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Introduction: The Glenn procedure is generally performed as a second-stage palliative procedure toward the completion of Fontan circulation in patients with a functionally univentricular heart. Safe Fontan completion requires normal ventricular function, competent atrioventricular valves, normal pulmonary arteries anatomy, and low pulmonary vascular resistances (PVRs), which is merely an estimation that considers both lungs as a single unit. Clinical observations revealed that patients who previously underwent bilateral bidirectional cavopulmonary anastomosis (b-BCPA) had a stormier postoperative course in respect to other patients with previous unilateral BCPA (u-BCPA) postoperatively after Fontan completion. This retrospective study was designed to compare and analyze the outcomes of patients following Fontan completion with previous u-BCPA versus b-BCPA. Patients and Methods: A total of 98 patients were divided into two groups based on the presence or absence of bilateral superior vena cava (SVC). Group 1 included 80 patients with unilateral SVC, and group 2 included 18 patients with bilateral SVC. Univariate and multivariate (SPSS) analyses were performed with regard to mortality and failure. P value of Results: Prior to the total cavopulmonary anastomosis operation, the G1 and G2 had similar demographic and physiological findings, ages and weights were 4.3 ± 1.72 years and 15.0 ± 3.71 kg in G1 and 4.8 ± 3.21 years and 17.5 ± 9.76 kg in G2. Mean end-diastolic ventricular pressures (in mm Hg) were 10.50 ± 2.86 in G1 and 10.28 ± 3.07 in G2 and the mean PVRs (in Woods units) were 1.66 ± 1.29 in G1 and 1.49 ± 0.82 in G2. The Stormy postoperative course, Fontan failure, and early mortality were all significantly higher in G2 in respect to G1 ( P value Conclusions: Our results have demonstrated that patients after b-BCPA might have a tendency for worse outcomes upon Fontan completion. The b-BCPA is associated with pulmonary artery bifurcation stenosis and presents multiple sources of pulmonary blood flow where conventional PVR calculation might be imprecise and misleading since it considers the lungs as a single unit. Therefore, b-BCPA engenders correction of the conventional PVR calculation to consider each lung separately. Furthermore, the surgical approach for patients with persistent left SVC and univentricular heart should be modified.

Objective The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase [AST]). We aimed to determine the contribution of blood products transfusion to acute hepatic injury. Design Single center retrospective cohort study. Setting Pediatric Cardiac Intensive Care Unit at a tertiary medical center. Patients Ninety-nine pediatric patients undergoing the Fontan procedure between January 2009 and December 2016. Interventions None. Measurements and main results Out of the four types of blood products, transfusion of platelets was found to significantly affect postoperative levels of ALT and AST. Additional factors included postoperative administration of sodium bicarbonate, decreased flow through the Fontan canal and decreased urine output. Preoperative pulmonary artery pressure and pulmonary vascular resistance, cardiopulmonary bypass time, aortic cross-clamp time, amount of postoperative bleeding, and vasoactive-inotropic score did not influence liver enzymes levels CONCLUSIONS: In pediatric Fontan patients, platelets transfusions contribute to an acute hepatic injury. The relation between platelets and transfusion-related acute lung injury (TRALI) has been well described, but this is the first time it is being described in regard to acute hepatic injury (TRAHI). Changing platelet transfusion strategy could decrease morbidity in Fontan patients but further research is needed.

Pain prevention in the pediatric population is mandatory and an integrative aspect of medical practice. Optimal pain management is the right of all patients and the responsibility of all health professionals. The key to adequate pain management is assessing its presence and severity, identifying those who require intervention, and appreciating treatment efficacy. The population of pediatric patients undergoing cardiac surgery is unique in both clinical severity and hemodynamic response to painful stimuli, thus making pain management even more challenging. In this review, we will describe the different pain assessment tools as well as intra- and postoperative regimens of pain management.

ObjectivesContinuous flow in Fontan circulation results in impairment of pulmonary artery endothelial function, increased pulmonary vascular resistance, and, potentially, late failure of Fontan circulation. Bidirectional cavopulmonary shunt is the interim procedure in palliation of patients with single-ventricle physiology, but pulmonary arteriovenous malformations occur in many patients. In a porcine chronic model of cavopulmonary shunt, we studied pulmonary hemodynamics, pulmonary arteriovenous malformation occurrence, and gas exchange capabilities. We hypothesized that residual antegrade pulsatile pulmonary flow may attenuate the deleterious effects of nonpulsatile Fontan-type circulation.MethodsThirty pigs underwent a sham procedure (n = 10, group I), a cavopulmonary shunt with right pulmonary artery ligation (n = 10, group II, nonpulsatile), or a cavopulmonary shunt with proximal right pulmonary artery partial ligation (n = 10, group III, micropulsatile). Three months later, in vivo hemodynamics, blood gas exchange, pulmonary arteriovenous malformation occurrence, and lung histology were assessed.ResultsAt 3 months, group II right lungs demonstrated significantly increased pulmonary artery pressure, pulmonary vascular resistance, and evidence of pulmonary arteriovenous malformations compared with groups I and III (all P

The objectives were to determine in patients with Tetralogy of Fallot (ToF) and abnormal coronary artery (ACA): the long-term outcomes of different surgical strategies; the risk factors for right ventricular outflow tract (RVOT) obstruction, reoperation, heart failure and mortality. To date, the surgical strategies and prognostic factors for repair of ToF with an ACA, crossing the RVOT and avoiding a classic repair, have not been evaluated in a large series using a multivariate analysis.A retrospective study (1986-2011) included 72 patients. The mean follow-up was 9.6 ± 6.8 years. Median age at surgery was 1.5 years (0.2-11.3). The main surgical techniques were 'tailored' right ventriculotomy and patch of the RVOT (63%; n = 45), implantation of a conduit between the right ventricle (RV) and the pulmonary artery (PA; 25%; n = 18) and a transatrial ± transpulmonary approach (11%; n = 8). Univariate and multivariate logistic regression analyses were performed.Intrahospital mortality was 2.7%. Actuarial freedom from reoperation and actuarial survival at 15 years were 77% (confidence interval [CI]: 70-83%) and 94% (CI: 90-97%), respectively. Reoperations occurred more frequently after conduit implantation (50%) than after patch reconstruction (17%) or transatrial ± transpulmonary approach (0%; P = 0.002). The transatrial ± transpulmonary approach was significantly less complicated, with a long-term RVOT obstruction of 0% compared with the other surgical techniques (45.4%; P = 0.03). Implantation of a RV-PA conduit was an independent risk factor for RVOT obstruction (odds ratio [OR]: 31; P0.001) and reoperation (OR: 20; P = 0.02). An immediate postoperative right ventricle/left ventricle (RV/LV) pressure ratio0.5 was independently associated with a long-term RV/LV pressure ratio0.5 (OR: 14; P = 0.001), but was not a risk factor for reoperation (P = 0.8). Postoperative electric ischaemic signs independently increased the risk of long-term heart failure (OR: 22; P = 0.04).The transatrial ± transpulmonary approach displays the best long-term outcomes, by reducing the risks for RVOT obstruction and reoperation, but does not improve the patient survival. A RV-PA conduit was an independent risk factor for RVOT obstruction and reoperation. An immediate postoperative RV/LV pressure ratio0.5 was not a risk factor for reoperation. The transatrial ± transpulmonary approach should be preferred to the implantation of a conduit or a tailored right ventriculotomy whenever possible.

