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Partial anomalous pulmonary vein connection (PAPVC) is characterized by drainage of 1 or more pulmonary veins (butnot all)into theright atrium orone ofits tributary veins. Anomalous connections of the left pulmonary veins are rare. Bilateral PAPVC is extremely unusual. We report the case of a patient who underwent surgical closure of the atrial septal defect (ASD), ventricular septal defect (VSD), and correction of the left superior pulmonary vein drainage at the age of 7 months. Subsequent postoperativecontrolsshowednoevidenceofrightchamberoverload regression. We performed cardiac magnetic resonance imaging (MRI) that showed a PAPVC of the right superior pulmonary vein to the superior vena cava and a PAPVC of the left inferior pulmonary vein to the left suprahepatic vein through a collector. The child underwent definitive surgical correction at the age of 5 years. Postoperative recovery was uneventful. Predischarge transthoracic echocardiogram showed an important reduction of the volume of the right chambers. PAPVC is characterized by drainage of 1 or more pulmonary veins, but not all, into the right atrium or one of its tributary veins. It often involves the right superior pulmonaryvein.Anomalousconnectionsoftheleftpulmonaryveins are rare. Bilateral PAPVC is extremely unusual (1%-2%). 1 BilateralPAPVCusuallyinvolvestheupperlobeofbothlungs with the left superior pulmonary vein draining into the left innominate vein through an anomalous vertical vein. 1,2

OBJECTIVES: This study focuses on the different surgical and endoscopical treatment alternatives when dealing with severe complications after slide tracheoplasty (STP). METHODS: Retrospective study of patients with symptomatic congenital tracheal stenosis (CTS) admitted to a single institution, between January 1997 and January 2013, surgically treated by means of STP. The following variables were evaluated: demographics, preoperative tracheal stenosis characteristics, associated anomalies and outcome measures. RESULTS: Cohort included 14 patients (8 males and 6 females) with a mean age of 8.7 months when treated (range, 1–43 m). Eleven patients (78%) showed a long segment CTS (>30% of total tracheal length) and 9 (64%) had associated cardiac or great vessel anomalies (left pulmonary artery sling). Three patients (21%) showed severe postoperative complications that required significant airway reintervention: tracheal resection of a restenotic segment, laser division with balloon dilatation of a residual stenosis and placement of a biodegradable endotracheal stent in an extensive tracheal narrowing. All patients are in good clinical condition with a mean follow-up of 6.3 years (range, 2 months to 16 years). CONCLUSIONS: STP has become the procedure of choice when dealing with CTS. Although it shows clear advantages compared with other surgical techniques, severe and difficult to manage complications may occur. Surgeons involved in their treatment should be familiar with diverse surgical and endoscopical procedures. Biodegradable airway stenting is a new and promising technique when long and severe post-surgical tracheal stenosis is present.

Introduction and objectives The Fontan operation is usually the final palliative procedure in patients with univentricular heart. The objectives of this study were, firstly, to describe the clinical and haemodynamic characteristics of a group of patients with univentricular physiology who had previously been palliated with a bidirectional Glenn procedure and, secondly, to identify risk factors that can influence postoperative outcomes after the Fontan operation. Methods Retrospective study with 32 patients who underwent a Fontan operation between March 2000 and December 2009. Clinical characteristics, catheterization data, type and duration of surgery were revised and analyzed as predictors of postoperative outcome. Results Hospital mortality was 3%. After a median follow-up of 44 months (interquartile range, 32-79), survival was 90%. Preoperative mean pulmonary arterial pressure (measured during catheterization) was correlated with late mortality. Of the remaining variables analyzed, the Nakata and McGoon indices, and duration of cardiopulmonary bypass showed the highest correlations with postoperative outcomes. Interventional catheterization before the Fontan operation was performed in 42% of patients. Conclusions Hospital mortality after the Fontan operation was very low. The performance of a haemodynamic study before the Fontan operation made it possible to select high-risk patients for surgery as well as permitting the performance of interventional procedures that could improve postoperative outcome in these patients.

