*****ANNALS OF THORACIC SURGERY***** (REFERENCE 1 OF 13) 99347500 Choudhary SK Bhan A Sharma R Mathur A Airan B Saxena A Kothari SS Juneja R Venugopal P Repair of total anomalous pulmonary venous connection in infancy: experience from a developing country. In: Ann Thorac Surg (1999 Jul) 68(1):155-9 ISSN: 0003-4975 BACKGROUND: Corrective surgery for total anomalous pulmonary venous connection in infancy still carries high morbidity and mortality rates in developing countries. The present study evaluates the factors responsible for it. METHODS: Seventy-three infants were operated on for total anomalous pulmonary venous connection from January 1987 through October 1997. Age ranged from 5 days to 12 months (mean, 3.9+/-0.24 months), with 10 (13.7%) patients younger than 1 month old. Patient weight varied from 2.0 to 5.2 kg (mean, 3.7+/-0.27 kg). Most (90.5%) patients were small for their ages (< 50th percentile). Anomalous connection was supracardiac in 42 (57.5%), cardiac in 18 (24.7%), infracardiac in 4 (5.5%), and mixed in 9 (12.3%) patients. Thirty-five patients had obstructed drainage. Preoperatively, 30 patients received antibiotic therapy for respiratory tract infection, 3 patients had balloon atrial septostomy, and 4 patients required mechanical ventilation. Fifteen patients (20.5%) were operated on as an emergency procedure. For supracardiac and infracardiac connections, a posterior approach was used for anastomosis. In cardiac type, coronary sinus was unroofed and the resultant defect along with atrial septal defect was closed with a single patch. RESULTS: The operative mortality rate was 23.3% (17 of 73). Pulmonary hypertensive crisis was the cause of death in 10 patients. Emergency operation and weight less than the 25th percentile were the important risk factors for operative mortality. Young age (< 1 month) and type of drainage did not affect the mortality. Follow-up ranged from 1 to 108 months (mean, 56.4+/-26.0 months). There were two late deaths. The actuarial survival (Kaplan Meier) at 9 years was 72.87%+/-5.39%. CONCLUSION: Failure of early recognition, and thus delayed referral, accounted for onset of cardiac cachexia, respiratory tract infection, and severe pulmonary hypertension, which had a major effect on unfavorable outcome. Institutional address: Department of Cardiothoracic and Vascular Surgery Cardiothoracic Center All India Institute of Medical Sciences New Delhi. (REFERENCE 2 OF 13) 99090949 Caldarone CA Najm HK Kadletz M Smallhorn JF Freedom RM Williams WG Coles JG Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies. In: Ann Thorac Surg (1998 Nov) 66(5):1521-6 ISSN: 0003-4975 BACKGROUND: Recent reports have cited improving results for surgical management of isolated total anomalous pulmonary venous drainage. Complex cases (with other cardiac anomalies) are less frequently reported and are associated with higher mortality. METHODS: Retrospective review identified 170 consecutive patients treated for total anomalous pulmonary venous drainage from 1982 to 1996: 44 cases were "complex" (with significant associated cardiac lesions) and 126 cases were "simple." RESULTS: Operative mortality for simple cases decreased from 26% to 8%, and mortality for complex cases remained constant at 52%. Age, size, and the presence of atrial isomerism were univariate predictors of mortality. Multivariable analysis identified only univentricular hearts and associated cardiac lesions as predictors of operative mortality. Pulmonary artery (n = 16) and arteriopulmonary (n = 7) shunting strategies for complex cases resulted in less than 30% long-term survival. CONCLUSIONS: Despite improvement in survival for simple cases, management of total anomalous pulmonary venous drainage with single-ventricle hearts or other associated cardiac lesions remains problematic. Institutional address: Division of Cardiovascular Surgery The Hospital for Sick Children University of Toronto and University of Toronto Faculty of Medicine Ontario Canada. Chris-Caldarone@UIowa.edu (REFERENCE 3 OF 13) 99090948 Caldarone CA Najm HK Kadletz M Smallhorn JF Freedom RM Williams WG Coles JG Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage. In: Ann Thorac Surg (1998 Nov) 66(5):1514-20 ISSN: 0003-4975 BACKGROUND: Progressive stenosis of the pulmonary veins after repair of total anomalous pulmonary venous drainage is