Cardiac output (CO), the product of stroke volume (SV) and heart rate, is essential to guarantee organ perfusion, especially in the intensive care setting. As invasive measurement of CO bears the risk of complications there is a need for non-invasive alternatives. We investigated if electrical velocimetry (EV) and transthoracic Doppler (Doppler-TTE) are interchangeable for the non-invasive measurement of SV and able to reflect the post-surgical SV/CO trend.Comparison of SV measurements by EV and Doppler-TTE was performed in 24 newborns after switch operation (n = 240 measurements). Three subgroups of measurements (=periods) were created according to the patients' status in the course of post-surgical CO recovery.Bland-Altman analysis found acceptable bias and limits of agreement for the interchangeability of the two methods. Mean overall SV was 3.7 ml with a mean overall bias of 0.28 ml (=7.6 %). The mean percentage error of 29 % was acceptable according to the method of Critchley and Critchley. Overall precision expressed by the coefficient of variation (CV) was 6.6 % for SV(TTE) and 4.4 % for SV(EV). SV(TTE) and SV(EV) medians in the three periods were significantly different and documented the post-surgical CO trend.EV and Doppler-TTE are interchangeable for estimating SV. EV has the advantages of easy handling and allows continuous measurement.

ObjectivePatients with idiopathic pulmonary hypertension are at risk for right-sided heart failure and sudden death. Despite improvement in pharmacologic management, some still require lung transplantation. Potts anastomosis has been demonstrated as a good palliation in children to alleviate symptoms and medical therapy despite desaturation in the lower part of the body. Young adult patients with pulmonary hypertension and isosystemic pressure remain at risk, particularly at exercise. The goal of this research was to find a palliation for patients in whom suprasystemic pulmonary hypertension developed at exercise. Creating a Potts anastomosis involved a unidirectional valve between the left pulmonary artery and the descending aorta.MethodsExperimental study was performed on 14 pigs. A prosthetic patch of polytetrafluoroethylene (Gore-Tex; WL Gore & Associates Inc, Newark, Del) was used to create the unidirectional valve and implanted in the Potts anastomosis. Via a left thoracotomy, an aorto-aortic shunt between the aortic isthmus and the distal descending thoracic aorta was instituted, allowing a safe surgical procedure. Intrapulmonary injection of Erciplex glue (Peters Surgical, Bobigny, France), diluted in 70% alcohol, was used to create acute pulmonary hypertension. The right to left shunt across the unidirectional valvular patch was evaluated after clamping the aorta in the acute phase of pulmonary hypertension by echo-pulsed Doppler at the level of the descending thoracic aorta by withdrawal of blood gas (arterial carbon dioxide tension, alveolar carbon dioxide tension) and assessment of peripheral oxygen saturation. Similar reevaluation of the shunt was performed at a mean interval of 13 ± 2.5 weeks.ResultsIn the first series, Erciplex glue increased pulmonary artery pressure from 15.3 ± 3.1 mm Hg to 38.7 ± 6.0 mm Hg. Mean peripheral oxygen saturation decreased from 100% to 85% ± 1.5%. Mean partial pressure of carbon dioxide increased from 31.9 ± 9.1 mm Hg to 46.2 ± 12.5 mm Hg after shunt opening (P

Heterotaxy syndrome (HS) is generally associated with complex congenital cardiac defects and has a high morbidity and mortality despite recent surgical progress. Only few reports deal with an overall surgical population.Between 1989 and 2008, 139 patients with HS entered a programme of surgical repair. Fifty-one patients were suitable for univentricular heart repair (UVR) and 88 for biventricular repair (BVR). Among those tracked for UVR, two were switched to BVR and 11 from BVR to UVR. Median age at first surgery was 4.4 months (range: 3 days to 43 years] of whom 34 were neonates. The mean number of surgical procedure per patient was 1.99. Primary BVR was performed in 37 patients. Re-operation was required in 22 patients, 15 after BVR and seven after UVR (p0.05).The overall mortality was 20.8%. It was 7.2% after the first surgery, 6.6% after the second and 11.5% after the third. The overall mortality in patients with univentricular physiology was 25.5% and 18.2% in the biventricular group (p0.05). According to the surgical track, in the UVR group, mortality was 18% and 15.6% in the BVR group (p=NS). This rate was 40% in patients with long-lasting palliation (p0.05 vs both other groups). Median follow-up was 127 months (range: 1 month to 19 years). The overall survival rate at 15 years was 70.6%. When considering ventricular anatomy, survival rates at 15 years were 69% for the univentricular group and 74.2% for the biventricular group (p0.05). According to the type of surgical approach, at 15 years they were 85.1% for UVR and 77% for BVR (p=NS). For the palliation group, it was 15% only at 15 years (p0.05 vs both other groups). Risk factors for overall mortality were neonatal surgery, long-standing palliation, total anomalous pulmonary vein return (TAPVR) and right ventricular outflow tract obstruction (RVOTO). At the last visit, all survivors were in the New York Heart Association (NYHA) class I to II and only two presented with supraventricular arrhythmias.HS remains a difficult situation with high morbidity and mortality. An aggressive approach to repair TAPVR when present should be considered. Early decision to track the patient in either uni- or biventricular repair programme should avoid long-lasting deleterious palliation.

Repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) carries a high risk of operation, particularly in those with poor left ventricular function. In this study, we assessed the outcomes of patients who presented with severe preoperative left ventricular dysfunction (shortening fraction15%) who underwent the repair under normothermic bypass.Since September 2002, 21 infants with severe left ventricular dysfunction underwent surgical repair using a direct coronary reimplantation technique. Mean age of patients was 5.3 +/- 3.8 months. Moderate or severe mitral regurgitation present in 5 patients was not addressed at the operation. Repair was performed under normothermic cardiopulmonary bypass and myocardial protection was achieved by intermittent antegrade normothermic blood cardioplegia.Mean cardiopulmonary bypass and cross-clamp times were 153.5 +/- 51 and 50 +/- 14.5 minutes, respectively. There was one (4.7%) early death because of sudden cardiac arrest 48 hours after successful weaning from mechanical ventilation. One patient required urgent left coronary artery stenting on the 34th postoperative day because of early reimplantation failure. There was no late death. Echocardiographic evaluation revealed a significant improvement in terms of shortening fraction (preoperative, 10.3 +/- 3.2% vs follow-up, 33 +/- 7.7%, p0.0001).Our results suggest that the repair of anomalous origin of the left coronary artery from the pulmonary artery can be accomplished with acceptable mortality and morbidity rates even in patients having severely depressed left ventricular function. Normothermic cardiopulmonary bypass is safe and effective in children who have a limited tolerance to further ischemic insult.

Background The surgical reconstruction of right ventricle outflow tract (RVOT) often requires the implantation of a valved conduit. Homografts are lacking availability and are associated with limited durability in children. Our experience with the Hancock porcine-valved Dacron (DuPont, Wilmington, DE) conduit (Medtronic, Minneapolis, MN) was retrospectively assessed. Methods Follow-up was studied in 214 survivors who underwent 247 conduit implants between January 1990 and January 2007. Pulmonary atresia/ventricular septal defect was present in 86 (40.2%) and truncus arteriosus in 62 (29%). Conduit implantation was associated with anatomic repair in 136, conduit replacement in 96, and secondary pulmonary valve insertion in 15. Median age at operation was 62.5 months (range, 1 week to 50 years), including 14 neonates (6%). Median conduit size was 17.4 mm because of routine over-sizing. Pulmonary bifurcation patch augmentation was necessary in 26 patients. Periodic echocardiography studies were performed for a median follow-up of 98 months (range, 13 to 142 months). Results Three (1.4%) late deaths occurred. No conduit-related deaths or complications occurred. Conduit degeneration was associated with increase in valvular gradient. Valve regurgitation was absent or mild. Higher RVOT systolic pressure gradient at discharge did not influence conduit longevity. Conduit reoperation was delayed due to percutaneous balloon dilatation in 14 patients, associated with stenting in 7. Survival with freedom from conduit reoperation was 98% (95% confidence interval [CI], 97% to 100%) at 1 year, 81% (95% CI, 75% to 87%) at 5 years, and 32% (95% CI, 22% to 42%) at 10 years. Conclusions The Hancock valved conduit is a safe and reliable alternative to homografts. It appears to be appropriate in patients with limited pulmonary vascular bed and high pulmonary artery pressures. Caution is required in neonates because of the rigidity of the Dacron housing. Initial results with secondary percutaneous procedures are encouraging.

