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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.

The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein’s anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. Only 22 cases are described in the literature. This patient’s antenatal diagnosis of both Ebstein’s anomaly and partial AVCD was made at 25 weeks of gestation. The delivery was organized in a tertiary center. The initial neonatal course was difficult but with adequate treatment, a rapid improvement allowed for a gap of almost 2 years before a complete surgical repair including a cone tricuspid plasty. To our knowledge, this is the first case of antenatal diagnosis, with carefully tailored delivery, neonatal care and subsequent follow-up before indication for successful surgery.

Background: Transcatheter closure for atrial septal defect (ASD) with inferior-posterior rim deficiency has been scarcely reported with proper identification of the indications and limits. We aimed to assess the safety and feasibility of transcatheter closure of ASDs with deficient rims, paying particular attention to cases with inferior-posterior rim deficiency. Methods: From January 2008 to January 2013, 241 patients underwent transcatheter ASD closure, including 50 cases (20.7%) with deficient rims, other than the anterior-superior one. Eighteen patients (12 females) presented inferior-posterior rim deficiency. Their median age was 8 (1.4–85) years and their median weight was 24 [9–97] kg. Transcatheter closure was performed in all cases under transesophageal echocardiography (TEE) guidance in children and intracardiac echocardiography (ICE) guidance in adults. Results: Out of 18 patients with inferior-posterior rim deficiency, only 8 underwent successful immediate transcatheter closure. Four cases failed to be closed. Major complications occurred in 6 patients, including 4 device embolizations, 1 pericardial effusion and 1 complete atrioventricular block that resolved after surgical removal of the device. During a median follow up of 54±13 months, a residual right-to-left shunt was documented in 2 more cases, requiring surgery in one case because of cyanosis. Transcatheter closure was successfully performed in the rest of the 223 patients, including in the 32 cases with deficient rims other than inferior-posterior. Conclusions: Transcatheter closure of ASDs with inferior-posterior rim deficiency cannot be recommended.

Objectives:Extracorporeal membrane oxygenation has become a gold standard in treatment of severe refractory circulatory and/or pulmonary failure. Those procedures require gathering of competences and material. Therefore, they are conducted in a limited number of reference centers. Emergent need for such treatments induces either hazardous transfers or a mobile pediatric extracorporeal membrane oxygenation team able to remote implantation and transportation. The aim of this work is not to focus on pediatric extracorporeal membrane oxygenation outcomes or indications, which have been extensively discussed in the literature. This study would like to detail the implementation, safety, and feasibility, even in a middle-size pediatric cardiac surgery reference center.Patients:This is a retrospective analysis of a series of patients initiated on extracorporeal membrane oxygenation in a peripheral center and transferred to a reference center. The data were collected from 10 consecutive years: from 2006 to 2016.Results:A total of 57 pediatric patients with a median weight of 6.00 (3.2-14.5) kg and median age of 2.89 (0.11-37.63) months were cannulated in peripheral center and transported on extracorporeal membrane oxygenation. We did not experience any adverse event during transport. The outcomes were comparable to our literature-reported on-site extracorporeal membrane oxygenation series with 42 patients (74%) weaned from extracorporeal membrane oxygenation and a 30-day survival of 60%. Neither patient’s age nor weight, indication for extracorporeal membrane oxygenation or length of transport, was statistically significant in terms of outcomes.Conclusion:Offsite extracorporeal membrane oxygenation implantation and ground or air transport for pediatric patients on extracorporeal membrane oxygenation appeared to be safe when performed by a dedicated and experienced team, even within a mid-size center.

The anomalous origin of the left coronary artery from the pulmonary artery in the position of a non-facing coronary sinus is extremely rare. The anatomical position of the ectopic ostia will determine which is the appropriate operative approach to create a dual-coronary supply. This report describes a technique of modified extra-anatomical rerouting using autologous pericardium patch and pulmonary artery flap to create a neo-composite coronary trunk passing anterior to the right ventricular outflow tract.

Extracorporeal life support and heart and/or lung transplant are the last resort in children with end-stage cardiac and/or pulmonary failure and short-term life threaten. Currently, circulatory support is used as a bridge to recovery or as a bridge to transplant but not as a destination therapy. The Excor Berlin Heart is the long-lasting external pneumatic ventricular assist system that is currently available from infancy to adulthood. Long-term prognosis after pediatric cardiac and/or pulmonary transplant is conditioned by the occurrence of graft failure, coronary disease of the cardiac graft, viral infections and bronchiolitis obliterans of the pulmonary graft, the incidence of which increase with time. The scarcity of grafts and the risk of acute rejection due to lack of compliance with immunosuppressive treatment require the transplant specialized teams to choose the best candidates according to psychosocial and biological criteria. The next expected developments concern mainly long-term ventricular assistance with systems that allow for greater autonomy and a return to the child's home.

