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OBJECTIVES Pulmonary valve replacement (PVR) performed for pulmonary valve regurgitation is the most common indication for reoperation during mid-to-long-term follow-up after tetralogy of Fallot repair. An aneurysmal dilation of the infundibulum is often associated secondary to the infundibulotomy performed in the first operation. The right ventricular outflow tract reconstruction with endo-exclusion aims to exclude the non-contractile segments of the dilated right ventricular. This study intends to assess the safety and efficiency of the endo-exclusion technique. METHODS Between January 2010 and December 2018, 86 patients underwent a PVR with (n = 46) or without (n = 40) endo-exclusion. The current study compares the outcomes in terms of survival, reintervention, structural valve deterioration, right ventricular function (volume and right ventricular ejection fraction) and pulmonary valve gradient. The median follow-up time was 4.45 years (1.9 months to 9.87 years). RESULTS There was no 30-day mortality. There was no difference in the freedom from reintervention at 7 years (without endo-exclusion, 97%, versus with endo-exclusion, 94%, log-rank = 0.68) or in the freedom from structural pulmonary valve deterioration at 7 years (without endo-exclusion, 94%, versus with endo-exclusion, 89%, log-rank = 0.94). No significant difference was observed in the indexed right ventricular end-diastolic volume (102.2 ± 34 ml/m2 in the PVR without endo-exclusion group and 93.3 ± 22 ml/m2 in the PVR with endo-exclusion group, P = 0.61). No significant difference was observed in the right ventricular function (right ventricular ejection fraction: 46 ± 11% in the PVR without endo-exclusion group and 46 ± 9% in the PVR with endo-exclusion group, P = 0.88). CONCLUSIONS PVR with or without endo-exclusion is a safe and effective procedure. PVR with endo-exclusion allows implantation without structural deformation of the valve and therefore excellent short- and medium-term results.

Background Berlin Heart EXCOR (BH) ventricular assist devices provide mechanical long-term circulatory support in children with end-stage heart failure, as a bridge to transplantation or to recovery. Most studies are from large-volume paediatric cardiac centres. Aim The aim of this study was to analyse the experiences of three French centres and to compare these with available published data. Method We performed a retrospective observational study of three paediatric cardiac intensive care units. All children supported with BH devices were included. Morbidity and mortality data were collected and risk factors analysed. Results Fifty-four (54) patients (54% male) were included. Survival rate was 73% while on a BH device. Median age at BH device implantation was 17 months (range 2–180 months). The predominant indication was dilated cardiomyopathy (61%). Bi-ventricular assist device was used in 25 (46%) cases. The total length of long-term circulatory support was 3,373 days, with a mean length per patient of 62.5 days (range 5–267 days). Thirty-two (32) patients were transplanted (59%) and seven (13%) were successfully weaned. Type and length of support did not influence morbidity. Main complications were renal dysfunction (57%), bleeding (41%), and infection (39%). In multivariate analysis, a weight Conclusions The weight seems to be the most important risk factor of mortality in this precarious condition.

Left atrioventricular valve aneurysm is rare. We present a rare case of partial atrioventricular septal defect with an extremely thin left atrioventricular valve aneurysm mimicking valve perforation. The preoperative echocardiography demonstrated severe left sided atrioventricular valve regurgitation on the “cleft” and leaflet perforation. But in per-operative, we discovered a left sided atrioventricular valve aneurysm instead of valve perforation. The “cleft” edge and a aneurysm were closed.

Background:Pulmonary atresia with ventricular septal defect and severe tetralogy of Fallot require a palliative procedure for pulmonary artery rehabilitation. For first-stage palliation, two main surgical options are still debated: right ventricle to pulmonary artery connection and modified Blalock–Taussig shunt. We compared the clinical outcomes of the two procedures.Methods:From 1995 to 2018, 88 patients needed palliation (pulmonary atresia with ventricular septal defect n = 47; tetralogy of Fallot n = 41). Among these patients, 70 modified Blalock–Taussig shunt and 18 transannular path augmentation were performed before 6 months of age. Using a 1:1 propensity score match analysis, 20 patients were included in the analysis. The primary outcome was in-hospital mortality and pulmonary artery growth.Results:After matching, the pre-operative Nakata was smaller in transannular path augmentation 54 ± 24 mm2/m2 than modified Blalock–Taussig shunt 109 ± 31 mm2/m2 (p < 0.001). The age and weight were similar (p = 0.31 and p = 0.9, respectively). There was no difference in in-hospital mortality (p = 0.3). The Nakata index before biventricular repair and delta Nakata were smaller in modified Blalock–Taussig shunt group (206 ± 80 mm2/m2, 75 ± 103 mm2/m2) than transannular path augmentation (365 ± 170 mm2/m2, 214 ± 165 mm2/m2; p = 0.03; p < 0.001). Median time to biventricular repair was similar (p = 0.46). The rate of interstage reintervention was similar (p = 0.63).Conclusions:The transannular path augmentation is better for the rehabilitation of the native pulmonary artery. Despite a smaller pulmonary artery, right ventricle to pulmonary artery connection is equivalent to modified Blalock–Taussig shunt for rate of biventricular repair and time to biventricular repair.

Patients with anomalous aortic origin of the left anterior descending coronary artery (AAOCA) from the right sinus of Valsava, and associated with a trans-septal course, are recommended for surgery only when symptoms of ischemia are present. The transconal unroofing method is straightforward and provides good anatomic result. In absence of significant coronary compression, surgical management of the trans-septal coronary course is proposed if the patient is a candidate to cardiac surgery for another reason, such as congenital heart disease. We describe a transconal approach in a patient with a trans-septal coronary artery and a ventricular septal defect.

