardiac chambers.

Among 57 neonates undergoing repair of total anomalous pulmonary venous return with severe pulmonary venous obstruction from 1980 through 1989, date of operation (1980 to 1984), preoperative hemodynamic instability, and failure to monitor pulmonary artery pressure postoperatively were risk factors for death. Thus, among the 30 patients having repair between 1985 and 1989, the 55-month survival rate including hospital deaths was 83%.

Background: Surgical correction of the sinus venosus syndrome has been associated with sinus node dysfunction and venous obstruction postoperatively. We present the long-term follow-up of a lateral transcaval approach, which closes the atrial communication and corrects the partial anomalous pulmonary venous connection to the superior vena cava with the use of a simple pericardial patch. Methods: The records of 66 patients undergoing repair between April 1981 and April 1997 were examined. Mean age at repair was 10.2 years (range, 1.5-65 years; median, 5 years). Six patients had a left superior vena cava, 4 had an additional atrial septal defect, and 2 had coronary artery bypass grafts. Immediate and long-term follow-up included physical examination, electrocardiography, transthoracic echocardiography, and use of a 24-hour ambulatory Holter monitor. Sinus node function, incidence of significant arrhythmia, and evidence of mechanical venous obstruction were assessed. Results: Follow-up data were available for 64 (97%) patients for a mean follow-up of 4.1 years (range, 1-9 years). There were no deaths. No evidence of residual atrial septal defect, superior vena cava, or venous obstruction were found by echocardiography. On electrocardiography all patients were in sinus rhythm, with no arrhythmia seen. Holter monitoring was performed at a mean of 7.3 years postoperatively. All patients had normal sinus node function, and no sustained atrial arrhythmia was seen. Conclusion: Transcaval repair is a simple technique that does not interfere with sinus node function. There is no evidence to suggest that this approach leads to venous obstruction. (J Thorac Cardiovasc Surg 2000;119:741-4)

The results of repair of total anomalous pulmonary venous connection (TAPVC) have dramatically improved over recent years) However, a higher mortality and morbidity still remain significant in patients less than 3 months of age who have right-sided hypertension, pulmonary venous obstruction, and low volume in the left side of the heart. 2" 3 Postoperative low cardiac output may persist because of a small and noncompliant left atrium and ventricle or episodes of reactive pulmonary hypertension. Several surgical techniques have been suggested to reconstruct an unrestrictive wide connection between the left atrium and pulmonary veins, as this connection will allow the left side of the heart time to adapt and maintain cardiac output . 4 Most authors suggest ligation or interruption of the vertical vein just above the diaphragm, although the authors of a past report 5 were concerned about the occurrence of acute liver necrosis immediately after the operation. We believe that leaving an adjustable suture during the immediate postoperative period may be useful for venting of the left ventricle, especially if low cardiac output persists despite the creation of an wide left atrium-pulmonary vein anastomosis. A 4-week-old infant weighing 2 kg was admitted because of increasing cyanosis, pulmonary edema, and hepatomegaly. Two-dimensional echocardiography with Doppler and color flow mapping study and cardiac catheterization demonstrated infracardiac type TAPVC with the portal system. The operation was performed with profound hypothermia and circulatory arrest (31 minutes). The repair was carried out by a wide anastomosis between the common pulmonary vein, with the incision being extended into the upper right pulmonary vein and the posterior left atrial wall.

ObjectiveSurgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome.MethodsOur cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990–2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0–4 years), and the median weight was 3.5 kg (range, 1.3–15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy.ResultsThere were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P