Resumen Introduccion y objetivos Los pacientes con corazon univentricular son sometidos a una serie de intervenciones paliativas que culminan en la operacion de Fontan. El objetivo de este trabajo es revisar las caracteristicas clinicas y hemodinamicas de un grupo de pacientes con fisiologia univentricular paliados previamente con operacion de Glenn bidireccional e identificar los factores de riesgo que influyen en la evolucion postoperatoria de la cirugia de Fontan. Metodos Estudio retrospectivo de 32 pacientes sometidos a cirugia de Fontan entre marzo de 2000 y diciembre de 2009. Se revisaron las caracteristicas clinicas, los datos derivados del cateterismo, el tipo de cirugia y los tiempos quirurgicos y se busco su relacion con la evolucion postoperatoria. Resultados La mortalidad hospitalaria fue del 3%. Tras una mediana [intervalo intercuartilico] de seguimiento de 44 meses [32-79], la supervivencia es del 90%. La presion media en arteria pulmonar medida en el cateterismo se relaciono con la mortalidad tardia. De las demas variables estudiadas, las que mejor se relacionaron con la evolucion postoperatoria fueron los indices de Nakata y McGoon y el tiempo de circulacion extracorporea. En el 42% de los pacientes se realizo cateterismo intervencionista previo a la operacion de Fontan. Conclusiones Realizamos la operacion de Fontan con muy baja mortalidad hospitalaria. El cateterismo previo a la operacion de Fontan permite seleccionar a los pacientes de alto riesgo para la cirugia asi como realizar procedimientos intervencionistas que podrian mejorar la evolucion postoperatoria.

Objective: This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). Methods: The records of all (n = 56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. Results: A wide range of ASD sizes (5-34 mm) and devices of various types and sizes (range 12-60 mm) were involved, including 13 devices less than 20 mm. Complications leading to surgery included embolisation (n = 29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n = 12), significant residual shunt (n = 12), aortic or atrial perforation or erosion (n = 9), haemopericardium with tamponade (n = 5), aortic or mitral valve injury (n = 2) and endocarditis (n = 1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% ( p = 0.001). Conclusions: Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory. (C) 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective: Chronic volume overload in repair of tetralogy of Fallot (TOF) with transannular patch leads to significant late morbidity and mortality. Preserving pulmonary valve integrity offers a better long-term prognosis, despite a risk of residual stenosis. In our study we analyzed the evolution of pressure gradients in patients operated with conservative approaches, with particular regard to those babies with an immediate postoperative Prv/Plv ratio � 0.70. Methods: Between January 2000 and June 2008, 24 patients with TOF underwent reparative surgery with a valve sparing procedure (median age 8.1 months, range 1.1—86.6). The intraoperative post-repair echocardiography showed a Prv/Plv ratio � 0.70ineightpatients(33%,groupA) and

Objective: Tracheobronchial obstruction is infrequent in the pediatric age group but it is associated with significant morbidity and mortality. The purpose of this study is to review the results of a single institution experience with endoscopic stent placement in children with benign tracheobronchial obstruction, and with special concern on safety and clinical effectiveness. Materials and methods: Twenty-one patients with severe airway stenosing disease in which stent placement was performed between 1993 and 2006. Inclusion criteria according to the clinical status were: failure to wean from ventilation, episode of apnea, frequent respiratory infections (>3 pneumonia/year), and severe respiratory distress. Additional criteria for stent placement were: failure of surgical treatment, bronchomalacia, and tracheomalacia refractory to previous tracheostomy. Selection of the type of stent depended on the site of the lesion, the patient’s age, and the stent availability when time of presentation. The following variables were retrospectively evaluated: age, type of obstruction, associated malformations, stent properties, technical and clinical success, complications and related reinterventions, outcome and follow-up period. Results: Thirty-three stents were placed in the trachea (n = 18) and/or bronchi (n = 15) of 21 patients with a median age of 6 months (range, 9 days—19 years). Etiology of the airway obstruction included severe tracheomalacia and/or bronchomalacia in 19 cases (90%), and postoperative tracheal stenosis in two. Twelve children had a total of 20 balloon-expandable metallic stents placed, and 10 had 13 silicone-type stents (one patient had both). In nine patients (42%) more than one device was placed. Stent positioning was technically successful in all but one patient. Clinical improvement was observed in 18 patients (85%) but complications occurred in five of them (27%). Eight patients died during follow-up but only in one case it was related to airway stenting. Thirteen patients (62%) are alive and in good condition with a mean follow-up of 39 months (1—13.8 years). Conclusions: Although the results were based on a small series, placement of stents in the pediatric airway to treat tracheobronchial obstruction seems to be safe and effective. Stenting is a satisfactory therapeutic option when other procedures have failed or are not indicated. # 2008 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease beyond 1 week of age. “Classic” TOF usually presents for surgical repair during the first 3–9 months of life. Surgical repair techniques vary among surgeons and institutions. The optimal timing and approach to complete (vs. staged) surgical repair remain a topic of continued debate in the modern surgical era. A pulmonary valve sparing or reconstruction surgical approach has become popularized over the past decade or so. The purpose of this chapter will be to review the morphologic and physiologic features of “classic” TOF with an emphasis on contemporary approaches to surgical treatment.