Background Pulmonary homografts (PH) are used as a first-line treatment for surgical right ventricular outflow tract (RVOT) reconstruction in patients with congenital heart disease (CHD). Despite a better freedom from reintervention than prosthetic conduits, PH are not spared from failure and cases of early dysfunction are regularly described. Aims The aim of this study was to assess the rate of early PH dysfunction in patients of the Bordeaux University Hospital and to identify associated risk factors. Methods A monocentric retrospective study was conducted in children and adults with CHD and PH implantation for RVOT reconstruction. Clinical and echocardiographic data were collected during follow-up. PH dysfunction was defined as a peak of gradient greater than 50 mmHg and/or as pulmonary regurgitation greater than moderate. Early dysfunction was defined as occurring within two years postoperatively. Primary endpoint was the early PH dysfunction rate at 2 years. The dysfunction-free survival curve was calculated according to the Kaplan-Meier method. A logistic regression with univariate then multivariate analysis was performed to identify risk factors for early dysfunction. Results Between January 2002 and November 2020, 112 PH were implanted in 110 patients and 11 cases of homograft dysfunction were reported during the follow-up, including 9 cases of early dysfunction. The rate of early dysfunction was 9.4 [3.3–15.1] % and freedom from reintervention was 94.6 [90.0–99.0] % at two years. The only independent risk factor identified by the multivariate analysis was duration of extracorporeal circulation (P = 0.007) but the length of stay in intensive care unit (P = 0.088) and the initial maximum pulmonary transvalvular gradient (P = 0.06) were also close to significance in the multivariate analysis. Conclusion Although PH provide a durable substitute for RVOT reconstruction, a significant proportion of patients presents early PH dysfunction and requires premature reintervention. An inflammatory mechanism is suspected but dedicated studies should be conducted to validate this hypothesis.

Atresia of the main stem of the left coronary artery is the least observed congenital coronary anomaly; most patients tend to receive a coronary artery bypass graft, although some anatomical corrections have been described. A 17-year-old female patient with left coronary artery main stem atresia underwent a coronary trunk construction with an autologous pericardial patch in our department. At a 3-year follow-up, the patient was asymptomatic, with a normal cardiac stress test. The coronary computed tomography showed no stenosis between the aorta and coronary bifurcation. Long-term patency has yet to be determined.

Plasma troponin is a risk factor for cardiac events in various populations. We evaluated the determinants and predictive value of plasma cardiac troponin I (cTnI) during the long-term follow-up of heart transplantation.We prospectively measured cTnI in 100 heart transplant recipients, 13.0 ± 6.0 years after transplantation, during a routine visit including echocardiography and laboratory measurements. The patients were followed for 21.3 ± 4.9 months after this troponin measurement. cTnI ≥0.006 μg/L (detection threshold) was found in 37 of these 100 patients. Plasma troponin significantly increased with the presence and severity of coronary lesions detected by means of coronary angiography and was significantly associated with age, left ventricular mass, history of post-transplantation heart failure, body mass index, and plasma creatinine. Of 37 patients with cTnI ≥0.006 μg/L, 13 had a cardiac event during follow-up compared with 2 of 63 patients with cTnI0.006 μg/L (P.0001). The relation between cTnI and cardiac events remained significant in multivariate analysis.cTnI is frequently detectable during long-term follow-up after heart transplantation and is associated with chronic graft rejection and renal failure. A detectable cTnI may help identify heart transplant recipients at high risk of cardiac events.

A 21-year-old man with univentricular and uniatrial heart came for a third attempt at ablation of recurrent supraventricular tachycardia. He had undergone a previous cardiac surgery of a Blalock–Taussig shunt, and bidirectional Glenn, and the electrophysiological study revealed an atrioventricular

We present the case of a 4-month-old child with atrioventricular canal associated with severe left atrioventricular valve dysfunction who previously underwent 3 surgical valve reconstructions without significant improvement. A Hybrid Melody valve (Medtronic, Minneapolis, MN) insertion was planned. Because of the low weight, the risk of left ventricular outflow tract obstruction was significant and therefore evaluated preprocedurally using a cardiac computed tomography-derived 3-dimensional printed model. In vitro tests showed good anchorage of the valve without subaortic obstruction and the procedure was then achieved with an excellent clinical result.

Background How reliable is home blood pressure monitoring (HBPM) in heart transplant recipients is not known. Possibly, it may underestimate hypertensive burden, because blood pressure (BP) nondipper profile is frequent among these patients. This prospective study has been designed to determine whether HBPM adequately identifies hypertension in heart transplant recipients. Method We compared HBPM with ambulatory blood pressure monitoring (ABPM) for the diagnosis of uncontrolled hypertension in 74 patients 13.5±6.7 years after heart transplantation. HBPM was measured with a validated semiautomatic device twice every morning and twice every evening on 7 consecutive days, within 15 days of ABPM. We also measured the relationship between HBPM, ABPM, and organ damage as measured by albuminuria and left ventricular mass. Results A nondipper profile was found in 53 (72%) patients. HBPM and ABPM were close according to Pearson bivariate correlations. There was no significant correlation between left ventricular mass and BP either at HBPM or ABPM. Proteinuria significantly correlated with systolic BP either at HBPM (R=0.42; P=0.0002) or ABPM (R=0.25; P=0.03). HBPM adequately classified 61 of 74 (82%) patients as hypertensives or as nonhypertensives or controlled hypertensives. Conclusion Despite a high prevalence of nondipper profile, HBPM gives a reliable estimate of BP burden in most heart transplant recipients. Thus, our results strongly suggest that HBPM is useful for the long-term follow-up of heart transplant recipients.