Guidelines summarize and evaluate all currently available evidence on a particular issue with the aim of assisting physicians in selecting the best management strategies for an individual patient, suffering from a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines are no substitutes for textbooks, and their legal implications have been discussed previously. Guidelines and recommendations should help physicians to make decisions in their daily practice. However, the ultimate judgement regarding the care of an individual patient must be made by his/her responsible physician(s). A large number of Guidelines have been issued in recent years by the European Society of Cardiology (ESC) as well as by other societies and organizations. Because of the impact on clinical practice, quality criteria for the development of guidelines have been established in order to make all decisions transparent to the user. The recommendations for formulating and issuing ESC Guidelines can be found on the ESC Web Site (http://www.escardio.org/guidelines/rules). Members of this Task Force were selected by the ESC to represent all physicians involved with the medical care of patients in this pathology. In brief, experts in the field are selected and undertake a comprehensive review of the published evidence for management and/or prevention of a given condition. A critical evaluation of diagnostic and therapeutic procedures is performed, including assessment of the risk–benefit ratio. Estimates of expected health outcomes for larger populations are included, where data exist. The level of evidence and the strength of recommendation of particular treatment options are weighed and graded according to pre-defined scales, as outlined in Tables 1 and 2 . View this table: Table 1 Classes of recommendations View this table: Table 2 Levels of evidence The experts of the writing and reviewing panels have provided disclosure statements of all relationships they may have which …

Objectifs La tracheoplastie de glissement ou « slide tracheoplasty » est progressivement devenue la technique de choix pour le traitement des hypoplasies etendues de la trachee chez l’enfant et le nourrisson. Cependant, certaines situations rendent difficile cette technique et l’hypoplasie est alors le plus souvent corrigee par mise en place de materiel etranger. Materiel et methodes Cinq patients âges de 1 a 6 mois presentant des hypoplasies complexes de trachees ont ete operes entre 2001 et 2005 par notre equipe. L’hypoplasie etait dite complexe car associee a une stenose critique, une stenose du cricoide, une hypoplasie des bronches, une bronche tracheale ou encore une atresie de l’oesophage avec tracheomalacie severe. Tous ont ete traites par des variantes de « slide tracheoplasty ». Resultats En postoperatoire, la duree moyenne de ventilation a ete de 8 jours et la duree moyenne de reanimation de 15 jours avec une mortalite nulle. Le principal facteur de morbidite a ete une paralysie recurentielle unilaterale chez 2 patients. A long terme (suivi moyen de 27 mois), aucun enfant n’a du subir de reintervention, tous presentent une croissance et une activite normales. Conclusions La slide tracheoplasty represente une bonne alternative therapeutique en cas d’hypoplasie severe et complexe de la trachee chez le nourrisson.

Objectives The aims of this study were to assess the prevalence and incidence of aortic valve regurgitation (AR) after arterial switch operation (ASO), its outcome, and the risk factors. Background After an ASO, the long-term fate of the aortic valve is a concern as follow-up lengthens. Methods Operative and follow-up data on 1,156 hospital survivors after ASOs between 1982 and December 2000 were reviewed. Results At last follow-up (mean duration 76.2 ± 60.5 months), 172 patients (14.9%) had an AR. Complex transposition of the great arteries, prior pulmonary banding done in 75 patients (21 with intact ventricular septum), aortic arch anomalies, AR at discharge, older age at ASO, and aortic/pulmonary size discrepancy were associated with AR. On multivariate analysis, the presence of a ventricular septal defect (VSD) or AR at discharge multiplied the risk by 2 and 4, respectively. Freedom from AR was 77.9% and 69.5% at 10 and 15 years, respectively; hazard function for AR declined rapidly and slowly increased thereafter. Reoperation from AR was done in 16 patients with one death, valvuloplasty being unsuccessful. Freedom from reoperation for AR was 97.7% and 96.8% at 10 and 15 years, respectively; hazard function slowly increased from 2 to 16 years. Higher late mortality was not associated with AR. Conclusions After ASO, AR was observed and was related to VSD with attending high pressure and flow and AR at discharge. Progression of AR was slow, but incidence increased with follow-up. Reoperation for AR was rare. Late aortic valve function warrants long-term monitoring.

Objectives Surgical management of airway compression of vascular origin requires an accurate analysis of anatomy and various mechanisms of compression. This study assessed the usefulness of 3-dimensional computed tomographic scanning in the preoperative and postoperative evaluation of airways compression in a pediatric population. Methods Thirty-seven consecutive patients (median age, 4 months) were examined with multislice 3-dimensional computed tomographic scanning: 18 patients before surgical treatment of anomalies of vascular rings, 2 patients because of respiratory symptoms after repair of esophageal atresia, and 17 patients because of persisting respiratory symptoms or prolonged mechanical ventilation after cardiac surgery for congenital heart disease. Results The procedure was successful, with high-quality diagnostic imaging obtained in all cases without any complications. The anatomy and relationship between the vascular arches and airways was analyzed in all referred patients with vascular arch anomalies confirmed on the basis of the surgical findings, and this helped the surgeon to plan the procedure and choose the best approach. After cardiac surgery, the airway and vascular structures involved and the mechanism of compression were specified in all but one case, and the 3-dimensional computed tomographic scan serves as an important tool for deciding whether to perform reoperation on patients requiring prolonged mechanical ventilation. Conclusion Three-dimensional computed tomographic scanning is a safe, fast, and noninvasive method useful for accurately analyzing the mechanisms of airway compression of vascular origin and thus possible improving the surgical management of pediatric patients.

Congenitally corrected TGA (CC-TGA) is characterized by discordant atrioventricular and ventriculo arterial connections. In absence of right ventricular outflow tract obstruction (RVOTO), repair by atrial and arterial switches remains a challenging procedure for which long term follow-up is uncertain.From 1995 to 2007, 20 patients (median age: 26 months) with CC-TGA had double switch procedure. Segmental anatomy was {SLL} in all patients, dextrocardia in two patients, mesocardia in two patients. Ventricular septal defect was present in 17 patients, aortic coarctation in 2 patients and interrupted aortic arch (IAoA) in 1 patient. Five patients had tricuspid valve regurgitation. Six patients had AV blocks, 4 patients had pacemaker implantation prior to repair. Pulmonary artery banding was performed in 17 patients, for congestive heart failure (14 patients) or left ventricular retraining (3 patients). Three patients, including one patient with IAoA had primary repair. After LV retraining, repair was performed when indexed LV mass to LV volume ratio was above 1.5. A median follow-up of 60 months was achieved in all.There were no deaths. Postoperative pacemaker implantation was required in four patients. Reoperation for Senning obstruction was necessary in one patient, and pacemaker battery replacement in another patient. One patient had mild neoaortic insufficiency, two had mild tricuspid regurgitation and two had mild mitral regurgitation. All were in NYHA I-II. Actuarial survival at 10 years was 100% and freedom from reoperation at 5 and 10 years were 93% and 77.4%, respectively.Double switch for CC-TGA without RVOTO can be performed with no mortality and low morbidity. Since these results seem to last for several years, it should be considered as the optimal procedure.