Tissue engineering offers the potential of providing vessels that can be used to replace diseased and damaged native blood vessels. The endothelization of a synthetic vascular graft minimizes the failures associated with blood clotting and platelet activation. The aim of this study was to culture vascular-derived endothelial and smooth muscle cells on both untreated and NaOH-treated poly(epsilon-caprolactone) (PCL) films, a biocompatible and bio-resorbable polymer, and to evaluate the behavior of both cell types as a preliminary study for vascular graft development. PCL films were prepared by hot pressing; characterized by DSC, IR, SEM, and scanning force microscopy; and treated with NaOH to increase the surface hydrophilicity before cell culture. Endothelial and smooth muscle cells, isolated from pig cava vein, were characterized by immunofluorescence and confocal microscopy studies of endothelial nitric oxide synthase and alpha-smooth muscle actin. Good adhesion, growth, viability and morphology of both the endothelial and smooth muscle cells on PCL films were obtained, but a light stimulation of mitochondrial activity was observed during short culture times. NaOH treatment improved the adhesion and enhanced the proliferation in both cell types. This verified the possible use of this modified polymer as a support in the preparation of a synthetic vascular graft. [Diagram: see text] SEM micrograph of smooth muscle cells cultured on NaOH-treated PCL film. (Original magnification: 1000x).

Although mortality is low after the modified Fontan procedure, there is a significant percentage of patients with prolonged postoperative recovery. The objective of this study is to evaluate the usefulness of postoperative administration of oral sildenafil and inhaled nitric oxide on early postoperative outcome.A prospective interventional and comparison study with a historical cohort was conducted. Between January, 2010 and March, 2013, 16 patients received oral sildenafil during immediate modified Fontan postoperative period. Inhaled nitric oxide was also administered if the patient was kept intubated 12 hours after surgery. Early postoperative outcome was compared with a historical cohort of 32 patients on whom the modified Fontan procedure was performed between March, 2000 and December, 2009.Postoperative administration of sildenafil and nitric oxide had no influence on early postoperative outcome after the modified Fontan procedure in terms of duration of pleural effusions, mechanical ventilation time, length of stay in the ICU, and length of hospital stay.

Between 1980 and 1993, 20 patients less than 1 year of age underwent operations for congenital mitral valve disease. Ten patients had congenital mitral incompetence and 10 had congenital mitral stenosis. Mean age was 6.6 ± 3.4 months and mean weight was 5.6 ± 1.5 kg. Atrioventricular canal defects, univentricular heart, class III/IV hypoplastic left heart syndrome, discordant atrioventricular and ventriculoarterial connections, and acquired mitral valve disease were excluded. Indications for operation were intractable heart failure or severe pulmonary hypertension, or both. Associated lesions, present in 90% of the patients, had been corrected by a previous operation in seven. In congenital mitral incompetence there was normal leaflet motion ( n = 3), leaflet prolapse ( n = 2), and restricted leaflet motion ( n = 5). In congenital mitral stenosis anatomic abnormalities were parachute mitral valve ( n = 4), typical mitral stenosis ( n = 3), hammock mitral valve (n = 2), and supramitral ring ( n = 1). Mitral valve repair was initially performed in 19 patients and valve replacement in one with hammock valve. Concurrent repair of associated lesions was performed in 12 patients. The operative mortality rate was zero. There were six early reoperations in five patients for mitral valve replacement ( n = 4), a second repair ( n = 1), and prosthetic valve thrombectomy ( n = 1). One late death occurred 9 months after valve replacement. Late reoperations for mitral valve replacement ( n = 2), aortic valve replacement ( n = 1), mitral valve repair ( n = 2), subaortic stenosis resection ( n = 1), and second mitral valve replacement ( n = 1) were performed in five patients. Actuarial freedom from reoperation is 58.0% ± 11.3% (70% confidence limits 46.9% to 68.9%) at 7 years. After a mean follow-up of 67.6 ± 42.8 months, 94% of living patients are in New York Heart Association class I. Doppler echocardiographic studies among the 13 patients with a native mitral valve show mitral incompetence of greater than moderate degree in one patient and no significant residual mitral stenosis. Overall, six patients have mitral prosthetic valves with a mean transprosthetic gradient of 6.2 ± 3.7 mm Hg. These results show that surgical treatment for congenital mitral valve disease in the first year of life can be performed with low mortality. Valve repair is a realistic goal in about 70% of patients and possibly more with increased experience. Reoperation rate is still high and is related to complexity of mitral lesions and associated anomalies, but late functional results are encouraging. (J Thorac Cardiovasc Surg 1995;109:164-74)