Background A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium. Methods We retrospectively studied such patients in 5 institutions. Results Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n = 2) and secundum atrial septal defects (n = 3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n = 2), secundum atrial septal closure (n = 2), left upper pulmonary vein stenosis stenting (n = 1), and coarctation stenting (n = 1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40 years of age tend to be symptomatic at presentation (p = 0.056). Conclusion In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients. © 2013 Elsevier Ireland Ltd.

A neonate with absent pulmonary valve syndrome (APVS) and features of tetralogy of Fallot presented respiratory distress. The prolonged need for mechanical ventilation was an indication for surgical repair. Preoperative thoracic CT imaging showed nearly complete closure of the distal trachea, compressed by the aneurysmal dilatation of the pulmonary arteries, and its reopening after ventilator pressure was increased. Management of APVS may require high-pressure ventilation to prevent bronchial collapse due to compression by the pulmonary arteries. In this context, thoracic CT imaging is very useful for adjusting ventilation support so that surgical repair can be performed in optimal conditions.

International audience; Background. - The ageing graft frequently shows coronary lesions and a restrictive physiology. Aims. - To determine the presenting features and outcome of chronic heart failure in heart transplant recipients. Methods. - In this cohort study, we compared 44 consecutive heart transplant recipients who developed chronic heart failure more than 1 year after heart transplantation with 44 control heart transplant recipients who did not develop heart failure. Results. - We found that patients who developed heart failure had more frequently a history of hypertension or diabetes before transplantation. During the 12 months after transplantation, significantly more patients had moderate-to-severe acute rejections (>= grade 2R) in the heart failure group than in the control group. At the time of heart failure diagnosis, systolic left ventricular function was preserved in 50% of patients and coronary angiography was normal or near normal in 36% of patients. Half of the 44 patients in the heart failure group died within 2 years of heart failure diagnosis. Ascites and end-stage renal failure requiring dialysis were significantly more frequent during follow-up in the heart failure group than in the control group (respectively, 10/44 vs 0/44 [P=0.001] and 18/44 vs 5/44 [P=0.003]). Conclusion. - In heart transplant recipients presenting with heart failure, systolic left ventricular function is frequently preserved and coronary angiography is frequently abnormal, but may be normal or near normal. During follow-up, the main features of these patients are a high mortality rate after heart failure diagnosis, a frequent need for renal dialysis and frequent ascites. (C) 2016 Elsevier Masson SAS. All rights reserved.

Background Scimitar syndrome may be corrected using different techniques. Repair using an extracardiac conduit has rarely been performed. This study assessed the intermediate-term outcomes of this technique in adults. Methods From January 2000 to June 2011, 7 adult patients underwent correction with a ringed polytetrafluoroethylene conduit used to connect the scimitar vein (SV) to the left atrium, posterior to the inferior vena cava (IVC). Preoperative and perioperative data were reviewed retrospectively. Results All patients (32 ± 10.6 years old) were symptomatic (3 patients were New York Heart Association [NYHA] functional class II, 4 had recurrent pneumonia), with a ratio of pulmonary to systemic blood flow greater than 2, without significant pulmonary hypertension. In all patients, the SV drained the entire right lung venous return to the IVC below the diaphragm. Surgical repair was performed by sternotomy, normothermic cardiopulmonary bypass, and aortic cross-clamping. Four patients required additional closure of an atrial septal defect. Mean conduit diameter was 14 mm (range, 12 to 16 mm). Patients received long-term platelet suppression therapy with aspirin. There were no deaths and no reoperations. Mean follow-up time was 9.1 ± 3.6 years. Postoperative morbidity was nil. No evidence of subclinical stroke or embolization was found in postoperative magnetic resonance imaging of the brain. No thrombi on the prosthesis or in the left atria were detected at the latest echocardiogram, with laminar flow from the SV to the left atrium. At last follow-up, all patients were in NYHA class I. Conclusions Correction of scimitar syndrome with an extracardiac conduit can be easily and safely performed in adults, with excellent intermediate-term durability, without graft thrombi or stenosis. This technique avoids deep hypothermic circulatory arrest when the SV is short or enters the IVC in an unusually caudad location.