Summary Background The main difference between extreme tetralogy of Fallot (TOF) and pulmonary atresia with ventricle septal defect (PA/VSD) is anterograde pulmonary blood flow (APBF). It is speculated that the association of modified Blalock–Taussig shunt (mBTS) with APBF favours shunt thrombosis, but promotes better pulmonary artery growth. Aim To compare pulmonary artery growth after mBTS between TOF and PA/VSD. Methods From 1995 to 2018, 77 mBTS procedures were performed in infants (aged Results After matching, the preoperative Nakata index was similar in the two groups (TOF 101 ± 34 vs. PA/VSD 106 ± 35 mm2/m2; P = 0.75). Age and weight were similar (TOF 24 ± 20 days, 3.3 ± 0.6 kg vs. PA/VSD 24 ± 33 days, 3.3 ± 0.9 kg; P = 0.84 and P = 0.77, respectively). There was no difference in rates of in-hospital mortality (TOF 0% vs. PA/VSD 10%; P = 0.13) or mBTS thrombosis (TOF 15% vs. PA/VSD 10%; P = 0.63). The left and right pulmonary artery diameters at time of biventricular repair were similar (TOF 7.5 ± 2.2 and 6.7 ± 2.1 vs. PA/VSD 8 ± 2.7 and 7.1 ± 2.5 mm; P = 0.43 and P = 0.78, respectively), as were delta Nakata (TOF 112 ± 102 vs. PA/VSD 107 ± 66 mm2/m2; P = 0.89), median age for biventricular repair (P = 0.83) and reintervention rates (TOF 10% vs. PA/VSD 15%; P = 0.67). Conclusions We found no difference in pulmonary artery growth between APBF with mBTS versus mBTS alone. Thus, we could not show an increase in mBTS thrombosis with APBF.

Background Management of patients with complex congenital heart defects (CHD) requires 3D imagination skills for the spatial evaluation of cardiac structures. Current 3D imaging [echocardiography, MRI, computed tomography (CT)], projected on a 2D screen, limits the appreciation of the relief and the interaction of anatomical structures. Purpose The contribution of 3D stereoscopic relief imagery (3D S/R), built by triangle-based mesh generation, is more qualitative than quantitative, which is why its use, rarely reported in the literature, must be evaluated. Methods 3D S/R was used from 2009 to 2019, as much as the CT made it possible to define a Region of Interest. Data and segmentation processing have been used to obtain four different types of reconstructions: MultiPlanar (MPR), Volume Rendering Technique (VRT), Surface Rendering Technique (SRT), and 3D S/R. The anaglyph 3D S/R effect requires the wearing of glasses (red - cyan blue filters) distributed during the session ( Fig. 1 ). Five complex CHD will be presented in MPR, VRT, then in SRT and 3D S/R: – Ventriculo-Arterial Malposition, – multiple Ventricular Septal Defects, – Anomalous Aortic Origin of a Coronary Artery, – Coarctation and hypoplastic Aortic Arch, – abnormal Pulmonary Arterial Arborization with Major Aorto-Pulmonary Collateral Arteries. Results The qualitative evaluation [MPR + VRT versus SRT and 3D S/R] will be carried out by questions/answers [Wooclap™ interactive system - voting on smartphone, in streaming, using a code associated with the presentation], allowing a global rating of the five CHD, concerning: – the perception of the relief, – the understanding of the cardiopathy, – the probability of appropriation of this 3D S/R imagery. Conclusions This presentation will allow: – to assess the potential influence of 3D S/R on the management strategy of CHD, – to compare these imaging studies with mesh generation techniques leading to the more commonly reported 3D printing.

Background Surgical repair of sinus venosus defects with partial anomalous pulmonary venous connection (PAPVC) to the superior vena cava (SVC) has been associated with venous return obstructions, and sinus node dysfunction. Purpose We aimed to evaluate the safety of four different surgical procedures, for repair of sinus venosus defects with PAPVC, in 23 adult patients, adapted to each patient's specific anatomy assessed by 3D computed tomography. Methods Between March 2010 and May 2019, we performed anatomic repair of sinus venosus defects with a single patch (n = 6; 26.1%), 2-patch (n = 9; 39.1%), Warden (n = 3; 13%), or modified Warden procedure with reconstruction of the divided SVC using a PTFE conduit (n = 5; 21.7%) ( Fig. 1 ). Median age was 40 years [range, 15 to 73 years]. Main associated procedures were: creation of atrial septal defect (n = 1), tricuspid valvuloplasty (n = 2), tricuspid valve replacement (n = 1), pulmonary valve replacement (n = 1), monoblock “cut and sew” of pulmonary veins and superior vena cava (n = 1). Results Follow-up was 3.26 years [range, 2 months to 10 years]. There were no mortality and no obstruction of SVC and pulmonary veins at the time of discharge and during follow-up. Aortic clamping was shorter (60 ± 39 min) with the single patch technique and longer (112 ± 31 min) with the modified Warden procedure. Nineteen patients (82.6%) had regained sinus rhythm at discharge; a patient with paroxysmal atrial fibrillation had regained a sinus rhythm after the “cut and sew” isolation technique of the superior vena cava and pulmonary veins. All patients were NYHA 1. Conclusions PAPVC can safely be performed with an appropriate technique, and low morbidity. The single patch technique, when anatomically possible, offers good results but now competes with transcatheter correction. When PAPVC is far from the SVC-right atrium junction, the modified Warden procedure offers an effective and obligatory surgical repair, without any increase in complications.

Objective Percutaneous closure of interatrial communications (ASD) has become the standard for therapeutic management. However, some patients are ineligible for this technique due to the morphology of the ASD, thus requiring conventional surgical closure by sternotomy or lateral thoracotomy. Minimally invasive surgery (MIS) has been introduced in several adult heart surgery centers especialy for mitral surgery. The purpose is to present the preliminary experience of ASD closure by MIS. Material and methods We analyzed all of the patients (n = 5) treated from March 2019 (start date of the MIS program) to December 2019. The MIC was performed by an anterior mini thoracotomy of approximately 5 cm with femoral cannulation ( Fig. 1 ). The pre, per, and postoperative data were prospectively collected. All patients underwent postoperative vascular Doppler ultrasound of the femoral cannulation site. Results Average age at intervention was 27.3 years (range: 14.5–41), weight was of 60 kg (43–80) (4 women for 1 man). The median duration of CPB was 108 min (101–139), the duration of aortic cross clamping 45 min (36–61). There were no hospital deaths or major complication. One patient had a pneumothorax on D3. The median duration of extubation was 3.5 hours (3–6), the median duration of hospitalization was 8 days (6–11). Postoperative echocardiography did not show any residual ASD or pericardial effusion. All patients were sinus rhythm. No venous or arterial stenosis was observed at the cannulation sites. The average systolic pulmonary arterial pressure, was measured 34 mmHg (15–74) preoperatively, and estimated 30 mmHg (20–55) postoperatively. Conclusion This clinical study shows the preliminary experience of MIS program for the closure of ASDs. This approach appears to be safe and effective, without major vascular complications. longer term follow-up is required to determine the specific role of MIS approach in the management of this patient population.