frequently refractory to surgical therapy. METHODS: Retrospective review of 170 consecutive patients treated for total anomalous pulmonary venous drainage identified 13 patients with postrepair pulmonary vein stenosis. Preoperative and operative data were analyzed to define the patterns of progression and efficacy of surgical techniques. RESULTS: Seventeen reoperations were performed in 13 patients. Postrepair pulmonary vein stenosis was most common in the infracardiac and mixed subtypes (p < 0.02). All 4 patients with unilateral stenosis, 2 of whom had progression of stenosis resulting in nearly complete unilateral pulmonary vein occlusion, survived. Six of 9 patients with bilateral disease died (p < 0.05 versus unilateral); 2 of the 3 survivors were repaired with a novel technique creating a sutureless neoatrium without evidence of restenosis at 1.8 years' follow-up. Stenting was uniformly unsuccessful. CONCLUSIONS: In unilateral stenosis, progression of disease may be survivable with loss of single-lung perfusion. Although bilateral disease is lethal in most cases, creation of a sutureless neoatrium has demonstrated short-term freedom from disease progression. Institutional address: Division of Cardiovascular Surgery The Hospital for Sick Children University of Toronto and University of Toronto Faculty of Medicine Canada. Chris-Caldarone@UIowa.edu (REFERENCE 4 OF 13) 99015458 Imoto Y Kado H Asou T Shiokawa Y Tominaga R Yasui H Mixed type of total anomalous pulmonary venous connection. In: Ann Thorac Surg (1998 Oct) 66(4):1394-7 ISSN: 0003-4975 BACKGROUND: The mixed type of total anomalous pulmonary venous connection is a rare condition in which some diagnostic and surgical problems still remain to be solved. METHODS: In 9 patients a single pulmonary vein was connected to the systemic vein at a site different from the drainage site of the confluence of three other pulmonary veins. In 2 other patients, four pulmonary veins made a confluence which had two drainage sites. Correct diagnosis was made in all 7 patients who received cardiac catheterization but only in 5 of the 9 patients by color Doppler echocardiography. Total correction was performed in 3 patients and the single anomalous pulmonary vein was left uncorrected in 8 other patients. RESULTS: There were two in- hospital deaths. Seven patients with a single residual anomalous pulmonary vein have been in good condition without clinical symptoms of congestive heart failure or pulmonary hypertension. CONCLUSIONS: Diagnosis of mixed type of total anomalous pulmonary venous correction by echocardiography is sometimes difficult. When a mixed type is suspected, cardiac catheterization is recommended if the condition of the patient permits it. A single anomalous pulmonary vein may be left uncorrected without serious complications, but close observation is needed to prevent congestive heart failure and pulmonary vascular obstructive disease. Institutional address: Department of Cardiovascular Surgery Fukuoka Children's Hospital Kyushu University Japan. (REFERENCE 5 OF 13) 95289768 Gaynor JW Burch M Dollery C Sullivan ID Deanfield JE Elliott MJ Repair of anomalous pulmonary venous connection to the superior vena cava. In: Ann Thorac Surg (1995 Jun) 59(6):1471-5 ISSN: 0003-4975 Complex forms of anomalous pulmonary venous connection to the superior vena cava (SVC) can be difficult to correct surgically. Since 1987, 11 patients have undergone repair of anomalous pulmonary venous connection to the SVC by diversion of the pulmonary venous drainage to the left atrium using a baffle with division of the SVC and reimplantation on the right atrial appendage to restore normal systemic venous drainage. Total anomalous pulmonary venous connection was present in 3 patients and partial anomalous pulmonary venous connection, in 8. All patients are alive and asymptomatic at a mean follow-up of 2.3 +/- 1.4 years. Postoperative echo-cardiograms (8 patients) revealed pulmonary venous obstruction requiring reoperation in 1 patient. No patient has clinical evidence of SVC obstruction, and all are in sinus rhythm. This is a safe and effective technique for repair of complex forms of anomalous pulmonary venous connection to the SVC, and the incidence of postoperative venous obstruction and rhythm disturbances is low. Institutional address: Cardiothoracic Unit Hospital