Background . In the pediatric population, glutaraldehyde-preserved bovine pericardium conduit containing a stentless porcine valve has been proposed as an alternative to homografts for right ventricular outflow tract reconstruction. Methods . Between June 1996 and March 2000, a total of 55 patients, 20 with truncus arteriosus, 21 with pulmonary atresia with ventricular septal defect, and 14 with miscellaneous defects, received this conduit. Median age at implantation was 3.4 months (range, 3 days to 19 years), and 27 patients (50%) were less than 3 months old. Clinical outcome, echocardiographic data, and pathologic analysis were recorded. End points for conduit failure were conduit replacement or dilation. A mean follow-up of 27 months (range, 2 to 46 months) was available for 47 survivors. Results . Procedure for conduit obstruction was required in 13 patients. The most common procedure was operation, and all but 3 patients had an unsuccessful balloon angioplasty before reoperation. Actuarial freedom from conduit dilation or reoperation was 93.6% (95% confidence limits, 82% to 99%), 81.9% (95% confidence limits, 64% to 91%), 77.8% (95% confidence limits, 39% to 78%), and 64.3% (95% confidence limits, 26% to 73%) at 1, 2, 3, and 4 postoperative years, respectively. Univariate analysis identified small conduit size as a risk factor for conduit obstruction. Conclusions . Although this new conduit was not free from progressive obstruction, our clinical results (easy to work and good valvular function) and the availability in small sizes encouraged us to use it as an alternative to small-size homografts when those were not available.

International audience; The thymus plays a primary role in early-onset Myasthenia Gravis (MG) mediated by anti-acetylcholine receptor (AChR) antibodies. As we recently showed an inflammatory and anti-viral signature in MG thymuses, we investigated in detail the contribution of interferon (IFN)-I and IFN-III subtypes in thymic changes associated with MG. We showed that IFN-I and IFN-III subtypes, but especially IFN-beta, induced specifically alpha-AChR expression in thymic epithelial cells (TECs). We also demonstrated that IFN-beta increased TEC death and the uptake of TEC proteins by dendritic cells. In parallel, we showed that IFN-beta increased the expression of the chemokines CXCL13 and CCL21 by TECs and lymphatic endothelial cells, respectively. These two chemokines are involved in germinal center (GC) development and overexpressed in MG thymus with follicular hyperplasia. We also demonstrated that the B-cell activating factor (BAFF), which favors autoreactive B-cells, was overexpressed by TECs in MG thymus and was also induced by IFN-beta in TEC cultures. Some of IFN-beta effects were down-regulated when cell cultures were treated with glucocorticoids, a treatment widely used in MG patients that decreases the number of thymic GCs. Similar changes were observed in vivo. The injections of Poly(I:C) to C57BL/6 mice triggered a thymic overexpression of IFN-beta and IFN-alpha 2 associated with increased expressions of CXCL13, CCL21, BAFF, and favored the recruitment of B cells. These changes were not observed in the thymus of IFN-1 receptor KO mice injected with Poly(I:C), even if IFN-beta and IFN-alpha 2 were overexpressed. Altogether, these results demonstrate that IFN-beta could play a central role in thymic events leading to MG by triggering the overexpression of alpha-AChR probably leading to thymic DC autosensitization, the abnormal recruitment of peripheral cells and GC formation.

Objective: Arterial switch is the operation of reference for the surgical treatment of transposition of the great arteries. In cases of late referral, perinatal complications or early left ventricular (LV) dysfunction, the one stage arterial switch is contra indicated. Anatomical repair remains possible in these patients following a LV retraining. Methods: From January 1992 to January 2000, a LV retraining was attempted in 22 patients with transposition of the great arteries with intact ventricular septum (TGA IVS), whereas 470 direct arterial switch and 2 Senning were performed. Indication for LV retraining was based on a combination of factors including: an age older than 3 weeks, a ‘banana shape’ aspect of the inter-ventricular septum and mainly a LV mass ,35G/m 2 . Results: The mean age at LV retraining was 3.2 months ranging from 9 days to 8 months. Usually conducted by sterntomy, it associated a loose PA banding with a LV/RV at 65% with a systemico-pulmonary shunt. The first stage was associated with frequent LV dysfunction and the LV retraining was discontinued in two patients in favor of one Senning and one early switch followed by ECMO. One patient died at first stage from a mediastinitis. Nineteen patients underwent a second stage arterial switch that was performed when the LV mass had reached 50 G/m 2 after a mean delay of 10 days, ranging from 5 days to 6 weeks. After a mean follow up of 25 months, there was one non-cardiac late death. The 17 patients followed and leaving with an arterial switch are in NYHA class I, with a mean LV shortening fraction of 39%. Conclusions: Arterial switch following LV retraining in TGA IVS is a satisfactory option. The inferior limit of 35 G/m 2 adopted, to indicate LV retraining, seems a safe landmark. The quality of the myocardium generated and the respective roles played by the LV afterload, LV wall shear stress, LV inflow and outflow to induce the LV remodeling remain under debate. q 2001 Elsevier Science B.V. All rights reserved.

Double-outlet right ventricle (DORV) is a heart malformation that describes an anomalous ventriculoarterial connection which can be associated with considerable variant of associated lesions. When this malformation is present with two ventricles, biventricular repair is feasible in the vast majority of cases. This report describes the surgical techniques for biventricular repair in all forms of encountered DORV, as well as the surgical strategy employed at our institution. Copyright 2000 by W.B. Saunders Company

Mortality in children with idiopathic pulmonary arterial hypertension is high, emphasizing the need for novel therapeutic approaches. A surgical approach consisting in the creation of anastomosis between descending aorta and left pulmonary artery, the Potts shunt, has been proposed to decompress right ventricle. We reported two cases of severe idiopathic pulmonary hypertension in children with right heart failure refractory to medical treatment who benefited of Potts shunt.

Slide tracheoplasty can be applied successfully to all types of long segment congenital tracheal stenosis. In case of short segment tracheal stenosis, direct resection and anastomosis is often preferred. We report a case illustrating a new side benefit of the slide tracheoplasty in a patient presenting a relatively short hypoplastic tracheal segment coexisting with tracheomalacia after EA with TEF repair.

Malformation of the aortic arch system has been described in details by Stewart et al. in 1964. Innominate artery originating via the ductus arteriosus from the pulmonary artery is a very rare type of congenital aortic arch anomaly that has been seldomly reported. We report the case of an aortic arch anomaly revealed by a pulmonary hypertension because of left to right shunt. Surgical procedure was performed through a median sternotomy, without cardiopulmonary bypass. After section. of the ductus arteriosus, the left innominate artery was extensively dissected and mobilized to be implanted on the left side of the ascending aorta under lateral clamping.