Direct communication between the right pulmonary artery and the left atrium in the presence of intact atrial septum is a rare congenital entity. A 12-year-old boy presenting with dyspnea at exertion was fortuitously found to have low arterial oxygen saturation at rest. Imaging studies showed a large tortuous vessel originating in the distal right pulmonary artery, and ending into the left atrium via the right upper pulmonary vein. The patient underwent successful elective videothoracoscopic stapling and division of the fistula.

BACKGROUND A low diastolic blood pressure (DBP) is associated with increased cardiovascular events in patients with coronary artery disease or chronic kidney disease. AIM The aim of this study was to assess the association of blood pressure with cardiac events during the long-term follow-up of heart transplant recipients. PATIENTS AND METHODS In this prospective cohort study, we performed ambulatory blood pressure monitoring and home blood pressure monitoring in 76 transplant heart recipients 13.5±6.6 years after transplantation. The patients were followed for 54±17 months after blood pressure monitoring. RESULTS Twenty-one patients had a cardiac event (cardiac death, acute coronary event, coronary revascularization procedure, and hospitalization for heart failure) during the follow-up. In the Kaplan-Meier survival analysis, we found that a DBP below the median value (

Background Advances in congenital heart disease (CHD) have transferred the mortality from childhood to adulthood. Exercise capacity in young patients with CHD remains lower than in the general population, resulting in deconditioning and impaired quality of life. Evidence based-medicine in cardiac rehabilitation in this age group with CHD remains limited. We present the QUALI-REHAB study rationale, design and methods. Methods The QUALI-REHAB trial is a nationwide, multicentre, randomised, controlled study, aiming to assess the impact of a combined centre and home-based cardiac rehabilitation program on the quality of life of adolescents and young adults (13 to 25 years old) with CHD. Patients with a maximum oxygen uptake (VO2max) Conclusion After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. The QUALI-REHAB trial intends to assess if a combined centre and home-based rehabilitation program, could improve the quality of life and the exercise capacity in youth with CHD. Trial registration: Clinicaltrials.gov ( NCT03690518 ).

Introduction The optimal timing for surgical management of severe PR in patients with repaired TOF is still controversial due to lack of prognostic data. Due to ageing of this population we wanted to analyse the prognostic factors associated with this surgery at a “senior” age. The aim of this study was to define clinical and paraclinical criteria associated with morbidity-mortality of RVP in “senior” patients with repaired TOF. Methods We conducted a single-centre, retrospective study of 48 patients aged 30 years old and above with repaired TOF who underwent RVP at Marseille University Hospital from January 2009 to December 2018. RVP for other congenital cardiopathy or endocarditis and RVP for TOF in patients younger than 30 years old were excluded. The primary outcome was to assess morbidity-mortality with composite criterion including supraventricular arrhythmia, rehospitalization due to any cardiologic cause or death due to any cause. Results Mean age at PVR was 41 ± 9.8 years old. Mean patient follow-up was 52 months. 23 (48%) patients had the primary outcome. Preoperative signs of late management of patients with repaired TOF complicated of PR, whether left ventricular dysfunction on echocardiography (P = 0.005), right heart failure (P = 0.002), syncope or ventricular tachycardia (P = 0.006), are associated with increased postoperative morbidity-mortality. In multivariate analysis, only elevated right ventricular end-diastolic pressure (RVEDP) was associated with our primary outcome. Preoperative right ventricular volumes on MRI were not associated with our primary endpoint. Conclusion RVEDP was a risk factor for postoperative morbidity-mortality of PVR. Right heart catheterization could be helpful in deciding the best timing for PVR in “senior” patients with repaired TOF. Our study supports recent research highlighting the limitations of considering right ventricular volumes in MRI as a therapeutic target.

Objectives The purpose of this work was to assess epidemiological aspects, surgical approach, morbidity and mortality rates of patients presenting with tracheal stenosis requiring surgery, and the evolution of surgical techniques over the last years. Methods We performed a retrospective observational study from 1990 to 2017 in a pediatric tertiary-care center with needing surgery for tracheal stenosis. We analyzed clinical patients' characteristics, type of stenosis, type of surgery and follow-up. Results Twenty-eight children presented with tracheal stenosis, half of them with congenital stenosis (complete tracheal rings) and the other half with acquired stenosis (neoplasic or post intubation injury). 39.3% of these stenoses were associated with a vascular ring (61.5% in case of congenital stenosis). Depending on the extent of the stenosis and its origin, the surgery could be performed endoscopically or by an external approach. Enlargement tracheoplasty with an autograft (14.3%) was replaced by slide tracheoplasty with Cardio Pulmonary By-Pass (CPBP, 28.6%) with improved results for the treatment of long segment tracheal stenosis, involving more than 30% of the tracheal length (all were congenital in our study). Slide tracheoplasty has been performed since the late 90's in our institution. 25% of children have had a resection and anastomosis of the trachea because they had a stenosis involving less than 30% of tracheal length. Endoscopic surgery was performed for membranous stenoses, which were often seen after intubation or tracheotomy (32.1% of patients). Conclusion Effective treatment of surgical tracheal stenosis was performed in 28 children between 1990 and 2015. Surgical techniques have evolved over time, leading to a better management of this rare and serious disease.

International audience; 6p25 deletion is a rare but well-known entity. The main clinical features include an abnormal facial appearance, developmental delay, and ocular anomalies. Cardiac anomalies are frequently seen but remain poorly delineated. We describe a 4-year-old girl with 6p25.3 deletion, which includes the FOXC1 gene, typical dysmorphic features associated with developmental delay and oculo-motor anomalies. Aortic valve dysplasia was diagnosed early in life. The cardiac lesion progressed very rapidly between the age of 3 and 4 years requiring aortic valve replacement. Genomic analysis of blood and excised valve tissue showed down-regulation of FOXC1 but also FOXC2 expression in the diseased aortic valve. This allows us to speculate on the potential role of FOXC1 in aortic valve anomalies.

Background Balloon aortic valvulopasty (BAV) is an alternative therapy to surgery in newborns with critical aortic stenosis and has become the first-line treatment in many centers. Concerns remain as to restenosis, aortic valve regurgitation and risks of reintervention. Aims The aim of the present study was to identify predictors of reintervention (surgery or catheterization) after BAV of neonatal aortic stenosis. Methods Monocentric, retrospective study. Thirty-six neonates with congenital aortic valve stenosis, who underwent BAV, were included between 10-2003 and 11-2016. Patients were divided into 2 groups [group 1: 15 days (n = 13)]. Median follow-up reached 3 [1–9] years. Immediate success was defined as residual aortic valve gradient Results Median age at intervention was 8 [1–45] days, median weight 3.4 [1.1–5.4] kg. Median balloon/annulus ratio was 1 [0.8–1.2]. Post-procedure mortality was 11%. Procedure was successful in 23 patients (71.9%). Freedom from reintervention was 78% at 1 year and 62.5% at 5 years. Age 35 mmHg at the end of the catheterization or on Doppler at the end of the hospitalization) were significantly associated with reintervention (P = 0.001 and P = 0.013, respectively). The balloon/annulus ratio was not significantly associated with reintervention. Conclusion BAV remains a valuable alternative to surgical valvotomy in neonatal aortic valve stenosis. Age of the patients and residual gradient were in our study, significantly associated with risk of reintervention.