Pulmonary valve replacement (PVR) often is performed in adults with repaired tetralogy of Fallot (TOF). For patients who have tricuspid regurgitation (TR), tricuspid valve (TV) repair associated to PVR is still debated.We sought to evaluate perioperative factors related to TV repair when performed at the time of PVR in patients with repaired TOF.We retrospectively reviewed 104 patients with repaired TOF (or its equivalent) who underwent PVR (2002-2014).Mean age at initial complete correction and at PVR was 20.1 ± 17.2 months and 26.3 ± 9.5 years, respectively. Forty-one patients had significant preoperative TR: 24 were moderate (group M) and 17 were severe (group S). A total of 16 TV repair were performed (8 for each group). Moderate and severe tricuspid regurgitation observed in the first year following the initial complete repair were significantly associated with severe TR at PVR (P .001). In group M patients, TR was improved regardless of TV repair, whereas, in group S, residual significant TR was reported in 7 patients who did not have TV repair. No cases were observed for patients who underwent concomitant TV repair (P = .002). Among these patients with residual significant TR, 2 needed a tricuspid valve replacement. The functional status (New York Heart Association classification) of group S patients was significantly improved by concomitant TV repair.In adults with repaired TOF, TV repair is a safe procedure when performed at the time of PVR. If, at mid-term follow-up, there is probably no benefit of TV repair when preoperative TR is moderate, TV repair may improve both tricuspid valve function and functional status of the patients in case of severe preoperative TR.

The association of an accessory hemidiaphragm and scimitar syndrome is extremely rare and occurs exclusively in the right hemithorax. We present the case of a 27-year-old female, who underwent complete resection of the accessory hemidiaphragm and direct anastomosis of the scimitar vein to the left atrium through a lateral right thoracotomy. This surgical approach offered easy access to both anomalies and allowed single-stage concomitant repair; this approach avoided postoperative respiratory complications and possible torsion or stenosis of the scimitar vein.

stv.2012.0693 Auteur(s) : Mina Amellal amellal_m@yahoo.fr, Bernard Kreitmann Hopital d’enfants La Timone, service de chirurgie cardiothoracique, 264 rue Saint-Pierre, 13385 Marseille cedex 5, France Tires a part : M. Amellal L’anevrisme congenital du ventricule gauche est rare, d’etiologie inconnue, et potentiellement fatal. Nous rapportons l’observation originale d’un enfant âge de 1 an chez qui le diagnostic d’anevrisme congenital du ventricule gauche a ete fait en [...]

Percutaneous closure has evolved to become the first-line treatment strategy for most cases of secundum atrial septal defect (ASD) in both adults and children. Its safety and efficacy have been proved; percutaneous ASD occlusion offers many advantages over surgical closure, including avoidance of cardiopulmonary bypass, avoidance of sternotomy scar, shorter hospitalization, and a potentially lower incidence of postprocedural complications. Periprocedural course and short-term outcome have been widely described, with low mortality and morbidity rates. However, the wide use of ASD closure devices and the growing experience worldwide brought some delayed and rare complications to light. Device thrombosis and cardiac erosion are the most severe late complications of device closure, whereas atrial arrhythmias are the most common. Other delayed complications include nickel allergy, cardiac conduction abnormalities, valvular damage, and device endocarditis. The long-term complication rate is not null and, although rare, some of these complications may be sudden and potentially life-threatening. Moreover, the occurrence and rate of these complications vary with the different devices used currently or in the past. Therefore, both operators and patients need to be aware of these issues to assist them in the choice of intervention or device, or both, and to adapt follow-up modalities. In this review, we sought to describe the type, incidence, and outcome of these rare but potentially serious device closure delayed complications.

International audience; To assess the health-related quality of life (QoL) in children with congenital heart diseases (CHD) with a validated questionnaire in comparison with control children. We prospectively recruited 282 children with CHD aged from 8 to 18 years in two tertiary care centers (France and Belgium) and 180 same-age controls in randomly selected French schools. Children's QoL was self-reported with the KIDSCREEN-52 questionnaire and reported by parents with the KIDSCREEN-27. QoL scores of each dimension were compared between CHD and controls and between the classes of disease severity. Both centers were comparable for most demographic and clinical data. Age- and gender-adjusted self-reported QoL scores were lower in CHD children than in controls for physical well-being (mean ± SEM 45.97 ± 0.57 vs 50.16 ± 0.71, p

ObjectiveThe study objective was to report the long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals.MethodsSince 1991, 20 patients with profound pulmonary artery hypoplasia (mean Nakata index 26 ± 14 mm2/m2) have undergone a medico-surgical strategy of native pulmonary artery rehabilitation to achieve complete repair with satisfactory hemodynamics (right ventricle to aortic pressure ratio