A quantitative analysis of small pulmonary arteries, pulmonary veins, and lymphatic vessels was conducted in autopsy cases of total anomalous pulmonary venous connection. The materials were obtained from 60 cases of total anomalous pulmonary venous connection without asplenia or pulmonary stenosis, ages ranging from 2 days to 19 months at the time of death (mean age 2.2 months). Pulmonary arterial pressure had been measured in 32 of these patients before death. Twenty cases of ventricular septal defect with pulmonary hypertension and 15 normal individuals were used as the control group. The mean thickness of the media of small pulmonary arteries and veins was 12.7 and 7.6 microns, respectively, in the total anomalous pulmonary venous connection cases, both values being significantly larger than those for normal and ventricular septal defect cases. No changes in thickness with aging were found. Medial thickness in the arteries and veins was greater in the cases of pulmonary venous obstruction than in those without such obstruction. The medial thickness of small pulmonary arteries in total anomalous pulmonary venous connection cases correlated with increased pulmonary arterial pressure. When the patients with the same pulmonary arterial pressure levels were compared, the medial thickness was always greater in those who had total anomalous pulmonary venous connection than in those who had ventricular septal defect. The medial thickness of pulmonary veins was also highly correlated with increased pulmonary arterial pressure in total anomalous pulmonary venous connection. The severity of the intimal lesions was milder in those who had total anomalous pulmonary venous connection than in those who had ventricular septal defect, suggesting the protective role of the thickened pulmonary arterial media against development of intimal lesions. Intimal fibrous thickening of pulmonary veins was not seen in the cases of ventricular septal defect, but it was present in 45% of the total anomalous pulmonary venous connection cases. Lymphangiectasia was characteristically present in 62% of the total anomalous pulmonary venous connection cases. Interstitial emphysema was often a complication of lymphangiectasia, and it led to eight postoperative deaths.

One hundred twenty patients with dextro-transposition of the great arteries (TGA) underwent intra-arterial baffle repair using thin Dacron fabric from 1971 to 1979. The ages of the patients ranged from 29 days to 17 years (mean age 28 months). Thirty-five patients had undergone 49 preliminary palliative operations. Early postoperative mortality was 4.8% for patients with simple TGA but was higher among patients with associated ventricular septal defect (26%), VSD and left ventricular outflow tract obstruction (28.5%) and intact ventricular septum with left ventricular outflow tract obstruction (12.5%). Early nonfatal postoperative complications included low cardiac output (23.3%), respiratory insufficiency (35.8%), junctional rhythm (34.1%), superior vena caval (SVC) obstruction (9.1%), and chylothorax (7.5%). Late postoperative mortality for all groups was 7.5% (nine patients). Late cardiac dysrhythmias occurred in 33 patients (40.7%). Normal sinus rhythm has been preserved in all patients since direct, high SVC cannulation was instituted. Late postoperative hemodynamic and angiographic evaluation in 61 patients revealed severe to total SVC obstruction in 12 patients (20.2%) and pulmonary venous obstruction in four patients (6.5%). Among the latter, two patients died following reoperation and another patient died without operation. SVC obstruction was clinically important in only three patients, two of whom have undergone successful reoperation. All other late survivors are acyanotic and clinically well. The Mustard operation has dramatically improved the survival rate and quality of life for patients with TGA. However, postoperative caval and pulmonary venous obstruction are problems which require additional technical modifications and stimulate the search for alternative corrective operations.

A case is presented in which a unique combination of events occurred as a complication of subclavian vein catheterization. Extravasation of intravenous hyperalimentation solution occurred, resulting in mediastinitis and venous obstruction of the jugulosubclavian confluence bilaterally. Bilateral chylothorax resulted which was successfully managed by conservative means. The methods used and the rationale for their employment are discussed.

Twenty-three infants, aged 5 days to 10 months, underwent repair of total anomalous pulmonary venous connection (TAPVC). Coronary sinus drainage was unusually common, accounting for 39 percent of the total. There were three early and four late deaths. Although the hospital deaths were related to perioperative errors, two of the infants appeared to have small left ventricles. Two late deaths in infants with coronary sinus drainage were due to obstruction to pulmonary venous return some distance proximal to the surgical anastomosis. All 16 survivors were well at last review, one after revision of his repair. Fifteen have undergone late reassessment, and one of these, also with coronary sinus drainage, showed evidence of pulmonary venous obstruction. Although the early survival rate is satisfactory, the late mortality rate has been significant and the high incidence of pulmonary venous obstruction in infants with coronary sinus drainage is of particular concern.