International audience; Background: Doppler-derived indexes associated with high left ventricular filling pressures are risk factors for cardiac events in various populations. The aim of this study was to evaluate the predictive value of two of these Doppler indexes during the long-term follow-up of heart transplantation. Methods and Results: In this cohort study, we measured E/A ratio combining early filling velocity (E) with late filling velocity (A) and E/E' ratio combining E wave with relaxation velocity on tissue doppler (E') in 122 transplant heart recipients, 6.9 +/- 5.9 years after transplantation. The patients were followed for 97 +/- 32 months after Doppler measurements. We found an E/A>2 in 68 patients and an E/E'>15 in 23 patients. Forty patients had a cardiac event (cardiac death, retransplantation, acute coronary events, hospitalization for heart failure, treated acute rejection episodes, and coronary revascularization procedures) during the follow-up. In multivariate analysis, E/A and E/E' were significantly associated with cardiac events (hazard ratio 2.2, 95% CI: 1.1-4.4; P=.02 and hazard ratio 2.3, 95% CI: 1.1-4.8; P=.02, respectively). E/E', E/A, and significant coronary stenoses were the strongest predictors of cardiac events. Conclusions: E/A and E/E' Doppler indexes may be used to predict cardiac events during the long-term follow-up of heart transplant recipients.

International audience; Objectives: To evaluate the use of non-ECG-gated computed tomography ă (CT) angiography to describe pulmonary and coronary defects in patients ă with tetralogy of Fallot (TOF). ă Patients and methods: This retrospective study was carried out on TOF ă patients having undergone pre-operative non-ECG-gated CT angiography ă between February 2007 and September 2012. The following clinical ă parameters were recorded: mean age at CT angiography, sex, the existence ă of genetic disease and the need to sedate the patient prior to CT ă angiography. CT data were analyzed retrospectively to determine the ă site(s) of pulmonary stenosis (infundibular, valvular or arterial), the ă size of pulmonary arteries and the presence of anomalous coronary artery ă courses. CT findings were then compared to the anatomy observed during ă surgery. ă Results: Thirty-five patients were included in the study. The mean age ă was 4.30 +/- 1.91 months (boys/girls = 17/18). Two patients had ă associated chromosome disorders (one 22q11 microdeletion and one CHARGE ă syndrome). Sixteen patients (45.71%) were sedated prior to CT. ă Pulmonary artery assessment revealed 24 patients (68.57%) with ă infundibular stenosis, 5 (17.5%) with infundibular and/or valvular ă stenosis, and 6 (21%) with anomalous pulmonary arteries. CT angiography ă also evidenced anomalous coronary arteries in 8 patients (22.85%). ă Conclusion: Due to its reduced scanning time and high spatial ă resolution, non-ECG-gated CT angiography is a non-invasive imaging ă modality that provides accurate information on pulmonary and coronary ă artery anatomy in patients with TOF. (C) 2016 Editions francaises de ă radiologie. Published by Elsevier Masson SAS. All rights reserved.

International audience

Objective Abnormal origin of coronary artery from the pulmonary artery (ACAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. Immediate surgical correction can provide excellent results. This study aimed to determine the surgical outcome of ACAPA. Methods From 1993 to 2016, 31 consecutive patients underwent coronary re-implantation. Concomitant mitral valvuloplasty was performed in two. The study cohort was divided into two groups according to age (group 1: ≤ 6 months and group 2: > 6 months). Data from demographic characteristics, electrocardiography, echocardiography, surgery, intensive care unit stay and follow-up were evaluated. Results The study included 29 patients with anomalous left coronary artery from the pulmonary artery and 2 with anomalous right coronary artery from the pulmonary artery. The median age at repair was 4.75 [2.3–16.3] months and the median weight was 6.2 [4.3–9.4] kg. Preoperative echocardiography showed severe LV dysfunction in 56.7% of patients. The median preoperative LVEF was 33.5% [20–55]. Mitral regurgitation (MR) was moderate to severe in 13 patients (41.9%). Requirement to extracorporeal membrane oxygenation (ECMO) support systems was necessary in 2 cases (6.4%) before surgery and 5 cases (16.1%) after correction. Postoperative mortality rate was 9.7%. Younger age ( Conclusions Young age is significantly associated with a more severe presentation, more ECMO requirement and mortality. Lesser development of coronary collateral circulation can explain these findings. After establishment of a two-coronary circulation both ventricular function and MR tend to be normalized over time. This ‘recovery’ usually occurs within 6 months but may require more time (maximum of thirty months in our population).

Purpose The medical information (diagnosis and treatment) around congenital heart disease can induce major stress to the parents. Visual analog scales have been validated to assess anxiety in the adult population. The aim of this study was to analyze parental anxiety using a Visual analog scales and to explore the influencing factors, in particular the level of understanding of the disease and its support. Methods and results Prospective transversal study lead from January 2017 to May 2017 in the pediatric cardiology and intensive care unit of La-Timone-Enfants’ University Hospital (Marseille, France). All available parents (mother and/or father) of children admitted for surgical or transcatheter cardiac intervention were offered to participate. The parents were asked to complete 2 Visual analog scales in order to assess their level of anxiety and comprehension, before intervention and after complete information given by the cardiologist, the surgeon and the anesthetists. We included 73 patients, mean age 3 years, median 11 months (range 7 days–13 years), 42 requiring surgery and 31 transcatheter intervention. The mean score of maternal anxiety was significantly higher than the paternal anxiety (8.2 vs. 6.3, P Conclusion This study highlights several factors that influence the parental anxiety. Identifying the parents at risk of high stress can be useful to set up psychological support during hospitalization.