OBJECTIVES: Right ventricular (RV) dysfunction and pulmonary insufficiency (PI) after tetralogy of Fallot (TOF) repair may contribute to early and late morbidity and mortality. RV dysfunction may be related to the ventriculotomy employed in the transventricular repair technique, particularly when it is combined with a transannular patch (TAP). Transatrial/transpulmonary (TA/TP) repair without ventriculotomy has been advocated as a method potentially diminishing such adverse events. However, the prevalence and early as well as the late results of these different surgical approaches in Europe have not been studied. To ascertain ‘the current prevalence and associated early mortality’ of various surgical approaches for repair of TOF, relevant data in the European Association for Cardio-Thoracic Surgery Congenital Database were analysed. METHODS: The study population was all types of reparative operations (n= 6654) for patients with primary diagnosis of TOF reported between 1999 and 2011. RESULTS: Overall hospital mortality (HM) was 2.58% (172 of 6654). Repair via ventriculotomy with TAP was the most prevalent technique (n = 3827, 57.5%), with HM 3.11%. Repair via ventriculotomy with non-TAP was performed in 1309 patients (19.7%, HM = 1.53%). Repair without ventriculotomy was performed in 1214 patients (18.2%, HM = 1.48%). Logistic regression analysis showed statistically significant association between HM risk and the type of surgery. In particular, ventriculotomy with TAP is associated with increased mortality risk significantly compared with ventriculotomy with non-TAP (crude odds ratio [OR] 2.13 (95% confidence interval [CI]: 1.33– 3.63). Similar results were obtained by analysing for operative mortality risk (30-day mortality, OM). Operations that have been performed before 2005 have resulted in increased surgical risk compared with those performed after 2005 (ORs for OM 1.45, 95% CI: 1.03–2.013). CONCLUSIONS: Overall HM for TOF repair is low. TOF repair by means of ventriculotomy with TAP is the most prevalent approach and is associated with higher mortality. Repair with ventriculotomy but no TAP and repair without ventriculotomy are both less prevalent and with lower mortality. Surgical risk appears to be decreasing over time.

Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate ( P= 0.006). Overall mortality was associated to malignancy ( P =0.0008), and adverse events during follow-up ( P =0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.

Between September 1, 1982, and March 1, 1992, 40 patients underwent anatomic repair of transposition of the great arteries, ventricular septal defect, and aortic arch obstruction. In group I, 26 patients (65%) underwent repair in a two-stage procedure, phases A and B. Phase A included repair of the aortic arch obstruction with (16 patients) or without (10 patients) pulmonary artery banding through a left thoracotomy (mean age 18.7 +/- 23.4 days). There were three deaths and three reoperations. Phase B included an arterial switch operation with closure of the ventricular septal defect (mean age 95.5 +/- 122 days). There were five early deaths and two late deaths. Eight patients required reoperation. Mean delay between phase A and phase B was 77.5 +/- 109 days. In group I, there were eight early and two late deaths, and 11 patients required reoperation. The mean stay in the intensive care unit was 24.7 +/- 20 days. Mean follow-up of 59.6 +/- 21.4 months was completed in all survivors. All but one were in New York Heart Association class I without medication. Actuarial survival rate and rate of freedom from reoperation at 5 years were 57.5% and 49.9%, respectively. In group II, 14 patients (35%) had a one-stage procedure through midsternotomy: an arterial switch operation with closure of the ventricular septal defect and repair of the aortic arch obstruction (mean age 10.2 +/- 5.5 days). There were two early deaths (14.2%) and one late death after reoperation for overlooked multiple ventricular septal defects. Two patients required reoperation. The mean stay in the intensive care unit was 11.7 +/- 2.5 days. Mean follow-up of 22.4 +/- 16.7 months was achieved in all survivors. They were all in New York Heart Association class I without medication. Actuarial survival rate and rate of freedom from reoperation at 3 years were 78.5% and 81.5%, respectively. The one-stage procedure allowed complete repair in neonates without the need for multiple operations. We believe that it may decrease early mortality rates (14.2% versus 30.7%), reduce the reoperation rate and cumulative stay in the intensive care unit (11.7 days versus 24.7 days, p = Not significant), and significantly decrease the overall rate of morbidity (p < 0.01). However, requirements for surgical intervention with a one-stage or a two-stage procedure must include accurate assessments of intracardiac and aortic arch anatomy.