Partial anomalous pulmonary venous connection to the right side of the heart often complicates surgery for atrial septal defects. Between 1964 and 1987, 39 patients, ranging from 2 to 52 years old, underwent repair of partial anomalous pulmonary venous connection. At least one anomalous pulmonary vein arose from the right upper lobe in 38 patients and right middle lobe in 30 patients and connected to the superior vena cava in 28 patients and the right atrium only in 11 patients. An atrial septal defect was present in 32 patients (82%). Patients who had partial anomalous pulmonary venous connection to the superior vena cava-right atrium junction, the right atrium or both were treated by septal translocation (two patients) or patch redirection of the anomalous pulmonary venous flow to the left atrium through a native atrial septal defect (eight patients) or a surgically created atrial septal defect in two patients with intact atrial septum. For partial anomalous pulmonary venous connection to the high superior vena cava (27 patients), the superior vena cava was transected and oversewn above the anomalous veins. The anomalous pulmonary venous flow was redirected through the proximal superior vena cava into the left atrium across a sinus venous atrial septum defect (22 patients) or a surgically created atrial septal defect in five patients with intact atrial septum. The atrial septal defect was coapted to the intracardiac orifice of the superior vena cava, and the distal superior vena cava was anastomosed to the right atrial appendage. One 31-year-old woman with severe pulmonary hypertension died early and was the only death in the series. A technical error early in the series resulted in one symptomatic superior vena cava obstruction. Only one patient remains in sick sinus syndrome late. All patients remain well over long follow-up (1 to 24 years). Postoperative catheterization or echocardiography has revealed no intracardiac defects, pulmonary venous obstruction, or superior vena cava obstruction (except the one technical error). Correction of partial anomalous pulmonary venous connection should be individualized according to the site of connection of the anomalous pulmonary veins and the location of the atrial defect to minimize undesirable postoperative sequelae often associated with other methods of repair.

Twenty-six patients aged 6 days to 3 months (mean 57 days) underwent a Senning procedure for transposition of the great arteries. Twenty-two had intact ventricular septum and four had a small ventricular septal defect. They were followed up for 1 month to 8 years (mean 4 years). There were no late deaths. At late examination, 25 patients were asymptomatic and there was no clinical or echographic evidence of caval or pulmonary venous obstruction. Growth was normal in all but two patients. Neurologic assessment was abnormal in eight patients. The electrocardiogram showed sinus rhythm in 22 patients and asymptomatic arrhythmias in four. Twenty-three patients underwent cardiac catheterization and angiographic studies 2 to 72 months postoperatively (mean 15 months), which demonstrated effective left and right atrial contraction. An atrial shunt was noted in one patient and a ventricular shunt in one. Two infants (8%) had a residual left ventricular outflow tract obstruction (gradients of 26 and 37 mm Hg). Two had mild superior vena caval obstruction (gradients of 4 and 5 mm Hg). We conclude that the Senning procedure can be performed in early infancy with good results and a low incidence of late complications.

Since February, 1978, 42 infants ranging in age from 15 days to 16 months (mean age 6 months) and weighing between 2.0 and 9.0 kg (mean weight 5.8 kg) underwent a modified Senning I operation. Eleven (26%) underwent operation during the first 3 months of life. Twenty-nine patients had dextro-transposition of the great arteries (d-TGA) and an intact ventricular septum (Group I), and 13 patients had d-TGA and a large ventricular septal defect (VSD) (Group 2). In addition to the Senning I procedure, 13 patients had transatrial closure of their VSD, eight had ligation of a patient ductus arterioses, two had removal of a pulmonary artery band, and four had trans-pulmonary artery resection of short-segment subpulmonary stenosis. Modification of the original Senning operation included (1) patch augmentation (pericardium or Gore-Tex) of the atrial septal flap and (2) pericardial patch enlargement of the pulmonary venous pathway. One patient in Group 1 (3%) and two patients in Group 2 (15%) died after operation. A 2-week-old infant (Group 1) was treated with prostaglandin E1 (PGE) for 2 weeks before operation. The other hospital deaths (Group 2) occurred in a 2-month-old infant with advanced ischemic damage to the right ventricle and a 4-month-old child with multiple VSDs and Grade IV pulmonary vascular disease. No caval gradients were found after modification of the right atrial incision. Two patients died later from pulmonary venous obstruction, one during attempted recatheterization and the other after repair of the pulmonary venous obstruction. One patient had transient complete heart block, and four were discharged in junctional rhythm. Thirty-four patients (87%) were in regular sinus rhythm when released from the hospital. Postoperative catheterizations in eight patients showed no significant gradients in six and severe pulmonary venous obstruction in two (late deaths). More late postoperative results are required, including postoperative catheterization and electrophysiological studies, before the relative merits of the Senning versus the Mustard operation can be assessed.