Repair of the complete atrioventricular septal defect, as with many other congenital heart defects, has changed with time from the one-patch technique (Rastelli) to the two-patch technique and then to the “modified” onepatch technique. In this review, we have reported our experience with one-patch Rastelli type correction in babies under one-years of age. Our experience confirmed that the classical one-patch Rastelli technique is a safe, reproducible, teachable technique giving satisfactory late results. Diger bircok dogustan kalp defektlerinde oldugu gibi, komplet atriyoventrikuler septal defeklerin tamiri de zaman icinde tek yama tekniginden (Rastelli) cift yama teknigine ve daha sonra “modifiye” tek yama teknigine degisim sergilemistir. Bu incelemede Rastelli tipi tamir

Endocarditis is uncommon in infants and is exceptionally related to Candida albicans on pulmonary banding. We report on a case in a 7-month-old infant who had pulmonary artery banding for a ventricular septal defect and who presented with candidal endocarditis. Banding was chosen because of the patient's poor trophic and unstable status, which could be risky for surgery involving extracorporeal circulation. A few weeks after the banding, the patient developed systemic Candida infection, which was treated successfully. At 7 months, cardiac failure appeared without fever or inflammatory signs. Cardiac echography showed that the banding was not protective as well as a hyperechogenic image on the pulmonary bifurcation. The angioscan showed a hypodense thrombus. Emergency surgery was performed consisting of pulmonary artery exploration, thrombectomy, and ventricular septal defect closure. The exploration showed a pulmonary artery perforation caused by the infected pseudoaneurysm and the migration of the banding into the pulmonary artery. The anatomopathologic analysis of the vegetation identified multisensitive Candida albicans. After surgery and prolonged antifungal treatment, progression was satisfactory.

Background Considering more recently proposed techniques, we have evaluated our midterm and long-term results of Rastelli one-patch repair in complete atrioventricular canal. Methods Between 1984 and 2005, 107 patients with a complete atrioventricular canal underwent a Rastelli one-patch procedure. Two groups were identified: 1984 to 1995 and 1995 to 2005 (respectively, 56 and 51 patients). Mean age at surgery was 5.3 ± 3.4 months; mean weight was 5.5 ± 3 kg; trisomy 21 was present in 81 patients; complete atrioventricular canal type A was found in 67 patients, type C in 40 patients. There were 12 cases of potentially parachute mitral valve and 14 associated anomalies treated simultaneously (pulmonary obstruction 11, coarctation 3). The coronary sinus was always left on the right side. After functional and anatomic evaluation, the cleft was closed completely in 8 and partially in 29, and was left intact in 70 cases. Results Early survival was 86% ± 3%. Five patients underwent early reoperation for residual ventricular septal defect (n = 2) and mitral valve repair (n = 3). Nine patients underwent late reoperations with successful repair: subaortic stenosis (n = 4) and mitral valve repair (n = 5). Late survival at 10 and 15 years was 84% ± 3%. Freedom from reoperation for mitral regurgitation was 94% ± 3% at 10 years, and 91% ± 3% at 15 and 20 years. At last follow-up 30 patients had mild and 3 had moderate mitral regurgitation. Conclusions Rastelli single-patch repair in complete atrioventricular canal is a safe and reproducible technique. Among survivors, freedom from late reoperation for mitral regurgitation is very satisfactory. A properly taught, learned, and transmitted Rastelli one-patch technique compares very well with any other proposed technique.

We evaluated the results of using extracorporeal membrane oxygenation (ECMO) as resuscitation for cardiac patients undergoing cardiopulmonary resuscitation (CPR) in our setting where neither perfusionists nor surgeons are always on site, and no circuit may be ready. Between 2003 and 2006, we used ECMO for all cardiac patients who underwent cardiac arrest in the pediatric intensive care unit (PICU) or Cath Laboratory. We reviewed retrospectively 14 consecutive files (15 episodes). Mean CPR time before ECMO institution was 44 minutes (10-110 minutes). The surgeons, perfusionist, and scrub nurse, not on site for three of these patients, had to be called in simultaneously with institution of CPR. Two died on ECMO, the third one was successfully transplanted after 5 days. Globally, 10 patients could be weaned (66%). Eight patients (57%) survived to hospital discharge, seven without obvious neurological damage. One patient was bridged to a left ventricular assist device (LVAD) and was eventually successfully transplanted. He had an ischemic brain lesion with good recuperation and no sequel. We obtained good results with resuscitation ECMO in our setting where a permanently on-site rapid deployment ECMO team is not present at all times.

Coronal RARE sequence showing the fetal visceroatrialsitus, assessed in relation to the bronchial tree: the embryologicallynormal left main bronchus (with the stomach on the same side) israther long with no early division (large arrow), whereas the rightone is short, more vertical, and has an early division (small arrow).