Background Extracorporeal membrane oxygenation (ECMO) has become a gold standard in treatment of severe refractory circulatory and/or pulmonary failure. The aim of this work is to give key points for pediatric mobile ECMO team implementation and to demonstrate its safety and feasibility, even in a middle size pediatric cardiac surgery reference center. Methods This is a retrospective analysis of our series of patients initiated on ECMO in a peripheral center and transferred to our center was conducted. Our data were collected from 10 consecutive years: from 2006 to 2016. Results Fifty-seven pediatric patients were cannulated in peripheral center and transported on ECMO. Forty-two patients (73,7%) were weaned from ECMO. We did not experience any adverse event during transport. Up to 40% of our population were neonates (age Conclusion Offsite ECMO Implantation and transport of pediatric patients on ECMO can be safe, when performed by a dedicated and experienced team.

OBJECTIVES Total cavopulmonary connection (TCPC) performed in the second decade of life has rarely been studied. Thus, we investigated (bicentric study) early and late morbidity and mortality following completion of TCPC in these patients. METHODS From January 1999 to June 2014, 63 patients (14.5 ± 2.9 years) underwent TCPC (extracardiac conduit). Palliation before completion was an isolated bidirectional cavopulmonary shunt (BCPS) in 3 patients or BCPS associated with additional pulmonary blood flow (APBF) that was either antegrade (Group 1) in 38 (63%) or retrograde (Group 2) in 22 (37%). Preoperative and perioperative data were reviewed retrospectively. RESULTS Mean pulmonary arterial and ventricular end-diastolic pressures were 12.2 and 9.2 mmHg, respectively. Mean Nakata index was 279 ± 123 and 228 ± 87 mm 2 /m 2 in Groups 1 and 2, respectively ( P = 0.01). Aortic cross-clamping was performed in 22 from Group 1 and 8 from Group 2 ( P = 0.04). Mean follow-up was 4.57 years [0.8-15]. Nine patients had prolonged stays in the intensive care unit (>6 days). There were 1 early and 2 late deaths (non-cardiac related). Actuarial survival was 96% at 4 years. At last follow-up, single-ventricle function remained normal or improved in all patients (Group 1) compared to 82% in Group 2 ( P = 0.02). New York Heart Association (NYHA) class had improved in both groups: 47 patients were NYHA class II and 16 class III preoperatively vs 50 class I and 10 class II postoperatively ( P < 0.001). CONCLUSIONS Single-ventricle palliation with BCPS and APBF allowed completion of TCPC in the second decade of life, with encouraging mid-term results. However, BCPS with retrograde APBF was associated with single-ventricle dysfunction: thus, this technique needs to be used cautiously as long-lasting palliation.

Case report An 11-month-old boy with an unremarkable medical record was referred to our institution for treatment of sustained VT leading to dilated cardiomyopathy (left ventricular ejection fraction [LVEF] 33%). On admission, 12-lead electrocardiogram (ECG) revealed broad tachycardia (QRS duration 130 ms, cycle length 300 ms), AV dissociation, and positive concordance pattern from lead V1 to V6 (Figure 1). Initial medical treatment using a combination of antiarrhythmic drugs including amiodarone failed to resolve the VT. On day 15 of treatment, cardiac function deteriorated suddenly, with 3 reversible cardiac arrests. Cardiogenic shock developed and required emergency mechanical circulatory assistance using venoarterial extracorporeal membrane oxygenation (ECMO) by peripheral cervical cannulation through the right internal jugular and common carotid artery. Cardiac monitoring during ECMO demonstrated incessant VT despite general anesthesia condition. Major left ventricular dysfunction developed with LVEF 25%. Cessation of VT was observed only during hypothermia or for a few seconds after administration of

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.

Background The low-output state is the chief cause of morbidity and mortality after Fontan operations. An alternative hemodynamic tool would be a welcome addition for these patients, who are typically resistant to conventional therapeutic measures. Methods and Results The hemodynamic effects of conversion from conventional intermittent positive pressure ventilation (IPPV) to cuirass negative pressure ventilation (NPV) was investigated in nine acute postoperative Fontan patients on the pediatric intensive care unit and nine anesthetized patients undergoing cardiac catheterization in the convalescent phase after Fontan operations. Pulmonary blood flow was measured using the direct Fick method during IPPV and after a brief period of NPV. In one subgroup of patients, pulmonary blood flow was measured again after reinstitution of IPPV, and in a second subgroup, pulmonary blood flow was measured after an extended period of NPV. A brief period of NPV increased pulmonary blood flow from 2.4 to 3.5 L · min−1 · /m−...

1 In whole-cell patch-clamped human atrial myocytes, the antiarrhythmic agent bertosamil (10 μM) inhibited the sustained component, Isus (38.6±3.1%), and enhanced the inactivating component, It (9.1±6.1%), of the outward K+ current elicited by 750 ms test pulses from −60 mV to +50 mV. Higher concentrations of bertosamil (>10 μM) inhibited both It and Isus. 2 Suppression of Isus and stimulation of It by 10 μM bertosamil was observed on renewed stimulation following a 2 min rest period during which the drug was applied and persisted after washout, indicating a rest-dependent effect of bertosamil on the outward K+ current. 3 Cell dialysis with an internal solution containing 10 μM bertosamil increased both It (78.0±14.7%) and Itotal (26.7±8.4%) and inhibited Isus (28.9±6.3%, n=6). In the presence of intracellular bertosamil, external application of the drug inhibited It and Isus in a concentration-dependent and use-dependent manner. 4 Following the suppression of Isus by 200 μM 4-aminopyridine (4-AP), bertosamil (10 μM) inhibited It. Washout of 4-AP was associated with a larger It amplitude than that observed in control conditions. In myocytes characterized by a prominent Isus and lack of It, bertosamil (10 μM) induced a rapid and partial inactivation of the current, together with inward rectification of the current measured at the end of the test pulse. 5 In the presence of bertosamil the activation/voltage relationships, steady-state inactivation and recovery from inactivation of It were markedly modified, pointing to changes in the conductance underlying It. 6 We conclude that bertosamil induces rapid inactivation of sustained outward current which leads to an apparent increase in It and decrease in Isus. This effect, which was distinct from the use-dependent inhibition of the outward K+ current, could represent a new antiarrhythmic mechanism. British Journal of Pharmacology (1997) 122, 291–301; doi:10.1038/sj.bjp.0701369

Background. Bidirectional superior vena cava-pulmonary shunt is widely used as an interim palliation for patients with univentricular hearts. Bidirectional inferior vena cava-pulmonary artery shunt, as an alternative approach of partial Fontan circulation, may offer the advantage of performing the complete Fontan circulation more easily due to the already constructed inferior vena cava lateral tunnel. Methods. We used bidirectional inferior vena cavapulmonary artery shunt in 2 patients. Contraindications to a complete Fontan circulation were due to, respectively, a volume-overloaded systemic ventricle and an irregular pulmonary arterial tree. Results. Postoperative courses were uneventful. There were no significant pleural effusions. Transcutaneous oxygen saturations were 77% and 78%. Pulmonary-tosystemic blood flow ratios were 0.57 and 0.63. A complete Fontan circulation was safely performed 8 and 12 months later, without any "Fontan-related" complications. Conclusions. Bidirectional inferior vena cava-pulmonary artery shunt can be useful in selected patients with univentricular hearts, although its place in the field of "partial Fontan operations" cannot be determined as yet.