Between November, 1978, and December, 1981, nine neonates with dextro-transposition of the great arteries (d-TGA) and intact ventricular septum underwent primary intracardiac repair (i.e., because of persistent hypoxemia following balloon atrial septostomy arterial Po2 between 17 and 27 mm Hg). Age and weight at operation ranged from 2 to 21 days (mean 6.7) and from 1,900 to 4,500 gm (mean 3,493), respectively. The Mustard procedure was used in the first three patients and the Senning technique in the last six. All operations were done with deep hypothermia and circulatory arrest lasting 43 to 88 minutes (mean 70). An associated patent ductus arteriosus was ligated in eight of the nine patients. There were no deaths. The patients have been followed up for an average of 23 months (range 1 to 36). All are asymptomatic and require no medication. In one patient, atrioventricular dissociation necessitated implantation of a pacemaker 2 weeks after operation. All others are in sinus rhythm with normal atrioventricular conduction. Hemodynamic and angiographic follow-up was obtained in seven patients 8 to 25 months postoperatively (average 14.5). Left ventricular pressure was less than 30 mm Hg and aortic oxygen saturation was greater than 90% in all patients. One of the patients who had the Mustard procedure had complete obstruction at the junction of the inferior vena cava and the lower limb of the baffle. No evidence of systemic venous obstruction was found in the patients who had the Senning procedure. Early primary intracardiac repair appears to be a satisfactory alternative in the treatment of neonates with persistent hypoxemia following balloon atrial septostomy.

The original Senning technique of interatrial transposition of venous return was selectively employed for physiological correction in 24 patients with transposition of the great arteries (TGA) ranging in age from 74 days to 26 months (median 7 months). Twenty-three had intact ventricular septum and one had a large ventricular septal defect and diaphragmatic subpulmonary stenosis. There were no hospital or late deaths, and at follow-up examination 1.5 to 12 months postoperatively, each patient was asymptomatic, in normal sinus rhythm, and had no clinical evidence of caval or pulmonary venous obstruction. Nine patients underwent cardiac catheterization and angiocardiographic studies between 1.3 and 9 months postoperatively which demonstrated widely patent venous pathways and effective “left” and “right” atrial contraction. This experience lends support to the continued use of the Senning technique for interatrial transposition of venous return.

Two hundred five infants and children with isolated transposition of the great arteries (TGA) underwent repair with the Mustard operation from October 1963, to October, 1979. To identify trends, we compared the results in the last 100 patients with the results in the earlier group of 105 children. Early mortality rate was lower in the recent group, with only two deaths compared to 11 deaths in the first group (p = 0.01). There were five late deaths in the recent group resulting from cardiac failure (two deaths), dysrhythmias, pulmonary venous obstruction, and congenital respiratory tract anomaly (one each). Complete pulmonary venous obstruction occurred once in the last 100 cases, but left pulmonary venous obstruction, also a technical problem, occurred twice. Inferior vena caval (IVC) obstruction was uncommon. Technical changes designed to reduce the incidence of dysrhythmias may have increased the incidence of superior vena caval (SVC) narrowing, which was identified nine times in the last 100 patients. The incidence of dysrhythmia was lower: 89% of patients were in sinus rhythm at discharge and 85% were in sinus rhythm at their last follow-up visit. The most serious late problems are right ventricular failure and tricuspid incompetence. Current results with the Mustard procedure are good, with a low mortality rate and few complications. These are presented to serve as a baseline for other repairs.

Infradiaphragmatic total anomalous pulmonary venous drainage (TAPVD) is a rapidly lethal congenital cardiac defect requiring early surgical intervention for survival. Six patients with this lesion were operated upon, and the last 3 of 5 survived. Clinical and hemodynamic findings include cyanosis, congestive heart failure, unimpressive murmurs, electrocardiographic signs of right axis deviation and right ventricular hypertrophy without right atrial enlargement, a typical radiologic picture, hemodynamic evidence of pulmonary venous obstruction with higher pulmonary than systemic pressures, and characteristic angiocardiograms. Principles of successful management are (I) early recognition and referral, (2) early cardiac catheterization and angiocardiography, (3) early complete surgical correction, and (4) intensive perioperative management.