Objective: The presence of associated multiple ventricular septal defects (VSDs) increases the risk of the anatomic repair for transposition of the great arteries (TGA). The aim of this study was to define the optimal management of this complex anomaly. Methods: Between January 1988 and December 1998, 45 patients underwent anatomic repair of TGA associated with multiple VSDs. The median age was 50 days and the median weight 4 kg. Eighteen (40%) had undergone previous palliation including 17 pulmonary artery banding procedure (PAB), seven associated with coarctation repair and one isolated coarctation repair. The perimembraneous septum was involved in 24 patients, the trabecular in 43, the inlet in seven and the infundibular in two. Closure of the VSDs included Dacron or pericardial patchs and matress sutures. The initial approach was through right atriotomy which was sufficient in 15 patients. VSDs were closed through right ventriculotomy in 13 patients, through pulmonary artery in six, through the aorta in one and in the remaining (na 10) combined approaches were used. Only one patient required left apical ventriculotomy. Results: There were five hospital deaths (11%; 70% CL: 6‐18%) including the one early reoperation for residual VSD closure. Five patients had successful early reoperation for secondary PAB for residual VSD. Three late deaths occurred (7%; 70% CL: 3‐13%). At the last visit, 95% of survivors were asymptomatic and without any cardiac medication. Conclusion: Mid-term survival with good quality of life can be achieved following either one or two-stage repair of this complex anomaly. In the presence of VSD closure failure a secondary PAB may be the procedure of choice. q 1999 Elsevier Science B.V. All rights reserved.

Objective: The term non-committed was used to define hearts in which the VSD was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. We report our experience of the surgical management of this subset, considered being of particular surgical relevance. Methods: Between January 1987 and December 1997, 23 patients having double-outlet right ventricle (DORV) with non-committed VSD underwent biventricular repair. Nine (39%) had undergone previous palliation. The median age was 20 months and the median weight was 8.5 kg. Two main types of repair were used: intraventricular baffle repair (n = 21) and arterial switch operation with VSD to pulmonary artery baffle (n = 2). At repair, 12 (52%) patients required concomitant VSD enlargement. In two other patients presenting with restrictive inlet VSD associated with tricuspid attachments, crossing the subaortic pathway biventricular repair was abandoned at operation. Results: There were two hospital deaths (9%, 70% CL: 3-19%). Eight patients (35%, 70% CL: 23-48%) underwent nine reoperations, six for subaortic stenosis. No late death occurred. At last visit, all patients were asymptomatic and only two had cardiac medication. Conclusions: The biventricular repair of DORV with non-committed VSD is feasible in the vast majority of cases with comparable results to other subsets of DORV. After repair, the subaortic region is at risk for development of subaortic stenosis.

Fas, a cell surface receptor, can induce apoptosis after cross-linking with its ligand. We report that Fas antigen is constitutively expressed in medullary epithelial cells of the human thymus. Expression is decreased in cultured thymic epithelial cells (TEC), similarly to HLA-DR antigen. TEC are resistant to anti-Fas–induced apoptosis after 4 days of primary culture, and this resistance is reversed by concomitant addition of cycloheximide. Cycloheximide also downregulated the expression of Fas-associated phosphatase-1, which has been found to inhibit Fas-induced apoptosis. This phosphatase could be involved in the resistance to Fas-induced apoptosis observed on day 4 of TEC culture. When TEC were subcultured after 10 to 13 days of primary culture, exposure to interleukin-1-β, tumor necrosis factor-, and interferon-γ, alone or together, reinduced Fas mRNA and protein expression. In coculture with activated thymocytes, TEC also upregulated Fas protein expression. Cytokine-activated TEC became sensitive to apoptosis induced by an agonistic anti-Fas antibody. This apoptosis was inhibited by Z-VAD-fmk but not by Z-DEVD-fmk and DEVDase activity was slightly increased in Fas-stimulated TEC, suggesting that DEVDase activity is not sufficient to induce TEC apoptosis. Taken together, these data show that the Fas receptor is expressed in medullary epithelial cells of the human thymus and is able to induce apoptosis.

Endothelium dysfunction with severe pulmonary hypertension may occur after total cardiopulmonary bypass (CPB) in infants as a result of a widespread inflammatory response. The aim of this study was to separate out the effects of lung ischemia-reperfusion from membrane oxygenator-induced activation of leukocytes on the function and viability of the pulmonary and systemic endothelia in neonatal piglets submitted to 90-min total CPB followed by 60-min reperfusion or in sham animals. Hemodynamics, gas exchange, endothelial-dependent relaxation in pulmonary and femoral arteries, and lung and skeletal muscle myeloperoxidase activity were assessed before, during, and after CPB, i.e., after reperfusion. Pulmonary and aortic endothelial cells and circulating leukocytes were harvested to assess reperfusion-induced changes in endothelial cells' viability and proliferation, and leukocyte-endothelial cell adhesion and cytotoxicity. Gas exchange worsened after reperfusion with pulmonary hypertension, increase in lung but not skeletal myeloperoxidase, and reduction of endothelial-dependent relaxation in pulmonary but not femoral arteries. After reperfusion, viabilities of pulmonary and aortic endothelial cells were reduced to 50%, endothelial cell growths were faster in pulmonary arteries than aorta, and leukocyte-pulmonary endothelial cell adhesion and cytotoxicity increased. These results suggest that in total CPB lung ischemia-reperfusion aggravates the inflammatory response and predisposes the lung endothelium to leukocyte-mediated injury.

We describe two cases of infectious endocarditis in infants complicated by aortic root abscess involving the coronary artery treated by the Ross operation. Multislice computed tomography was important in defining the diagnosis and in planning subsequent surgical management.

Congenital tracheal stenosis is an uncommon but life-threatening condition whose management is still debated. The rarity of this disease explains the lack of a standard management. Between 1986 and 1996, eight children younger than 1 year were referred to our Institution with a tracheal stenosis. The median age at operation was 3.15 months and the median weight was 4.5 kg. The diagnosis was made after an episode of respiratory distress in all but one and was confirmed by fiberoptic bronchoscopy. The median length of tracheal stenosis was 24.5 mm (Ranges: 4-30 mm). Only one patient was free from associated cardiovascular defect. Tracheal repair was performed under cardiopulmonary bypass in all. In three it was achieved by pericardial augmentation of the stenosed area, in four by resection and end to end anastomosis and in one by sliding tracheoplasty. Concomitant cardiac repair was performed in six. Two patients died after pericardial patch augmentation. In both, death was related to profound hypoxemia due to patch collapse. Two patients developed restenosis after resection and end to end anastomosis. They both had stent placement and one required reoperation and underwent a sliding tracheoplasty. At a median follow-up of 21 months (Ranges: 6-120) all the survivors are doing well and are free from respiratory symptoms. Bronchoscopic evaluation revealed in all a widely patent anastomosis without restenosis. In conclusion, tracheal stenosis in children remains a challenging lesion. Surgical technique, whether resection and end to end anastomosis or sliding tracheoplasty offer better results and should be discussed according to the length of the stenosis. Pericardial plasty should be used with caution.

To the Editor: A three-week-old boy was admitted to the hospital because of cardiac failure with diffuse ventricular hypocontractility and normal cardiac anatomy on echocardiography. Coronary and v...