for Sick Children London England. (REFERENCE 6 OF 13) 98391270 McElhinney DB Reddy VM Anomalous pulmonary venous return in the staged palliation of functional univentricular heart defects. In: Ann Thorac Surg (1998 Aug) 66(2):683-7 ISSN: 0003-4975 BACKGROUND: Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic, pulmonary, or systemic and pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. METHODS AND RESULTS: Between March 1990 and March 1997, 32 patients with functional single ventricle and anomalous pulmonary venous return underwent operation at our institution. Five of 25 patients who underwent neonatal palliation died in the early postoperative period, all of whom had obstructed anomalous pulmonary venous return. Twenty-one patients have undergone bidirectional cavopulmonary shunt, including 7 in whom this was the primary palliative procedure. There was one early and two late deaths after the bidirectional Glenn procedure, two in patients with asplenia syndrome and none in patients with previously obstructed pulmonary venous return. Seven patients have undergone Fontan completion, 5 with an extracardiac conduit. There was one early death and one take-down to a classic Glenn shunt, both in patients who did not undergo the extracardiac conduit Fontan operation. CONCLUSIONS: Anomalous pulmonary venous return can significantly complicate the management of the single- ventricle patient, with the major impact on survival coming in the neonatal period. Palliation with the aim of performing an extracardiac conduit Fontan procedure allows greater latitude and more streamlined management in this group of patients. Institutional address: Division of Cardiothoracic Surgery University of California San Francisco 94143-0118 USA. (REFERENCE 7 OF 13) 98257228 Serraf A Belli E Roux D Sousa-Uva M Lacour-Gayet F Planche C Modified superior approach for repair of supracardiac and mixed total anomalous pulmonary venous drainage. In: Ann Thorac Surg (1998 May) 65(5):1391-3 ISSN: 0003-4975 BACKGROUND: The main goal in the surgical repair of total anomalous pulmonary venous drainage is to reestablish a wide patent connection between the common pulmonary vein and the left atrium. Several techniques have been proposed for achieving this objective, each of which has advantages and disadvantages. The superior approach between the superior vena cava and the ascending aorta was introduced in 1976 for the repair of supracardiac forms of total anomalous pulmonary venous drainage, but it often provides a less than optimum exposure, particularly in tiny infants. We proposed a modification of this approach that includes division of the ascending aorta and offers excellent exposure. METHODS: Seventeen patients (15 neonates and 2 infants) with supracardiac total anomalous pulmonary venous drainage (n = 13) or mixed forms of total anomalous pulmonary venous drainage (n = 4) underwent surgical repair with the use of the modified superior approach. Circulatory arrest was not required in 10 patients and the mean cross-clamp time was 32.5 +/- 13.8 minutes. RESULTS: There was 1 postoperative death resulting from intractable pulmonary hypertension in a compromised infant who was referred to our unit receiving extracorporeal membrane oxygenation. One patient with common hypoplasia underwent reoperation twice at 2 months and then 3 months after the first procedure. All the other patients had a smooth postoperative course, and midterm evaluation showed a widely patent anastomosis between the common vein and the left atrium. CONCLUSIONS: The modified superior approach for the repair of supracardiac total anomalous pulmonary venous drainage can be useful to enhance exposure during surgical repair and may contribute to improved patient outcome. Institutional address: Department of Pediatric Cardiac Surgery Marie-Lannelongue Hospital Le Plessis-Robinson France. aserraf@centre-chirurgical-marie-lannelongue.asso.fr (REFERENCE 8 OF 13) 94107074 Heinemann MK Hanley FL Van Praagh S Fenton KN Jonas RA Mayer JE Jr Castaneda AR Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. In: Ann Thorac Surg (1994 Jan) 57(1):88-91 ISSN: 0003-4975 Children with visceral heterotaxy often present with total anomalous pulmonary venous drainage (TAPVD) associated with univentricular congenital heart disease. We reviewed our experience with the primary