Between 1968 and 1985, 80 children underwent correction of total anomalous pulmonary venous drainage. There were 47 boys and 33 girls whose ages ranged from 3 days to 16 years (median 2 months, interquartile range 5 years). Seventy (87.5%) were less than 1 year of age at operation. Fifty-eight (72.5%) weighed less than 5 kg, the range being 1.6 to 42 kg (median 3.7 kg, interquartile range 2.4 kg). Forty-five (56%) patients had supracardiac, 14 (17.5%) cardiac, 15 (19%) infracardiac, and 6 (7.5%) had mixed total anomalous pulmonary venous drainage. Follow-up was complete in 78 (97.5%) and ranged from 6 to 189 months (median 58 months, interquartile range 59 months). There were 14 (17.5%) early and six (7.5%) late deaths. Analysis by various factors revealed year of operation as the only factor to affect survival at the 5% level of significance. Early mortality was 29% between 1968-1977 and 11% between 1978-1985 (p = 0.04). Postoperative pulmonary venous obstruction occurred in five (6%) patients between 6 weeks and 3 months after operation. All 5 died, three after reoperation. Five (6%) other children had reoperations, four for residual shunts and one for superior vena caval obstruction.

Sixty-three infants with transposition of the great arteries (TGA) aged 3 weeks to 12 months, have had Mustard's operation between 1967 and 1973. Their weights ranged from 3.7 to 9.5 kilograms. Fifty-two had TGA and atrial septal defect (ASD), 10 had TGA and ventricular septal defect (VSD), and 1 had TGA, VSD, and left ventricular outflow tract obstruction (LVOTO). Fifty-eight infants were operated upon with cardiopulmonary bypass, while deep hypothermia and circulatory arrest was used in 5. Fifty of 52 infants with TGA and ASD survived the operation. In the group with complex transposition, 4 out of 10 infants with VSD survived the operation. The only infant who had TGA and LVOTO survived the operation after a prolonged postoperative period. Among 17 survivors in whom pericardium was used for the patch (1967 to 1970), fatal pulmonary venous obstruction occurred in 1. In a later group of 28 infants in whom Dacron was used, all survived the operation, but 10 developed SVC and/or IVC obstruction. This was severe enough to require operative revision in 9 (one death). Since September, 1972, pericardium has been used exclusively for the patch. Our early and late results confirm the view that early primary repair in patients with simple TGA is a preferable method of treatment

The surgical experience with total anomalous pulmonary venous connection (TAPVC) at the Vanderbilt Hospital between the years 1969 and 1979 was reviewed. Twenty-five patients with TAPVC were studied. All but one patient presented at less than 1 year of age, and 11 patients were operated upon in the first 2 weeks of life. Operative mortality rate was 20% (5/25). Four of the five deaths were in critically ill neonates requiring preoperative ventilatory support. All operative deaths were in patients with pulmonary venous obstruction. All operative survivors have been followed for a mean 4.5 years (6 months to 10 years). There have been no late deaths or cases of symptomatic pulmonary venous obstruction. All children have had normal growth patterns. Ten patients have been recatheterized. Pulmonary artery systolic pressure, which was markedly elevated prior to operation, fell to normal levels after operation. Ventricular function has been evaluated by quantitative angiocardiography in nine patients before and after operation. Left ventricular size and function were markedly depressed preoperatively and rose to normal levels in postoperative survivors. Left atrial maximal volume was found to be 94% of normal values. These data support the contention that preoperative pulmonary venous obstruction is the single risk factor predicting higher risk of operative death. The presence of severe depression of left ventricular size and function preoperatively does not predict a higher operative risk, and postoperative survivors can expect normal ventricular function.

Four cases of obstruction of the superior vena cava caused by inoperable bronchogenic carcinoma are presented in which the signs and symptoms were disabling. Palliation was achieved by placing a 10 mm. Dacron prosthetic bypass graft between the left innominate vein and the right atrial appendage, resulting in prompt relief. All patients were given warfarin for anticoagulation and subsequently deep x-ray therapy and diuretics were added. There was no operative or hospital death or morbidity. Two of the patients died of distant metastases at 14 and 6 months, respectively, postoperatively. The other two are alive and well at 11 and 5 months after the operation. Venous obstruction has not recurred to date in any of the four patients. A relevant review of the literature has been made.