Objective: To report our experience with perventricular closure of muscular (apical) ventricular septal defects (VSDs) in small infants, with echocardiographic guidance only, in a nonhybrid suite. Methods: Eight infants with nine large muscular (apical) VSDs underwent perventricular device closure in a nonhybrid operating room, with transesophageal and epicardial echocardiography guidance, at a mean age and weight of 3.07 (0.3-7.28) months and 3.7 (2.5-6.2) kg, respectively. Five patients had multiple VSDs. Four had associated cardiac defects. Results: Nine Amplatzer muscular VSD devices with a mean size of 10 (4-14) mm were deployed. Seven patients were discharged from the intensive care unit with a mean length of stay of 8.6 days. Four patients had minimal postprocedural residual shunt; no one had a residual shunt at six-month follow-up. Mid-term results are excellent. Conclusion: Perventricular closure is feasible under echocardiographic guidance only in small patients, even without hybrid suite. This may be a good approach for very symptomatic low-weight infants with apical VSD and may also be useful, in any center, at any time, and in any operating room, to treat an associated apical VSD, even unexpected.

The Ca2+ dye indo 1 was used to record internal Ca2+ (Cai) transients in order to investigate the role of the Na+/Ca2+ exchange current (INa/Ca) in whole cell patch-clamped human atrial myocytes After the activation of the L-type Ca2+ current by test pulses (20 ms) at +20 mV, a tail current (I(tail)) was activated at a holding potential of -80 mV with a density of -1.29 +/- 0.06 pA/pF. The time course of I(tail) followed that of Cai transients I(tail) was suppressed by dialyzing cells with ethylene glycol-bis(beta-aminoethyl ether)-N,N,N',N'-tetraacetic acid, applying 5 mM caffeine, or substituting external Na+ with Li+, indicating that this current was mainly generated by INa/Ca. Two types of action potential were recorded: type A, which is characterized by a narrow early plateau followed by a late low plateau phase, and type B, which is characterized by a small initial peak followed by a prolonged high plateau phase. Type B action potentials were found in larger cells than type A action potentials (membrane capacitance 81.8 +/- 4.5 and 122.4 +/- 7.0 pF in types A and B, respectively, P < 0.001). Substitution of external Na+ with Li+ shortened the late plateau of the type A action potential and the prolonged plateau of the type B action potential. Suppression of Cai transients by caffeine shortens the late part of both types of action potentials, whereas its lengthening effect on the initial phase of action potentials can result from several different mechanisms. The beat-to-beat dependent relationship between Cai transients and action potentials could be mediated by Ina/Ca- Delayed afterdepolarizations were present in a significant proportion of atrial myocytes in our experimental conditions. They were reversibly suppressed by Li+ substitution for Na+, suggesting that they are generated by INa/Ca. We conclude that INa/Ca plays a major role in the development of action potentials and delayed afterdepolarizations in isolated human atrial myocytes.

Background. Bypass grafting for complex forms of coarctation has been poorly documented as an alternative to decrease the high complication rate associated with anatomic repair. Methods. Between mid-1980 and the end of 1994, 16 patients underwent bypass grafting for complex forms of isthmic aortic coarctation. Age ranged from 11 to 49 years (mean age, 28.4 ± 13 years). Indications were atypical anatomic forms of coarctation (n = 12) and reoperation after multiple or complicated previous coarctation repair (n = 4). Lateroisthmic bypass grafts were performed in 14 patients and ascending aorta—descending aorta bypass grafts in 2. Results. There was no hospital mortality. Morbidity consisted of postoperative paradoxical hypertension in 3 patients. There were no spinal cord complications. One death 10 years postoperatively was unrelated to the surgical technique. One patient successfully underwent ascending aorta—descending aorta bypass grafting for a false aneurysm 10 years after lateroisthmic grafting. All patients were asymptomatic and all grafts, patent after a mean follow-up of 5.7 ± 4 years. Conclusions. On the basis of these results, bypass grafting appears to be a safe alternative in this select group of patients. The lateroisthmic bypass graft is the procedure of first choice, and the ascending aorta—descending aorta bypass graft should be reserved for failure of previous lateroisthmic bypass grafting.

SummaryPulmonary atresia with ventricular septal defect (VSD), hypoplastic native pulmonary arteries (PAs) and major aorto-pulmonary collateral arteries (MAPCAs) is a rare and complex congenital cardiac disease. In broad outline, two surgical approaches are available for patients with this condition. The first is characterized by one or several stages of complete unifocalization of the supplying MAPCAs, with or without incorporation of the native pulmonary arteries (PAs), connection of the right ventricle to the ‘neo-Pas’ and, if possible, concomitant or delayed closure of the VSD. The second strategy is based on rehabilitation of the native pulmonary arteries. The first step is a direct right ventricle to native PA connection, to promote the growth of native PAs. The establishment of antegrade flow also allows an easier approach for interventional catheterization, enabling dilatation or stenting of the stenosis and then closure of the communicant collaterals. When the development of the native PAs is satisfactory, the complete repair is performed. If it is necessary to suture a MAPCA to the PA (‘unifocalization’), this is accomplished by connecting the collateral artery to an already developed native branch. Our team developed this multidisciplinary strategy with good results. Based on this experience as well as on the published literature, we describe this strategy of management of patients with pulmonary atresia, VSD, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals (MAPCAs).