surgical management of this lesion under these circumstances. Over a recent 10-year span, 38 patients within the first 3 days of life were admitted to our institution and underwent primary palliation. Twenty- one of them had TAPVD, 18 to a systemic vein. Twelve (67%) of these 18 were seen with obstruction of the anomalous connection and underwent emergency operation. In 7 patients, repair of TAPVD was combined with a systemic-pulmonary artery shunt because of additional obstruction of the pulmonary blood supply, with two deaths. One patient had primary shunting and then repair of TAPVD. Four patients underwent repair of TAPVD alone. Two of them then showed signs of insufficient pulmonary blood flow, received a shunt in a second procedure, and subsequently died. Early mortality in the group with obstructed TAPVD was thus 4 (33%) of 12 patients. Statistical analysis of all 38 patients (univariate analysis, chi 2 testing) showed that neither the presence of TAPVD (p = 0.7) nor TAPVD repair alone (p = 0.8) or with shunting (p = 0.8) was a definite risk factor for early death. The performance of a shunt during the first operation, however, was associated with lower early mortality (p = 0.03). Total anomalous pulmonary venous drainage is a common finding in newborns with visceral heterotaxy. Its presence and its subsequent early repair (requiring cardiopulmonary bypass) do not increase the mortality risk. The need of a concomitant shunt in obstructed TAPVD can initially be underestimated. Institutional address: Department of Cardiac Surgery Children's Hospital Harvard Medical School Boston Massachusetts. (REFERENCE 9 OF 13) 93111822 Cobanoglu A Menashe VD Total anomalous pulmonary venous connection in neonates and young infants: repair in the current era [see comments] In: Ann Thorac Surg (1993 Jan) 55(1):43-8; discussion 48-9 ISSN: 0003-4975 Total anomalous pulmonary venous connection has been one of the more challenging congenital heart defects in newborns and young infants despite improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care. Since 1981, 30 patients with total anomalous pulmonary venous connection have undergone primary total correction. Mean age at operation was 28 +/- 6 days and mean weight, 3.3 +/- 0.7 kg. Essential features of the surgical approach in these small patients included early surgical intervention, profound hypothermia with total circulatory arrest, cardioplegic myocardial preservation, and a wide posterior anastomosis. Operative mortality was 13% +/- 6%. All four deaths were in patients having emergency operation within 24 hours of the surgical consult and requiring ventilator support preoperatively. The mean follow-up is 47 +/- 7 months. There have been two late deaths, and the 7-year survival rate is 79% +/- 8%. There have been two reoperations, and 91% +/- 6% of the patients are reoperation free at 7 years. Only 1 of the 24 surviving patients is symptomatic. Growth in survivors is closely monitored. The height growth percentile is less than 5% in 15% +/- 8% of survivors and the weight growth percentile, less than 5% in 17 +/- 8%. During the past decade, with a consistent surgical approach to neonates and infants with total anomalous pulmonary venous connection, it has been possible to achieve low early mortality, low attrition, and excellent late results. Comment in: Ann Thorac Surg 1993 Aug;56(2):395 Institutional address: Division of Cardiopulmonary Surgery Oregon Health Sciences University Portland 97201-3098. *****CIRCULATION***** (REFERENCE 10 OF 13) 97057373 Bando K Turrentine MW Ensing GJ Sun K Sharp TG Sekine Y Girod DA Brown JW Surgical management of total anomalous pulmonary venous connection. Thirty-year trends. In: Circulation (1996 Nov 1) 94(9 Suppl):II12-6 ISSN: 0009-7322 BACKGROUND: Reports of surgical correction of total anomalous pulmonary venous connection (TAPVC) over the past 30 years indicate a general improvement in operative survival. However, prevention of late pulmonary venous obstruction continues to be a cornerstone of successful repair. The purpose of the study was to identify factors associated with improvement in perioperative mortality and to determine risk factors for death and reoperation due to pulmonary vein stenosis after repair of TAPVC. METHODS AND RESULTS: Using univariate and multiple regression analysis, we analyzed