A total of 256 patients survived 301 consecutive Mustard operations and were discharged from the hospital. In 34 survivors, obstruction of the superior or inferior vena cava was diagnosed clinically and confirmed by angiography. A retrospective study has shown that venous angiography is the most helpful single investigation to demonstrate the presence and site of obstruction. Of those patients in whom pericardium had been used for the baffle, 8 of 172 survivors developed systemic venous obstruction (SVO). This complication occurred in 26 of 84 survivors with a Dacron baffle. The fact that 22 of these patients were smaller than 12 kilograms at the time of the original operation suggests a possible relationship between the size of the patient and SVO in the Dacron baffle group. Twenty-one of the 34 patients with SVO had an operative revision with three hospital deaths; 4 died before revision could be undertaken. In an attempt to reduce the incidence of obstruction we now use a pericardial baffle tailored into a “trouser” shape, vena caval and atrial pressures are measured at operation, and 2 to 3 days later, SVC angiography is performed. Our results since April, 1973, suggest that these measures have been effective, but our longest follow-up to date is only 12 months. Diligent follow-up after the Mustard operation is important, as the clinical onset of obstruction may be delayed.

Increased myocardial water content has been shown to be accompanied by decreased ventricular compliance. We determined the effect of cardiopulmonary bypass for 2 (2CBP) and 3 (3CBP) hours, hypercalcemic cardiopulmonary bypass (CA), normothermic cross-clamping (CCNT), and hypothermic cross-clamping (CCHT) on myocardial water content. We found that myocardial water content was increased in the CA, CCNT, and CCHT groups. The distribution of myocardial water was different in the three groups with edema. Water accumulated in the subepicardium more than in the subendocardium in the CCNT group. The distribution of myocardial water and the histologic examinations suggest that subendocardial edema occurs in CA and CCHT because of venous obstruction in the subendocardium with intact capillary membranes. The greater accumulation of water in the subepicardium in CCNT may be related to increased capillary permeability and decreased interstitial pressure in the subepicardium. We conclude that myocardial edema during bypass may be greater in the small, firmly-contracted heart and after normothermic or hypothermic aortic cross-clamping.

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, an 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.

Between, May of 1973 and May of 1978, 130 patients underwent correction of complete transposition of the great arteries by a modification of the Mustard operation specifically designed to avoid dysrhythmias and to minimize systemic and pulmonary venous obstruction. Of the 102 surviving patients who underwent this operation, 99 still exhibited sinus rhythm on the latest standard electrocardiographic (ECG) recording. On 24 hour ECG recording between 2 weeks and 4 years postoperatively, nine (15.5%) of 58 patients having the Mustard operation for simple and complex transposition showed dysrhythmias. There were no cases of pulmonary venous or inferior vena caval obstruction in this series. Six cases of superior vena caval obstruction were encountered. Our operative and autopsy findings point to the importance of avoiding the area of the sinus and atrioventricular nodes, since by doing so, we have reduced postoperative dysrhythmias. Operative technique, in particular, wide excision of the septum secundum and baffle design as suggested by Brom, have reduced the incidence of superior and inferior caval obstruction and pulmonary venous obstruction. The modifications we have applied in our series suggest that Mustard’s operation should not be discarded prematurely.

Background: The occurrence of a progressive pulmonary venous obstruction after the repair of the total anomalous pulmonary venous connection is a severe complication. Objectives: The objectives of this study were to retrospectively review the patients with this condition and to report our experience with a new surgical technique with a sutureless in situ pericardium repair. Methods: Of 178 patients who underwent correction of total anomalous pulmonary venous connection, 16 patients (9%) experienced the development of a progressive pulmonary venous obstruction in a median interval of 4 months (5 weeks–12 years). Three patients had isolated anastomotic stenosis, 4 patients had isolated pulmonary venous ostial stenosis, and 9 patients had both. Pulmonary venous obstruction was bilateral in 7 patients. The surgical procedures used at reoperation included 8 patch enlargements, 5 ostial endarterectomies, 1 intraoperative stenting, and 7 sutureless in situ pericardium repairs. Results: There were 4 deaths after reoperation (4 of 15 patients; 27%). The only significant mortality risk factor was the bilateral location of the pulmonary venous obstruction ( P = .045). In patients with isolated anastomotic stenosis or with only 1 pulmonary venous ostial stenosis (n = 5), there was no death, except the patient presenting with a single ventricle. In patients with 2 or more pulmonary venous ostial stenoses (n = 10), there were 3 deaths; 5 of the 7 survivors were successfully treated with the in situ pericardial technique, with normalized pulmonary artery pressure at a mean follow-up of 26 months. Conclusion: Progressive pulmonary venous stenosis after repair of total anomalous pulmonary venous connection remains a severe complication when bilateral. The sutureless in situ pericardial repair offers a satisfactory solution, particularly on the right side. (J Thorac Cardiovasc Surg 1999;117: 679-87)