OBJECTIVES To examine whether preoperative heart failure and cardiac surgery influence nitric oxide production and atrial natriuretic peptide (ANP) biological activity in infants and whether nitric oxide and ANP participate in the control of postoperative pulmonary vascular tone. DESIGN Prospective, clinical study. SETTING Tertiary pediatric cardiac intensive care unit in a referral cardiosurgical center. PATIENTS Nineteen infants (median age 4 months) undergoing cardiac surgery: 13 infants with ventricular or atrioventricular septal defect associated with heart failure and pulmonary hypertension (group 1); and six infants with tetralogy of Fallot, without heart failure (group 2). INTERVENTIONS Blood samples obtained from indwelling catheters or bypass circuit outlets. MEASUREMENTS AND MAIN RESULTS Nitrite and nitrate blood concentrations (as a marker for nitric oxide synthesis) and the molar ratio of cyclic guanosine 3',5'-monophosphate (cGMP) to ANP (as a marker for ANP biological activity) were determined before, during, and up to 24 hrs after cardiopulmonary bypass (CPB). In group 1 patients, these biological parameters were related to postoperative pulmonary arterial pressure. Preoperative nitrite and nitrate concentrations were higher in group 1 patients than in group 2 patients (p < .02), and this difference persisted during CPB. Nitrite and nitrate concentrations 24 hrs postoperatively were lower than preoperative values in group 1 patients (p < .05) and were unchanged in group 2 patients. An inverse correlation was observed postoperatively between nitrite and nitrate concentrations and systolic pulmonary arterial pressure (r2 = 0.4, p < .05). Group 1 patients had a lower preoperative cGMP/ANP ratio than group 2 patients (p < .05), despite higher ANP levels (p < .005). The cGMP/ANP ratio decreased during CPB in both groups (p < .0001), and in group 2 patients, cGMP and ANP values remained below preoperative values < or = 24 hrs postoperatively. A correlation was observed between ANP levels and systolic pulmonary arterial pressure 2 and 4 hrs postoperatively (r2 = .4, p < .05, respectively), but no correlation was observed between ANP biological activity and postoperative pulmonary arterial pressure. CONCLUSIONS Infants with heart failure and pulmonary hypertension have increased nitric oxide synthesis and decreased ANP biological activity; both phenomena may be involved in the pathophysiology of this clinical condition. CPB has no detectable effect on nitric oxide production but does decrease ANP biological activity. In patients with preoperative heart failure and pulmonary hypertension, endogenous nitric oxide appears to play a role in the control of postoperative pulmonary vascular tone.

The Fontan operation was initially designed to provide a “physiological correction” of tricuspid atresia, but its indications have been extended to more complex forms of congenitally malformed hearts. In selected patients with two adequately sized ventricles, but highly complex intra cardiac anatomy which makes a biventricular repair more hazardous and which would require multiple reoperations, a univentricular repair using Fontan's principle has been proposed. Between 1978 and 1992, a modified Fontan operation was carried out in 34 such patients. Of the patients. 13 had double outlet right ventricle and 21 had transposed great arteries. Anomalies of ventricular loop were present in 14 patients. A non-committed ventricular septal defect was found in 26 whilst multiple defects were present in 10. All patients had anomalies of the atrioventricular valves, with straddling of the tricuspid valve,11and/or of the mitral valve11, and abnormal tricuspid5, or mitral4cordal attachments. Two patients had mild tricuspid hypoplasia, 1 had mitral hypoplasia and 3 had a cleft mitral valve. Fourteen patients had anomalies of both atrioventricular valve but only 6 had mild atrioventricular valvar incompetence. Pulmonary stenosis or atresia was seem in 26. All the patients had at least one or more (mean 2.35) intracardiac anomalies that preculded a biventricular repair. The Fontan operation was performed at a median age of 4.75 years. There were 4 early (11.4%, 70% CL: 5.1–19.8) and 2 late deaths, with a 4 year actuarial survival rate of 79.6±8.1%. Univariate analysis did not reveal any risk factor for early of late death. Follow-up at a mean interval of 3.6 ±2.6 years was available in all but 1 survivor. There were 6 early and 2 late reoperations, and 3 patients required implantation of a pacemaker. In conclusion, the modified Fontan operation can provide satisfactory early and mid term results in patients with two ventricles but with complex intracardiac anatomy which precludes a biventricular repair.

Objecti6e: Atrio ventricular septal defects (AVSD) with normal caryotype represent in average 25% of AVSD. They constitute a more complex group of patients characterized by frequent left sided heart obstructive lesions, raising the problem of the appropriate indications between biventricular and univentricular procedures. Methods: Sixty-nine consecutive patients, who had AVSD with normal caryotype underwent surgery. According to the anatomical complexity there were 22 intermediate AVSD, 36 complete AVSD and 11 complex AVSD. Associated lesions were present in 68% of the patients including left sided heart anomalies in 57%. According to the size of the left ventricle (LV) evaluated on the LV:RV end diastolic diameter ratio calculated at 2D echocardiogram: right ventricular (RV) dominance was found in 29%; with border line LV in 13 patients and truly hypoplastic LV in 7 patients. Biventricular repair was always favored in case of border line LV and precluded when the LV:RV ratio was less than 0.33. Results: There were 57 biventricular repairs with 10 years actuarial survival of 709 8% and respectively, 100% in the complex AVSD, 86% in the intermediate AVSD and 51% in the complete AVSD (P 0.006). The risk factors for biventricular repair were the association to a subaortic stenosis (P 0.01) and the severity of the mitral valve lesions (P0.03) that led to 38% reoperation. There were 12 univentricular palliation with 10 years survival of 669 14%. The risk factor for univentricular palliation was the association to a severe pre-operative mitral regurgitation (P 0.005). Conclusions: biventricular repair should be precluded in patients presenting with subaortic stenosis. Severe mitral valve anomalies lead to elevated mortality and morbidity with frequent reoperations. Univentricular repair might have larger indications and cardiac transplantation might be considered in patients with truly hypoplastic LV presenting with severe pre-operative AV valve regurgitation. © 1997 Elsevier Science B.V.

Background Aortico-left ventricular tunnel is a rare congenital abnormal communication between the aorta and the left ventricle presenting in early childhood as aortic regurgitation and cardiac failure. This condition has rarely been reported in fetuses. Operation is the only treatment, and postoperative aortic incompetence could be related to the age or the type of repair. Methods We conducted a retrospective, two-institution review, from 1983 to 1995, of aortico-left ventricular tunnel diagnosed in utero and before 6 months of age. Results Three cases of aortico-left ventricular tunnel were diagnosed in utero by Doppler echocardiography between 22 and 24 weeks' gestation. Prenatal aortico-left ventricular tunnel was associated with severe left ventricular dysfunction, aortic valve anomalies, and fetal hydrops. One death occurred in utero and one immediately after birth, and in 1 case pregnancy was interrupted. In these 3 cases the diagnosis was confirmed by autopsy. Three neonates and 2 infants had the diagnosis of aortico-left ventricular tunnel made after birth and underwent successful surgical repair. At short and midterm follow-up all patients are alive and aortic valve regurgitation is absent or trivial. Conclusions This series shows that aortico-left ventricular tunnel covers an anatomic spectrum of lesions. Cases diagnosed in utero by Doppler echocardiography are characterized by severe ventricular dysfunction, associated aortic valve lesions, and poor outcome. Postnatal cases represent the more favorable end of the spectrum, with no associated lesions, and can be repaired without mortality and with good functional results.