The outcomes of homografts (HGs) in the reconstruction of the right ventricular outflow tract (RVOT) in non-Ross patients are often considered disappointing, compared with Ross patients; and the risk factors for HG degeneration are still controversial. The objective of this study was to determine the durability and prognostic factors related to the HGs implanted in non-Ross patients and to propose potential ways to improve the results.A retrospective study (1993-2010) included 115 consecutive non-Ross patients who received a HG for RVOT reconstruction. The median age at implantation was 2.8 years (4 days-58 years). The main heart defects were pulmonary atresia with ventricular septal defect (n = 40; 34%), truncus arteriosus (n = 28; 24%) and tetralogy of Fallot (n = 23; 20%). Thirty-eight percent had preoperative pulmonary hypertension. A low-dose corticosteroid therapy was used during the postoperative period in patients displaying a HG-related inflammatory response (no septic context) (n = 11). The median diameter of the implanted HG was 22 mm (range 9-30 mm). The median age of the HG donor was 14 years (range 0.5-65 years). ABO compatibility rules were not systematically respected for the HG implantation: 43% of the implanted HGs were ABO compatible with the recipient. The endpoints were HG stenosis (peak gradient ≥20 mmHg), regurgitation (moderate or severe), dysfunction (peak gradient ≥ 50 mmHg or regurgitation moderate or severe) and failure (explantation or balloon dilation).Freedom from HG explantation and failure were 89 and 80% at 5 years, and 76 and 69% at 10 years, respectively. HG donor age30 years [hazard ratio (HR): 2; P = 0.012], preoperative pulmonary hypertension (HR: 3; P = 0.02) and HG mismatch (HR: 5; P = 0.04) were multivariate risk factors for HG stenosis, regurgitation and failure, respectively. HG diameter22 mm was a multivariate risk factor for HG regurgitation (HR: 8; P0.001), dysfunction (HR: 9; P = 0.02) and failure (HR: 5; P = 0.03). ABO incompatibility increased the risk of HG stenosis (HR: 4; P = 0.009) and dysfunction (HR: 2; P = 0.04). The use of corticosteroids significantly protected against the risk of HG regurgitation (HR: 0.08; P = 0.04) in the multivariate analysis.The cryopreserved HG implanted to reconstruct the RVOT in non-Ross patients remains one of the most acceptable options in this specific non-Ross population. The outcomes of HGs in non-Ross patients might be improved by implanting an ABO-compatible HG with an adapted diameter, coming from a donor30 years and by optimizing the perioperative afterload of the HG.

The study aim was to determine the dynamics and risk factors related to cryopreserved homografts implanted to reconstruct the right ventricular outflow tract (RVOT) during the Ross procedure.A retrospective study, conducted between 1993 and 2009, included 107 consecutive patients who underwent a Ross procedure with implantation of a homograft (102 pulmonary, five aortic). The median patient age at implant was 18.8 years (range: 2 months - 67 years). The main cardiac defects were congenital aortic stenosis (n = 44), congenital aortic regurgitation (n = 20), and combined (n = 23) and acquired (n = 20) aortic regurgitation. The study endpoints were homograft stenosis (peak gradientor = 20 mmHg), regurgitation (gradeor = moderate), homograft dysfunction (stenosisor = 50 mmHg or regurgitationor = moderate), homograft explant, and failure (explant or balloon dilation). The mean follow up was 5.7 +/- 0.4 years. The statistical analysis was conducted using univariate and multivariate Cox regression tests.The overall patient survival was 97% at 18 years. Homograft stenosis occurred in 54% of patients, regurgitation in 18%, dysfunction in 27%, explant in 5%, and failure in 6%. Freedom from homograft dysfunction was 75% and 55% at five and 10 years, respectively, while freedom from homograft failure was 99% and 84% at five and 10 years, respectively. The main multivariate risk factors for dysfunction were: homograft diameter22 mm (OR: 11, p = 0.019), aortic homograft (OR: 18, p = 0.019) and preoperative right ventricle/left ventricle pressure ratio0.4 (OR: 5, p = 0.018). Univariate risk factors for failure were: homograft diameter22 mm (p = 0.006), donor age30 years (p = 0.03), cold ischemia time2 days (p = 0.04), and decontamination time12 h (p = 0.01).In the Ross procedure, the cryopreserved homograft represents an excellent means to reconstruct the RVOT, with a good long-term longevity. Almost 85% of patients did not require any surgery or percutaneous intervention at 10 years after implantation. The homograft long-term durability depends on both homograft-related and patient-related factors. Pulmonary homografts, with a diameter22 mm, a donor age30 years, and a cold ischemia time2 days are to be preferred.

Background The ideal technique for addressing partial anomalous pulmonary venous drainage into the superior vena cava (SVC), with or without sinus venosus atrial septal defect (ASD), is debated. The risk of sinus node dysfunction, systemic, or pulmonary venous channels obstruction has led to different techniques being developed. We present our experience with 45 patients operated on using a vertical transcaval approach, without atrial or cavoatrial junction incision. Methods Between 2001 and 2010, 45 patients (28 females, 17 males, with a mean age of 5 years (range, 8 months to 70 years), underwent operations using 1 patch of autologous pericardium, after vertical SVC incision anterior to the anomalous pulmonary veins: 43 had associated sinus venosus ASD, and 6 had associated left SVC. Access was through sternotomy in 19 and right posterior thoracotomy in 26. An additional right atrial incision, without crossing the cavoatrial junction, was used in 2 patients without ASD. Mean cardiopulmonary bypass time was 76 minutes. Mean cross-clamp time was 44 minutes. Results No deaths or important morbidities occurred. Mean follow-up was 4.4 years (range, 2 months to 9.3 years). All patients had regular echocardiographic examination, electrocardiogram, and midterm 24-hour Holter electrocardiogram. No new arrhythmias occurred. All patients showed unobstructed caval and pulmonary venous flow. Conclusions The vertical transcaval approach is a simple, highly reproducible technique for correction of partial anomalous pulmonary venous drainage into the SVC. It yields excellent results, with unobstructed pulmonary and systemic venous flow and without arrhythmia development. It can also be performed through a cosmetic right posterior thoracotomy.