ObjectivePreoperative pulmonary venous obstruction has been reported to be a risk factor negatively impacting survival in total anomalous pulmonary venous connection. We examined lung tissue from total anomalous pulmonary venous connection patients with pulmonary venous obstruction and demonstrated hypoplasia of small pulmonary arteries to elucidate the mechanism underlying the poor outcome.MethodsTen total anomalous pulmonary venous connection patients with preoperative pulmonary venous obstruction between the ages of 2 days and 10 months were studied. As histological parameters, we assessed the size of small pulmonary arteries in relation to the size of accompanying bronchioles to identify small pulmonary artery underdevelopment. Other parameters, such as the radial alveolar count, which reflects alveolar maturity, intimal lesions, lymphangiectasia, and the medial thickness of small pulmonary arteries and small pulmonary veins, were also examined. As a control group, we examined 24 autopsy cases with no congenital heart or pulmonary disease.ResultsWhen the radius of the accompanying bronchiole was 100 μm, the radius of small pulmonary artery in the control group was found to enlarge for the first 2 months and then remain stable at approximately 80 μm from 2 to 10 months. In total anomalous pulmonary venous connection with preoperative pulmonary venous obstruction, the radius was significantly lower than in the control (47.0 ± 21.8 μm versus 75.9 ± 9.8 μm, P < .001), and the difference between dead and surviving patients was significant at P < .001 (33.0 ± 14.6 μm versus 68.2 ± 9.2 μm). Examination of the alveoli yielded an radial alveolar count of 4.6 ± 1.5 in the control group and 4.4 ± 0.8 in the total anomalous pulmonary venous connection patients, and the difference was not significant (P = .71).ConclusionsThe small pulmonary arteries of total anomalous pulmonary venous connection patients with preoperative pulmonary venous obstruction were underdeveloped compared with controls but their alveolae were not hypoplastic. These results suggested that the small pulmonary artery hypoplasia may be responsible for the poor outcome of these patients.

Background and methods: Between January 1, 1984, and December 1, 1997, 73 infants with functional single ventricle and total anomalous pulmonary venous connection were admitted to our institution. A retrospective review was undertaken to determine factors influencing survival. Results: Heterotaxy syndrome was present in 52 patients and hypoplastic left heart syndrome in 14. Obstructed total anomalous pulmonary venous connection was present in 21 patients. The pulmonary venous connection was supracardiac in 32 patients, cardiac in 21 patients, infracardiac in 13, and mixed in 7. Twelve patients died before the operation. The remaining 61 patients underwent surgery at a median age of 5 days (range 1 day–2.5 years). Overall survival was 45% at 6 months of age, 37% at 1 year, and 19% at 5 years. Survival for patients undergoing surgery was 54% at 6 months of age, 44% at 1 year, and 23% at 5 years. By univariate analysis with the Cox proportional hazards model, younger age at the time of the initial operation and repair of total anomalous pulmonary venous connection were predictors of mortality. Lung tissue from 14 patients was available for histologic examination. The pulmonary veins were dilated and wall thickness was increased. Increased muscularization of the arteries was seen in 11 patients. Conclusions: The long-term prognosis for children undergoing staged reconstructive operations for single ventricle and total anomalous pulmonary venous connection is poor. Early mortality is high and late death is a continuing risk. Development of the pulmonary vasculature, especially the pulmonary veins, is abnormal, even in children without clinical evidence of pulmonary venous obstruction. (J Thorac Cardiovasc Surg 1999;117:506-14)

Two cases of a very uncommon congenital association (cor triatriatum and total anomalous pulmonary venous connection) are presented. This association should be suspected in any infant with signs of pulmonary venous obstruction.