Fifty-six consecutive patients underwent total correction of truncus arteriosus. Median age at repair was 41 days, with a range of 2 days to 8 months. In 71% the operation was done in the first 2 months of life. Nine patients had complex forms of truncus and 11 patients had aortic insufficiency. The truncal aortic root was transected, which provides a clear exposure of the coronary ostia. The aorta was reconstructed by direct end-to-end anastomosis, and the truncal valve was preserved in every case. Several different techniques were used for pulmonary reconstruction, including three types of anatomic reconstruction of the pulmonary valve with a trisigmoid leaflet system and two types of nonanatomic reconstruction. The anatomic techniques included use of 33 Dacron valved conduits, eight homograft valved conduits, and one porcine aortic root bioprosthesis. The nonanatomic reconstructions included direct anastomosis to the right ventricle in nine patients and insertion of autologous pericardial valved conduits in five. The hospital mortality was 16% (9/56; 95% confidence limits, 2% to 30%). Multivariate analysis outlines two independent incremental risk factors for hospital death: nonanatomic pulmonary valve reconstruction techniques and age younger than 1 month. The hospital mortality was 7.1% in the group with anatomic pulmonary valve reconstruction versus 43% in the group with nonanatomic pulmonary valve reconstruction (p = 0.015). The hospital mortality was 5.7% in those older than 1 month versus 33% in those younger than 1 month of age (p = 0.04). There were two late deaths. The actuarial freedom from reoperation and angioplasty at 7 years was 100% for patients receiving pericardial conduits, 80% for those undergoing direct anastomosis, 77% for those receiving Dacron conduits, and only 43% for those receiving homografts (p = 0.02). In conclusion, anatomic reconstruction of the pulmonary valve seems important at the time of the operation, age younger than 1 month remains an incremental risk factor, and the truncal valve can be preserved. (J THORAC CARDIOVASC SURG 1996;111:849-56)

The surgical management of patients with double-outlet right ventricle or transposition of the great arteries and straddling atrioventricular valves remains a subject of controversy. Biventricular repair has theoretic advantages because it establishes normal anatomy and physiology. In some instances, however, it seems to carry too high operative risk, and a univentricular heart repair is preferred. Since 1984, we have operated on 34 patients with double-outlet right ventricle (n = 15) or transposition of the great arteries (n = 19) with isolated straddling tricuspid valve (n = 17), isolated straddling mitral valve (n = 9), both mitral and tricuspid straddling (n = 2), or abnormal insertion of tricuspid (n = 7) or mitral (n = 2) chordae in the left ventricular outlet, precluding an adequate tunnel construction. Straddling was categorized according to the location of the papillary muscle insertion in the opposite ventricular chamber: type A, on the edge of the ventricular septal defect (n = 14); type B, on the opposite side of the ventricular septum away from the edge of the defect (n = 8); type C, on the free wall of the opposite ventricular chamber (n = 8). Abnormal chordal insertions were classified according to the location of their attachments around the edges of the defect. Three types of chordal distribution were identified: on the aortic conus, on the pulmonary conus crossing the ventricular septal defect, or around the defect closing it like a curtain. All but three patients had two ventricles of adequate size. Sixteen patients underwent palliation. Median age at the definitive operation was 6.5 months (range 1 to 130 months). Thirty patients underwent a biventricular repair and four had a univentricular repair. Biventricular repair was achieved by an arterial switch operation in 18 patients and by tunnel construction from the left ventricle to the aorta in 12. In isolated straddling of types A and B, the ventricular septal defect was closed by adjusting the septal patch on the ventricular side above the straddled papillary muscle. In type C, the patch was sewn over the papillary muscle by applying it on the septum. In double straddling, the ventricular septum was incised between the two papillary muscles, and an ellipsoid patch was used to reconstruct the septal defect, directing each subvalvular apparatus into its own ventricular chamber. When the abnormal chordae in the left outflow tract inserted on the aortic or pulmonary conus, the conus was incised and tailored to make a flap, leaving an unobstructed left ventricular outflow tract. In two patients the subvalvular apparatus was resected and reattached to the patch. Curtainlike chordae were a contraindication to biventricular repair in double-outlet right ventricle but not in transposition. There were four early deaths and one late death, all occurring in the group having biventricular repair. Death was due to myocardial ischemia (n = 1), right ventricular hypoplasia (n = 1), pulmonary hypertension (n = 1), and residual subaortic stenosis (n = 1). Two patients had moderate to severe postoperative atrioventricular valve incompetence, caused by a cleft in the mitral valve in one patient. Three patents were reoperated on for subaortic stenosis (n = 1), pulmonary stenosis (n = 1), and mitral regurgitation (n = 1). Mean follow-up of 30.7 ± 19.4 months was achieved in the survivors. All but one patient (univentricular repair) were in New York Heart Association class I, without atrioventricular valve incompetence. Actuarial survival at 4 years was 85.3% ± 3%. We conclude that straddling or abnormal distribution of chordae tendineae of the atrioventricular valves does not preclude biventricular repair in double-outlet right ventricle or transposition of the great arteries provided that the ventricles are of adequate size. Curtainlike abnormal tricuspid chordae remain a contraindication to biventricular repair in double-outlet right ventricle. (J Thorac C ardiovasc S urg 1996;111:527-35)

Background Idiopathic pulmonary arterial hypertension (IPAH) remains a progressive fatal disease. Palliative Potts'shunt has been proposed in children displaying supra-systemic IPAH. Methods A retrospective multicenter study to evaluate Potts'shunt in pediatric IPAH. Results Between 2003 and 2010, 8 children with supra-systemic IPAH and in WHO functional class IV despite medical PAH therapy underwent Potts'shunt. Age at IPAH diagnosis ranged from 4 to 180 months (median age: 64 months). Surgical procedure was performed in a mean delay of 41.9±54.3 months (from 4 to 167 months, median delay: 20 months) after IPAH diagnosis. Mean size of the Potts'shunt was 9.25±3.30 mm. Two patients, whose medical PAH therapy had been interrupted just after surgery, died at post-operative day 11 and 13 from acute pulmonary hypertensive crisis. After a mean follow-up of 63.7±16.1 months, the 6 children who were discharged from hospital were alive. Functional status improved markedly in the 6 survivors with a WHO functional class I (n=4) or II (n=2) at last follow-up, consistent with significant improvement of 6 minute-walk distance [302±95 (51±20% of theoretical values) vs 456±91 meters (68±10% of theoretical values), p=0.038] and decrease of brain natriuretic peptid (BNP) levels (608±109 vs 76±45 pg/ml, p=0.035). No Potts'shunt was found restrictive at last echocardiography. Conclusion Palliative Potts'shunt constitutes a new alternative to lung transplantation in severely ill children with supra-systemic IPAH, carrying a prolonged survival and persistent improvement in functional capacities.

Background We reviewed 212 consecutive patients who underwent stage I palliative surgery for hypoplastic left heart syndrome (HLHS) at our institution between January 1983 and June 1993. Methods and Results Six surgeons participated in the care of these patients. Follow-up is 97% complete. Preoperative anatomic and physiological factors and procedural features of the stage I operation were analyzed for impact on stage I mortality, survival to stage II palliation, and actuarial survival. Hospital mortality was not significantly lower during the second half of the study period ( P =.242). Operative mortality was 46.2%. Multivariate analysis revealed improved stage I operative survival in patients with mitral stenosis (MS) and aortic stenosis (AS; P =.006). Additional risk factors for stage I mortality were a lower immediately pre–stage I pH ( P =.034) and weight P =.015). Overall first-year actuarial survival for MS/AS was 59%, and it was 33% for all others ( P =.001). Among stage I survivors, patients with MS/AS were more likely to survive to stage II palliation ( P =.031). Analysis of actuarial survival of stage I survivors showed that a smaller ascending aorta ( P P P =.002) were all risk factors for intermediate death. Conclusions Preoperative anatomic and physiological state are predictors of stage I mortality. HLHS anatomic subtype also influences intermediate outcome, most notably pre–stage II attrition. These data may be useful in choosing initial management for patients with HLHS.