Objective: After treatment of transposition of the great arteries (TGA), ventricular septal defect (VSD), pulmonary stenosis, or atresia by various surgical procedures, two main problems led to reoperation: RV—PA conduit or connection stenosis and subaortic stenosis. We report here our mid- and long-term experience of a technique described in 1997 using a segment of aortic autograft as a RV—PA conduit. Methods: Between 1993 and 2005, 25 patients with TGA, VSD, PS, or atresia were corrected with a technique using an intra-ventricular rerouting with conal septum resection (as in reconstruction-endo-ventriculaire (REV)) and interposition of tubular segment of autograft aorta between RV and PA without Lecompte maneuver (as in Rastelli), using in some cases an additional monocusp patch. The patient’s age at correction ranged from 2.5 months to 11 years (mean 2.2 years); seven patients were under 1 year and 13 had a previous shunt procedure. Patients were regularly followed by twodimensional(2D)annualechocardiogram, occasionally bycatheterizationandmorerecentlybyMNR.Results:There wasonepostoperativedeath essentially due to RV failure. There was one late death after 15 years. There were 23 long-term survivors with a mean follow-up of 12.8 years. All patients were in class I category and all of them were in sinus rhythm. Three of them needed a reoperation at 4, 5, and 6 years for subaortic stenosis and one of them with abnormal chordae in the left ventricle (LV) outflow, Only one patient needed a reoperation for RV—PA stenosis, 13 years after correction done at age 2.5 months, the autograft tissue not being implied and found normal at histology. Mean RV pressure by echo at last follow-up was 41 mmHg. Ten patients exhibit a mild-to-moderate pulmonary regurgitation. Freedom from reoperation for RV—PA obstruction is 90%at10and15years.Conclusion:Comparedwithallothertechniques(Rastelli,REV, andNikaidoh)ourautografttechniqueprovidesthebestreported outcome for RV—PA outflow freedom from reoperation, the aortic segment employed being a living tissue susceptible to growth. However, it remains most often a long-term valveless procedure and does not avoid occasional unexpected LV—AO stenosis. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

IntroductionIn our centre, device closure is the first-intention therapy for secundum atrial septal defect (ASD). In difficult cases we use a Meditech sizing balloon to optimize position of the device. We sought to determine (1) the efficacy of this sizing balloon-assisted technique (SBT) and (2) the percentage of ASDs that can be percutaneously closed.PatientsIn 2009, 65 patients (37 female) were referred for secundum ASD closure, at a median age and weight of 27.5(0.8 to 88) years and 40.6(5.6 to 97) kg, respectively. Six had pulmonary hypertension.ResultsEight patients had surgical closure because of referral cardiologist and/or patient's choice. Among the 57 remaining patients, 4 were unsuitable by echocardiography and surgically closed. Transcatheter closure was attempted in 53 cases, including 28 children (53%), and was successful in 50 cases, including 10 with SBT (20%). Amplatzer devices were used in all the patients with a median Amplatzer septal occluder (ASO) size of 20 (10 to 40) mm. A 12 mm ASO could not be positioned in a 5.6 kg infant. Surgical closure was contraindicated for poor general condition (former 26 weeks premature baby with severe bronchodysplasia). Transcatheter closure failed in 2 cases, despite SBT. No major complication occurred. Five patients (10%) had a trivial residual shunt. By univariate analysis, the deficiency of superior rim ( 25mmHg) were associated with failure to percutaneously close the ASD (p = 0.02 and 0.03, respectively). Out of 57 patients candidates for transcatheter closure, 50 were successfully closed percutaneously (87%).ConclusionTranscatheter closure of ASD is successfully accomplished in the majority of the cases. The SBT is safe and useful for device positioning and delivery in patients with large ASDs and deficient superior rim.

The L-arginine-nitric oxide (NO) pathway was investigated in human internal mammary artery (HIMA) in vitro. HIMA rings were mounted in organ bath, and then incubated in Krebs buffer for 1 to 8 hrs, relaxing agents were tested. Under these conditions, L-arginine (0.1 microM - 1 mM) elicited only minor relaxation after 2 hr incubation, whereas with increased incubation time (4, 6, 8 hrs), the concentration-dependent relaxation to L-arginine increased significantly in endothelium-intact and -denuded vessels. NG-nitro-L-arginine (100 microM) or NG-monomethyl-L-arginine (100 microM) or methylene blue (2.7 microM) partially inhibited L-arginine relaxation. In endothelium-intact HIMA and in both types of rings A23187 (10 microM) and L-arginine (100 microM), respectively, increased the concentration of NO in medium and cGMP content of vascular tissues. These increases were partially inhibited by NG-nitro-L-arginine (100 microM) or methylene blue (2.7 microM).in smooth muscle of HIMA L-arginine-NO conversion is calcium independent, which is different from that in endothelium.

The characteristics of cultured myocardial cells isolated from small mammals are well documented, but there is a dearth of data on cultured human cardiocytes. The aim of this study was to determine the main features of myocytes isolated from human atria and maintained in culture in the presence of 10% fetal calf serum (FCS), according to the age of the donor. The following characteristics were analysed: (1) yield and viability; (2) adhesive properties; and (3) changes in cell morphology. Myocytes preferentially adhered to laminin-coated dishes and could be maintained in culture for at least 2 weeks, whatever the age of the donor (which was from 6 days to 85 yr). Maintenance in culture induced morphologic changes characterized by myocyte spreading and changes in myofibrillar organization. Interestingly, the time of onset of these changes depended on the age of the donor: they occurred earlier in young atrial myocytes (1 yr) than in older cells (13 yr).

This study reports the case of a large intramural right ventricular cardiac fibroma, causing a right medio-ventricular stenosis and full loss of consciousness in a 31-year-old female patient, which was successfully treated by enucleation.

Objective: In presenceof adequate pulmonary bloodflow, patients presenting with unoperated or palliated pulmonary atresia with ventricular septal defect (PA/VSD) can reach adult age. However, they remain symptomatic with a limited life expectancy. Methods: Since 1993, 27 patients underwent surgery for unrepaired PA/VSD. Median age was 20 (range: 15—43) years. Nineteen patients had 33 previous palliative procedures whileeight were unoperated survivors.Majoraortopulmonary collateralartery (MAPCA)had beenobservedin all but 2 and were still patentin 23. All bronchopulmonary segments were connected to the native pulmonary arteries (NPA) in 4 (type A), to both NPA and MAPCA in 18 (type B) and only to MAPCA in 5 (type C). The biventricular repair was performed in 17 patients: 3 type A, 12 type B and 2 type C. Ten patients underwent palliativeprocedure:eightaortopulmonary shunt,with unifocalisationin twoand onerightventricleto NPArestrictiveconduit.Results:One (4%) hospital death occurred following the failure of a palliative procedure. No clinical improvement was observed in seven patients including one repaired and six palliated survivors. Two late cardiac death occurred 1 and 7 years after repair. At last visit, 15 of 16 repaired survivors were in NYHA class I or II. Only one patient awaits septation, while eight other with subsequent palliation were considered not repairable. Conclusion: The outcome was encouraging in patients who were eligible for completed biventricular repair. Although considered as unique alternative to cardiopulmonary transplantation, the justification for palliative surgery to improve pulmonary blood flow remains to be established. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.