We retrospectively reviewed the files of 19 extracorporeal life support (ECLS) applications performed after cardiac surgery in 15 patients from January 2002 to December 2004. We placed 16 arteriovenous ECLS applications with oxygenator, 2 venovenous ECLS applications with oxygenator, and 1 biventricular ECLS application without oxygenator (graft dysfunction after heart transplant). Mean age was 4.9 +/- 7 years (median 5.9 months, range 11 days to 21 years). All patients underwent surgery for congenital heart disease, except for one patient who had a heart transplant. Indications were hemodynamic failure in 12 cases, respiratory failure in 5 cases, and mixed failure in 2 cases. Four patients were undergoing cardiopulmonary resuscitation during ECLS placement (no deaths). Mean delay between surgery and ECLS placement was 3.2 +/- 3.4 days (median 2 days). Mean ECLS duration was 3.4 +/- 5.8 days (mean 6 days, range 3-16 days). Three patients had further surgery for residual lesions. Thirteen patients (86.7%) survived to ECLS weaning; 12 patients survived to hospital discharge (80%). No survivor presented obvious neurologic damage. Specific morbidity included reentry for bleeding, multiple transfusions, and mediastinitis. These results support early placement of ECLS in children whenever a severe postoperative hemodynamic or respiratory failure, refractory to medical treatment, is present.

a Department of Cardiology, La Timone Hospital, Aix-Marseille Universite, Marseille, France b Department of Cardiac Surgery, La Timone Hospital, Aix-Marseille Universite, Marseille, France c Department of Pathology, La Timone Hospital, Aix-Marseille Universite, Marseille, France d Department of Medical Genetics, La Timone Hospital, Aix Marseille Universite, INSERM, GMGF UMR S 910, 13385 Marseille, France

OBJECTIVES We sought to highlight the clinical, morphologic, and pathogenetic features in patients with a cleft mitral valve (MV). BACKGROUND Few studies have addressed the morphologic features of cleft MV and the outcome of these patients. The pathogenetic features, including the developmental relation to an atrioventricular (AV) septal defect, remain unclear. METHODS We reviewed the patients with cleft MV that were diagnosed by echocardiography since 1980. Patients with an AV canal, ventriculo-arterial discordance, and hypoplastic ventricles were excluded. RESULTS Twenty-two patients were identified at a median age of 0.5 years (range 0 to 10.6). In three patients, no chordal attachments of the cleft to the ventricular septum were seen. Ten patients had significant mitral regurgitation (MR), and three had subaortic obstruction by the cleft. Associated cardiac lesions and extracardiac features were present in 13 and 10 patients, respectively. During the median follow-up period of 1.5 years (range 0 to 11.8), two patients died of extracardiac causes, and one neonate died of severe subaortic obstruction. Surgical repair was performed in 10 patients at a median age of 5.2 years (range 1.3 to 10.6). Multivariate analysis showed no predictors for MV surgery. One patient was re-operated for mitral stenosis associated with aortic valve stenosis. Follow-up echocardiography demonstrated moderate MR in two unoperated patients and moderate MV stenosis in two operated patients. CONCLUSIONS A cleft of the MV comprises a wide spectrum. Important morphologic differences exist with an AV septal defect, although the two lesions may be pathogenetically related. Surgical repair always seems possible. Long-term echocardiographic follow-up is warranted.

A right ventricular outflow tract stent was implanted in a 1-month-old (3.5 kg) baby boy with tetralogy of Fallot and worsening cyanosis to relieve infundibular and pulmonary valve stenosis in the setting of well-developed pulmonary artery branches. This management allowed a symmetric and optimal growth of the pulmonary artery branches, with right and left pulmonary arteries measuring 7-mm (z-score, +1.3 SD) and 7.3-mm (z-score, +1.7 SD), respectively, 3 months after stent implantation. No signs of pulmonary overcirculation developed, and complete surgical repair was performed at the age of 6 months.

Objectives: The tight syndrome after open-heart procedures in neonates renders delayed sternal closure (DSC) a life-saving measure. The goal of this study is to analyze the risk factors that may predict the need for DSC. Methods: Between January 1991 and December 2000, 312 consecutive open-heart procedures in neonates (180 males, 132 females) were studied retrospectively. Median age was 11.9 days (range 1–30 days) and weight 3.63 kg (range 1.8–4.2 kg). The major pathologies were transposition of the great arteries (153), interruption of the aortic arch (IAA) (33), total anomalous pulmonary venous drainage (TAPVD) (24) and single ventricle (19). Two hundred and twenty-eight patients had profound hypothermia with circulatory arrest and 74 normothermic cardiopulmonary bypass (CPB), 195 had crystalloid cardioplegia and 111 blood cardioplegia. Median CBP time was 146 min (range 37–284 min) and aortic clamping 67.6 min (range 0– 164 min). Two hundred and fifty-five patients had a continuous ultrafiltration and 57 had a modified ultrafiltration. The criteria for DSC were hemodynamic instability, deterioration of the central venous saturation, metabolic status and/or high ventilatory pressures. Results: One hundred and nineteen patients had DSC (38.12%). Median CBP time was 145 min (range 37–284 min) and aortic clamping time 67.6 min (range 0–164 min). Twenty-one patients (6.7%) needed reopening in the intensive care unit (ICU) during the first 24 h. Among the studied factors, the age below 7 days (P ¼ 0:014), the diagnosis of IAA and TAPVD (P , 0:05), CBP duration over 185 min (P ¼ 0:048), clamping time over 98 min (P ¼ 0:039) and central venous saturation below 51% P ¼ 0:024) were statistically significant risk factors. All the patients who had more than 106 min of clamping, more than 196 min of cardiopulmonary bypass or less than 47% of central venous saturation were either left opened or reopened in the ICU. Conclusions: Many of the factors thought to be associated with the need for delaying the sternal closure had no statistical significance as risk factors. On the other hand, the diagnosis of IAA or TAPVD, an age less than 7 days, aortic clamping more than 98 min, CPB time more than 185 min and a post-bypass central venous saturation less than 51% were statistically significant risk factors that could be used in predicting the need for delaying the sternal closure. q 2002 Elsevier Science B.V. All rights reserved.