We report a modification of the Norwood stage I procedure in a neonate with right aortic arch and complete atrio-ventricular canal in a variant of hypoplastic heart syndrome. Because of the unusual anatomy, the neo-aorta was reconstructed with a specially trimmed homograft patch and the pulmonary flow was maintained with implantation of a right ventricle to pulmonary artery shunt on the left side of the aorta. The patient had a favourable outcome and is now awaiting the stage II procedure.

The anomalous origin of the left subclavian artery is known to be associated with right aortic arch and tetralogy of Fallot. In our case, the left subclavian artery arose from the left pulmonary artery. Therefore, the left arm was perfused by poorly oxygenated blood from pulmonary arteries and some retrograde vertebral artery flow. Thus, the left arm was cyanotic and less developed than the right one. The patient underwent surgical repair with complete correction of tetralogy of Fallot and reimplantation of the left subclavian artery to the left carotid artery.

SummaryDuring a 10 year interval, between January 1, 1981 and January 1, 1991, primary repair of atrioventricular septal defect with a common atrioventricular orifice was performed in 95 patients younger than 1 year. Patients with atrioventricular septal defect having two atrioventricular orifices and a small ventricular septal defect, and those with severe hypoplasia of the left ventricle, were not included in the present study. According to the presence of associated anomalies, patients were divided into those with simple forms (72%) and those with complex forms (28%). These included parachute deformity of the left atrioventricular valve (10 cases), multiple muscular ventricular septal defects (5 cases), low-lying infundibular stenosis of the right ventricle (4 cases), obstruction of the left ventricular outflow tract (3 cases), tetralogy ofFallot (2 cases) and hypoplasia of the left ventricle (2 cases).

A transitory but significant stimulation of mitochondrial activity, increase of reactive oxygen species (ROS) and oxidative stress were previously observed in L929 fibroblasts cultured on poly(epsilon-caprolactone) (PCL) films. ROS, mainly formed in mitochondria, play a physiological role but an excessive production can promote endothelial dysfunction, cause oxidative injury to vascular cells, oxidize lipoproteins and accelerate atherothrombogenesis. On the other hand, mitochondria have a crucial position in programmed cell death control and are responsible for ATP synthesis through the coupling of oxidative phosphorylation to respiration. This coupling requires the existence of a mitochondrial membrane potential (Deltapsi(m)). The aim of the present study was to evaluate by flow cytometry the ROS content and Deltapsi(m) of both endothelial (EC) and smooth muscle cells (SMC) cultured on PCL films as a potential substrate for vascular graft development. Cell size, internal complexity and cell cycle were also analyzed to detect the possible appearance of the subG(1) cell fraction, characteristic of apoptotic cells. The effect of treating PCL films with NaOH before culture was also studied. PCL decreases the ROS content of EC during the culture but produces an increase of these levels in SMC after 7 days. PCL also induces variations of Deltapsi(m) which show a significant parallelism with the changes observed in ROS levels proving the importance and sensitivity of these measurements as indicators of the mitochondrial function. The treatment of PCL with NaOH decreases these effects demonstrating the benefits of increasing the surface hydrophilicity before cell culture which improves cell adhesion and proliferation and reduces oxidative stress. Since no important changes have been detected in subG(1) fraction of EC and SMC cultured on either PCL or PCL-NaOH, the changes of Deltapsi(m) observed in the present study cannot be related to apoptosis. These results confirm the potential utility of PCL as a suitable scaffold in Vascular Tissue Engineering.