A patient with total anomalous pulmonary venous connection to the coronary sinus and stenosis of the right atrial opening of the coronary sinus is described. While the initial studies were compatible with increased pulmonary blood flow, subsequent evaluation demonstrated severe pulmonary venous obstruction. Successful operative correction was performed.

Forty-three consecutive patients with previous balloon atrial septostomy have undergone a Senning type repair of transposition of the great arteries without patch augmentation of the atrial septum. A technique was used that allows expansion of the atrial septum without the use of additional foreign or autogenous materials. The operative (30-day) mortality rate was 4.6% (2/43) with no late deaths. A mean follow-up of 20 months shows that 83% of the patients are in sinus rhythm and none of these patients have clinically detectable caval or pulmonary venous obstruction or baffle leaks. In patients selected for atrial switch, the Senning operation may be performed without patch augmentation of the septum. Performed by the method described herein, the operation provides predictable early and late results with a low prevalence of arrhythmias. Significant venous obstruction has not occurred.

underline the relative greater frequency of this complication in heterotaxy syndrome when associated with anomalous pulmonary and systemic venous connections, which can make surgical repair complicated with the need to create a tortuous and potentially obstructive pathway. Despite this concern, a recent report from our institution 5 has shown excellent outcome with cavopulmonary connection in patients with heterotaxy syndrome and anomalous pulmonary venous return. This is likely due to improved surgical technique and use of extracardiac conduit in cases with complex atrial and venous anatomy (18%). None of the 3 patients had evidence of obstruction at the pulmonary venous pathway early after the Fontan procedure, as determined by 2-dimensional echocardiography. Pulmonary venous obstruction developed slowly through a period of several months to years. In patient 2, pulmonary venous obstruction developed 4 months after device closure of the fenestration, suggesting that the additional scarring from device implantation contributed to the obstruction. The obstruction was due to fibrotic tissue at either the interatrial septum or in the interatrial course of the pulmonary veins (patient 2). To prevent this complication, and to ensure a wide open pulmonary venous outflow, particularly in patients with mitral atresia or stenosis, we currently enlarge the communication between the pulmonary veins and the right atrium by unroofing the coronary sinus posteriorly into the left atrium, in addition to resection of the interatrial septum. In conclusion, late onset obstruction of pulmonary venous pathway after lateral tunnel cavopulmonary connection is a rare but serious complication that may be prevented by unroofing the coronary sinus at the time of Fontan procedure.

may have been promoted. In this case, endocarditis was initially suspected because of vegetation-like mobile masses that were found in the aortic valve on the transesophageal echocardiogram, but infection was negative in all other examination findings. We determined before surgery that these masses were most likely cardiac tumors. Mild exertional dyspnea was the only clinical symptom, but it is known that mobile tumors attached to an aortic valve can cause serious complications such as thromboembolism and myocardial ischemia, and we decided that a surgical excision was necessary. QAV with a narrow aortic annulus presenting with the development of multiple CPFs is a rare complication. There have been no similar cases reported in the literature. However, according to the characteristics of each pathologic condition, there is a possibility that CPF may develop with QAV.

We have recently encountered three patients with the hemodynamics of complete transposition accompanied by unusual atrial arrangements. The flow patterns produced all required correction by means of a “mirror-image” Mustard operation. Two of the patients had left atrial isomerism as evidenced by the finding of bilateral atrial appendages of morphologically left type. In both patients the systemic venous return was to the left-sided atrium and the pulmonary venous return to the right-sided atrium. The major effect of the isomerism was to reduce the volume of the left-sided chamber. This initially produced concern, since we now try to avoid enlargement of the pulmonary venous atrium when performing Mustard's operation. However, despite the small volume, it was possible to place the baffle without producing postoperative pulmonary or systemic venous obstruction. The other significant effect of left atrial isomerism is to deviate the position of the sinus node, but thus far we have not encountered any postoperative rhythm problems. The third patient had a mirror-image arrangement of the atrial chambers and the venous connections (situs inversus), and the anatomy was corrected by straightforward mirror-image reversal of our current technique for Mustard's procedure.

J Thorac Cardiovasc Surg 1998;116:648-9

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