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.

Background Right aortic arch with aberrant left subclavian artery is the most frequent cause of vascular ring. Ligamentum arteriosus division opens the ring but leaves the Kommerell diverticulum in place, with a risk of residual compression, aneurysmal dilation, dissection, or even rupture. A procedure consisting of translocation of the aberrant left subclavian artery to the left carotid artery with removal of the Kommerell diverticulum and division of the ligamentum is currently advocated. Methods Between September /2009 and August 2011, 12 consecutive patients underwent the foregoing procedure. Clinical findings, surgical procedure, complications, histopathologic findings, and follow-up data were retrospectively analyzed. Results Mean age at the time of operation was 7.7 years (median, 4.3 years, range, 0.9 to 18.9 years), and mean weight was 26 kg (median, 18 kg; range, 8.4 to 59 kg). All patients had symptoms. Computed tomography scan or magnetic resonance imaging, or both, had confirmed the diagnosis in all patients. Postoperative events, all transient, included chylothorax (1 patient), phrenic palsy (1 patient), pneumonia (1 patient), and the need for prolonged chest drainage (3 patients). Mean follow-up reached 19 months (median, 13 months; range, 12 to 41 months). Mild residual respiratory symptoms were noted in 3 patients. Echo-Doppler analysis showed a patent left subclavian–to–carotid artery anastomosis. Histopathologic analysis of the resected diverticulum (n = 6) showed cystic medial necrosis in four diverticula (focal in one). Conclusions Translocation of the aberrant left subclavian artery with diverticulum resection and ligamentum division is an efficient procedure for symptom relief. Profound wall abnormalities such as medial necrosis in at least 50% of the analyzed diverticula encourage us to maintain this strategy, to reduce the risk of aneurysm formation and dissection.

Surgical approach for persistent ductus arteriosus ligation is typically a left lateral thoracotomy opening the pleural-space with left lung retraction. We describe an alternative approach, with a minimally invasive anterior parasternal incision. This is particularly adapted to preterm infants weighing less than 1.5 kg. This approach ensures a good exposure of vessels. We believe that it is safe, reliable and reproducible. The learning curve should not be an issue for surgeons used to manage low weight patients.

Background Lung or heart-lung transplantation is a useful therapy in life-threatening pulmonary disorders during childhood. Cyclosporine A is a major immunosuppressive treatment but has a number of adverse effects including nephrotoxicity. There have been no reports on the long-term evolution of renal function in a large series of paediatric pulmonary transplantation recipients. Methods. We examined 19 patients followed up for at least 3 years after pulmonary transplantation. The mean time of follow-up was 5.36 years. Kidney function was evaluated by calculation of glomerular filtration rate (GFR) according the Schwartz formula. Results. The GFR was normal before transplantation in all patients. The short-term evolution of GFR was marked by a significant drop during the first and until the 6th month. Then, regardless of the level reached at the end of the 6th month, the GFR remained stable in all patients except one until the end of follow-up. At the end of follow-up, 31% had normal GFR, 57% had mild chronic renal failure, and 5% had advanced renal failure. Hypertension was frequent and associated with renal failure. Conclusions. Paediatric pulmonary recipients showed evidence of long-term cyclosporine A-associated nephrotoxicity. Most of this toxicity occurred during the first 6 months.

Purpose: Acute myopathy of intensive care has been described infrequently in children and never after organ transplantation. We report a case of acute myopathy of intensive care in a child after heart transplantation. Clinical features: An II-yr-old girl, with no previous medical history, developed acute cardiomyopathy leading to cardiac shock. Family history revealed four cases of unidentified myopathy and/or cardiomyopathy. Preoperatively, while muscle biopsy was near normal, myocardial biopsy revealed non specific mitochondrial disorders. A few days after heart transplantation, she developed acute hypotonia and flaccid quadriplegia, consistent with the diagnosis of acute myopathy of intensive care. Nerve conduction studies were normal, electromyography showed myopathic changes and a new muscle biopsy from quadriceps femoris showed severe loss of myosin filaments and ATPase activity in type 2 fibres. A large laboratory screening failed to demonstrate a metabolic disease or a known myopathy. Muscle strength recovered progressively in three weeks allowing home discharge. A few months later, she was free of symptoms and muscle biopsy showed full histopathological recovery. Conclusion: Acute myopathy of intensive care can occur in children after heart transplantation. It should be suspected in the presence of muscle weakness and difficulty in weaning from ventilatory support. Electromyography confirmed a myogenic process and muscle biopsy allowed diagnosis. Full clinical and histopathological recovery usually occur within three weeks.