OBJECTIVE: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation. METHODS: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded. RESULTS: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis. CONCLUSIONS: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.

An 11-month-old child underwent unifocalization of the major aortopulmonary collateral arteries, but did not tolerate occlusion of both vessels simultaneously. Using a Y-shaped homograft tube, we translocated the vessels sequentially and avoided severe hypoxemia.

OBJECTIVE: To assess the impact of the bidirectional cavopulmonary shunt, total cavopulmonary connection, and baffle fenestration on outcome of the Fontan operation in our unit.METHOD: We reviewed 123 bidirectional cavopulmonary shunts and 264 Fontan operations performed from 1980 to 1995. Analysis of pulmonary artery size (right and left main and lower lobe branches) before and after bidirectional cavopulmonary shunt was performed. Outcome of the Fontan operation was analysed in various time periods to assess the effect of prior bidirectional cavopulmonary shunt, use of the lateral tunnel, and fenestration.RESULTS: Operative risk for the bidirectional cavopulmonary shunt was 4% (CI = 2-10%) with a survival of 89% (CI = 83-95%) at 36 months. Probability of conversion to Fontan operation at 36 months was 49% (CI = 38-61%). Pulmonary artery size (Nakata and lower lobe indices) fell after bidirectional cavopulmonary shunt (P = 0.0006). Fontan risk dropped from 8.5% (1980-1987) to 1.8% (1988-1995) (P = 0.02), coinciding with the use of the bidirectional cavopulmonary shunt. There was no further risk reduction after introduction of the lateral tunnel and baffle fenestration, although these comparisons are limited by relatively small numbers. Duration of hospital stay related to pleural effusions was lowest for patients with a fenestrated lateral tunnel operation (P < 0.05).CONCLUSION: The bidirectional cavopulmonary shunt is a suboptimal stimulus for pulmonary artery enlargement, but may reduce the risk of Fontan operation in selected children. Fenestrated lateral tunnel operations have reduced the duration of postoperative pleural effusions. (Less)

From January 1985 to March 1992, 64 consecutive neonates with transposition of the great arteries (TGA) and ventricular septal defect (VSD) underwent an arterial switch operation and VSD closure. The mean age at operation was 18.5 +/- 12 days and the mean weight was 3.3 kg. Seventeen patients had an associated aortic coarctation, of whom 15 underwent single-stage repair through median sternotomy. Coronary artery distribution was: type A: 45 patients; type B: 2; type D: 11 and type E: 6 patients. The location of the VSD was perimembranous in 42 patients, trabecular in 13, infundibular in 5, and 4 presented with the Taussig Bing heart anomaly. The hospital mortality was 9.3% (n = 6). There were four late deaths (one TGA-VSD and three TGA-VSD and coarctation). Nine patients required reoperation. The mean follow-up of all survivors was 36 +/- 19 months. They were in NYHA class I without medication. Six patients developed mild-to-moderate aortic insufficiency. The actuarial survival and freedom from reoperation at 5 years were 81.06% and 84.6%, respectively. We conclude that neonatal anatomic repair of TGA and VSD offers good medium-term results and avoids iterative operations.

Objectives. This retrospective study attempts to assess the results of the neonatal anatomic repair of transposition of the great arteries by a single institution. Background. Anatomic correction of transposition of the great arteries by means of the arterial switch operation is now widely accepted as the therapeutic method of choice. Methods. Four hundred thirty-two consecutive neonates underwent an arterial switch operation for various forms of transposition of the great arteries. There were 362 neonates with transposition and intact ventricular septum, 47 with a ventricular septal defect, 6 with intact ventricular septum and coarctation of the aorta and 17 with ventricular septal defect and coarctation. Among patients with coarctation, 18 underwent a single-stage repair through median sternotomy. The mean age was 13.1 ± 4.2 days. Coronary artery distribution was described according to the origin and initial course of the arteries. Results. Overall in-hospital mortality was 7.8% (34 patients) and was 7.6% for transposition with intact ventricular septum, 8.5% for transposition with ventricular septal defect and 13.3% for transposition with ventricular septal defect and coarctation. Univariate analysis of risk factors revealed that coronary anatomy was the main determinant for operative survival. A mean follow-up time of 44 ± 19 months was achieved in all but five survivors. More than 95% were in New York Heart Association functional class I, without medication and with normal left ventricular function. Reoperation was performed in 20 patients: early reoperation (