risk of early and late mortality and need for reoperation in 105 patients operated on between April 1966 and June 1995. Despite increased frequency of neonatal repair in the most recent time period (29% in 1966 through 1985; 55% in 1991 through 1995, P < .05), operative mortality declined (13% in 1966 through 1985; 0% in 1991 through 1995). The incidence of postoperative pulmonary hypertensive episodes and death related to pulmonary hypertension decreased significantly over the study period (P < .001). Aggressive preoperative elective medical stabilization and prophylaxis of postoperative pulmonary hypertensive episodes may have contributed to this improvement. By univariate analysis, preoperative pulmonary hypertension (P < .02) and preoperative pulmonary vein obstruction (P < .01) correlated with early mortality up to 1990 but not in the past 5 years. Multiple logistic regression analysis showed that only a small pulmonary confluence associated with diffuse pulmonary vein stenosis was an independent risk factor for early (P < .001) and late (P = .01) death as well as need for reoperation (P = .007). Type of TAPVC was not a significant risk factor throughout the three decades of our experience. At a median follow-up of 87 months, late survival was 98% (93 of 95 operative survivors), and all are NYHA class I. CONCLUSIONS: Improvements on surgical technique as well as preoperative and postoperative management account for the reduction in mortality and need for reoperation for most types of TAPVC. However, the presence of a small venous confluence and diffuse pulmonary vein stenosis remains a risk factor for adverse outcome. Institutional address: Section of Cardiothoracic Surgery James W. Riley Hospital for Children Indianapolis IN USA. *****JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY***** (REFERENCE 11 OF 13) 92356635 Raisher BD Grant JW Martin TC Strauss AW Spray TL Complete repair of total anomalous pulmonary venous connection in infancy. In: J Thorac Cardiovasc Surg (1992 Aug) 104(2):443-8 ISSN: 0022-5223 From 1983 to 1990, 20 infants underwent complete repair of isolated total anomalous pulmonary venous connection. Twelve were male; ages ranged from 1 day to 240 days (mean 32 days). The abnormal anatomic connection was supracardiac in nine, cardiac in four, mixed in five, and infradiaphragmatic in two. In seven patients the pulmonary venous drainage was obstructed. Corrective operations were performed 2.4 days after admission; after medical stabilization, seven patients underwent emergency operations, including extracorporeal membrane oxygenation in one. All operations were performed with the use of circulatory arrest (mean 33 minutes) and cardiopulmonary bypass (mean 54 minutes). There was one hospital death, which occurred in a premature infant (1.5 kg) who had supracardiac drainage. No preoperative risk factors correlated with a poor surgical outcome. Pulmonary hypertensive episodes were expectantly managed after the operation with 100% oxygen, sedation, and hyperventilation. Postoperative arrhythmias occurred predominantly in patients with intracardiac drainage. All survivors (mean follow-up of 42 months) are in sinus rhythm, receiving no medications, and are growing and developing normally. Surgical correction of total anomalous pulmonary venous connection in infancy can be performed at low risk with good results after aggressive preoperative stabilization and postoperative management. Institutional address: Division of Cardiology St. Louis Children's Hospital Mo. (REFERENCE 12 OF 13) 99197171 Lacour-Gayet F Zoghbi J Serraf AE Belli E Piot D Rey C Marcon F Bruniaux J Planche C Surgical management of progressive pulmonary venous obstruction after repair of total anomalous pulmonary venous connection. In: J Thorac Cardiovasc Surg (1999 Apr) 117(4):679-87 ISSN: 0022-5223 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. Institutional address: Marie-Lannelongue Hospital Paris-Sud University Paris France. (REFERENCE 13 OF 13) 94048176 Lupinetti FM Kulik TJ Beekman RH 3d Crowley DC Bove EL Correction of total anomalous pulmonary venous connection in infancy. In: J Thorac Cardiovasc Surg (1993 Nov) 106(5):880-5 ISSN: 0022-5223 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. Institutional address: Department of Surgery University of Michigan School of Medicine Ann Arbor.