Although most children after an arterial switch operation for transposition of the great arteries have normal development and cardiac function, a few require reoperation. During the last 10 years, 68 of 753 patients who underwent arterial switch operations (9.3%) underwent 75 reoperations. Thirty underwent early reoperation (

Background. The surgical management for bridging patients with univentricular heart and systemic obstruction to a Fontan procedure remains controversial. Methods. Twenty-seven of 96 patients with univentricular heart and unobstructed pulmonary blood flow referred for surgical palliation were seen with systemic obstruction. Twenty-six were neonates with coarctation of the aorta in 21 and subaortic stenosis in 5. In 8 other patients, subaortic stenosis developed after initial pulmonary artery banding. Four different palliative procedures were performed: coarctation repair with pulmonary artery banding (group I, n=15); Norwood or Damus-Kaye-Stansel or arterial switch operation (group II, n=9); coarctation repair with pulmonary artery banding and bulboventricular foramen enlargement (group III, n=2); and orthotopic heart transplantation with coarctation repair (group IV, n=1). Results. The mortality rate was 34.3% (n = 12) for all patients, 53.3% in group I, 33.3% in group II ( p = 0.003 versus group I), and 50% in group III. Nine patients (8 in group I and 1 in group II) had development of subaortic stenosis and underwent a subsequent procedure: Damus-Kaye-Stansel operation in 5, arterial switch operation in 3, and bulboventricular foramen enlargement in 1. Three had a concomitant or subsequent Fontan procedure and 2, a bidirectional Glenn procedure. In group II, 1 patient underwent a subsequent Fontan procedure and another, a bidirectional Glenn anastomosis. Six of the 8 patients with subaortic stenosis after initial pulmonary artery banding underwent a second stage consisting of a Damus-Kaye-Stansel procedure (n = 3), bulboventricular foramen enlargement (n = 2), or creation of an aortopulmonary window (n = 1). Three had a concomitant Fontan procedure and 2, a bidirectional Glenn procedure. Actuarial 4-year survival was 65.5% ± 8.4% (70% confidence limits) for all patients; it was 40% ± 13.3% in group I and 66.6% ± 16.3% in group II ( p Conclusions. Initial management of patients with univentricular heart and systemic obstruction by Norwood-like procedures provides a better outcome. Success of the Fontan operation relies on the ability to provide timely relief of subaortic stenosis.

The transposition of the great arteries {S,D,L} complex is delineated for the first time from the anatomic, diagnostic, and surgical standpoints in this study of 26 cases: 16 surgical and 10 postmortem. Transposition of the great arteries with situs solitus of the viscera and atria (S), D-loop ventricles (D), and L-transposition (L) was characterized by six additional interrelated anomalies that largely determined surgical management: (1) ventricular septal defect, usually conoventricular, in 96%; (2) malalignment of the conal septum, typically leftward and posteriorly, in 80%; (3) right ventricular hypoplasia in 50%; (4) pulmonary outflow tract stenosis in 27%; (5) ventricular malposition, such as superoinferior ventricles, in 23%; and (6) absent left coronary ostium resulting in "single" right coronary artery in 23%. Complete surgical repair was done in 81% of the surgical patients with a 12.5% hospital mortality rate and no late deaths. When there was no pulmonary outflow tract stenosis and intracardiac anatomy was uncomplicated, we undertook anatomic repair before 1 month of age. However, when pulmonary outflow tract stenosis coexisted, complete repair was deferred until after age 1 year, our currently preferred operation being the REV procedure (reparation a l'etage ventriculaire). When complex intracardiac anatomy precluded biventricular repair, a palliative procedure was performed in 19% without mortality. Hence, this experience indicates that surgical management of patients with the transposition of the great arteries {S,D,L} complex is feasible. (J THORAC CARDIOVASC SURG 1995;110:613-24)

Between 1983 and 1994, 307 consecutive neonates underwent coarctation repair by a single surgical technique: extended end-to-end anastomosis. Mean age at operation was 13 ± 8 days. Isolated coarctation was present in 95 patients (group 1), 102 patients had associated ventricular septal defect (group 2), and 110 patients had associated complex intracardiac lesions (group 3). Aortic arch hypoplasia was present in 81% of the patients (62% in group 1 versus 85% in group 2 and 93% in group 3: p < 0.001). In 271 patients, the aortic arch reconstruction was performed via a left thoracotomy with normothermia (100% of group 1, 95% of group 2, and 72% of group 3); in the other 36 patients, undergoing one-stage repair or palliation of the associated lesion, it was performed via a midline sternotomy during a short period of deep hypothermia and circulatory arrest (5% of group 2 and 28% of group 3). Pulmonary artery banding was performed in 94 patients. Spontaneous ventricular septal defect closure was observed in 39% of the patients of group 2 operated on via thoracotomy. Early mortality rates in groups 1 (2%) and 2 (2%) were significantly lower than in group 3 (17%) ( p < 0.001). There were 29 late deaths, all related to associated cardiac lesions or their subsequent repair. The overall total mortality was 16.9%. In group 3 this rate was significantly higher in patients undergoing two-stage procedures (47%) than in those undergoing one-stage repair (23%) ( p < 0.05). All but 14 survivors were followed up for a mean of 61 ± 36 months. Actuarial survivals at 10 years were 98% in group 1, 94% in group 2, and 60% in group 3. The recoarctation rate was 9.8%, leading to 21 reoperations and three angioplasties without mortality. Patients with a more extended or severe form of aortic arch hypoplasia had a significantly higher risk of recoarctation ( p < 0.001). Actuarial freedom from reoperation for recoarctation at 10 years was 93%. The findings of this study suggest that extended end-to-end anastomosis provides an adequate and safe repair of neonatal coarctation. Low recoarctation rate, owing to effective relief of the obstruction created by aortic arch hypoplasia and to complete resection of ductal tissue, freedom from major morbidity, and feasibility via both lateral and anterior approaches are the main advantages of the extended end-to-end anastomosis. Mortality is mainly dependent on the complexity of the cardiac associations. Successful management of the majority of the neonates with coarctation and associated ventricular septal defect is possible with repair of coarctation alone. One-stage repair of neonatal coarctation and associated complex heart defects (with indication for two-ventricle repair) by means of an anterior approach provides a better outcome than a two-stage repair. (J THORAC CARDIOVASC SURG 1995;109:663-75)

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)

There is still no consensus regarding the most appropriate protocol for managing symptomatic patients with tetralogy of Fallot (TOF) presenting during early infancy. From January 1987 to April 1994, 83 patients with TOF were operated on at less than 6 months of age. Sixty patients (mean age 109 +/- 5 days) underwent primary repair and 23 (mean age 72 +/- 10 days) underwent initial palliation with a modified Blalock-Taussig shunt (MBTS) in 22 cases. No strict protocol was used but patients who received initial palliation had lower weight, smaller pulmonary annulus or had an anomalous coronary artery. Nineteen of the patients initially palliated underwent repair at a median of 13 months after palliation. During this interval, the pulmonary annulus size increased from a Z-value of -3.0 +/- 0.4 at palliation to -1.6 +/- 0.7 at repair (P = 0.06) and the summed diameters of pulmonary artery branches from -2.2 +/- 0.4 to -1.6 +/- 0.7 (P = 0.2). There were no operative deaths among the repaired patients, but two patients died early after shunt insertion. Transannular patch was required in 58% of the patients regardless of the management protocol. Actuarial survival rate and freedom from reoperation at 48 months were 98.0% and 77.4% (P = 0.003) and 87.6% and 90.0% in primarily repaired and initially palliated patients, respectively. Primary repair of TOF at the time of presentation is the preferred approach regardless of age.(ABSTRACT TRUNCATED AT 250 WORDS)