In non-transplant patients mild hyperhomocysteinemia is an independent risk factor for vascular disease. The aim of this study was to determine whether hyperhomocysteinemia is associated with graft vascular disease. Fasting total plasma homocysteine was assessed in 18 patients with graft vasculopathy and 18 transplanted patients without graft vasculopathy matched for age, sex and the time since transplant. All were on cyclosporin. Graft vasculopathy was defined at coronary angiography as stenoses ]25%, or aneurysms. We found that hyperhomocysteinemia (] 15 mmol:l) is common among transplanted heart recipients and significantly more frequent in the patients with graft vasculopathy (17:18 versus 11:18). Accordingly, the mean homocysteinemia was significantly higher in the group with graft vasculopathy (23.69 7.8 versus 16.99 7.1 mmol:l, P 0.01). The elevation of homocysteine plasma levels in the heart transplant recipients has probably multiple causes. The main cause seems to be renal failure. Additional causes could be azathioprine treatment or genetic polymorphisms. These results suggest that besides the immunological factors, homocysteine can play an additional role in the pathogenesis of graft vascular disease. © 1998 Elsevier Science Ireland Ltd. All rights reserved.

Background: It is well recognized that a right ventricle (RV) may eventually fail if working as systemic. This has led to arterial switch operation for transposition of the great arteries (TGA), but more recently to anatomic correction of ‘corrected transposition’ or atrio-ventricular discordance (AVD)combining an atrial switch and a ventricular outflow repair (arterial switch or Rastelli type). Methods: We report here 8 cases of such anatomic correction of AVD in patients from 3 months to 10 years. Of these patients, 6 had situs solitus, and 2 situs inversus; 7 patients had a ventricular septal defect (VSD); 5 had an increased pulmonary flow, and 3 had a decreased pulmonary flow with pulmonary stenosis (PS) or pulmonary atresia (PA). In all, 6 had discordant and 1 had concordant ventriculo-arterial (VA) relations, and 1 had double-outlet RV, 2 had an associated coarctation, and 1 had a cor triatriatum. Of the 8 patients, 5 had pulmonary artery hypertension, 3 a moderate to severe tricuspid insufficiency and 6 had undergone one or more previous operations. Correction was done between 3 months and 10 years of age, with 7 having a Senning procedure (twice left-sided) and 1 a Mustard type operation. VA outflows were repaired with: 4 arterial switches, 1 1eft ventricle to aorta rerouting, 1 right ventricular outflow tract (RVOT) repair and 2 Rastelli. A tricuspid annuloplasty was done once. Results: All patients survived the operation, but 2 patients needed a reoperation (both successful): late obstruction of pulmonary venous channel after a left-sided Senning, and residual VSD closure. Mean follow-up is 45 months (3‐122). Of the 8 patients, 6 patients are in sinus rhythm, 2 have junctional rhythm. A patient with junctional rhythm and occasional tachycardia died suddenly 18 months after surgery 1 week after satisfactory evaluation. All have a normal left ventricle function as evidenced by echocardiography. Conclusions: Despite a more demanding procedure, the ‘anatomic repair’ of hearts with AVD is possible, even in infancy, with good early and mid-term result. Despite the potential late rhythmic problems of the Senning operation (somehow more frequent with AVD in our experience), it may become, with increasing experience, the procedure of choice in corrected transposition. © 1998 Elsevier Science B.V. All rights reserved.

Isolation of the left subclavian artery is a rare aortic arch anomaly. Association with a right interrupted aortic arch and a double patent ductus arteriosus is exceptional. We report a case of such an association with successful surgical correction.

We describe a patient in whom we found dual orifices in a straddling and overriding tricuspid valve, with two normally sized ventricles and a cleft in the mitral valve. The patient underwent successful surgical repair. We discuss the concept of “double-orifice right atrium”, as well as the need to differentiate the isolated cleft of the morphologically mitral valve from the zone of apposition between the left ventricular components of the bridging leaflets seen in the setting of atrioventricular septal defect with common atrioventricular junction. We emphasise the unusual association of these abnormalities of the right and left atrioventricular valves in patients with separate atrioventricular junctions.

We present the case of an infant with congenital heart disease which includes a partial atrioventricular canal defect with the absence of the atrial septum (common atrium) and an extremely uncommon course of the right-sided superior vena cava (SVC) including an intra-atrial segment coursing intramurally along the right posterolateral atrial wall, with an intracardiac orifice situated low within the right side of the atrium, close to the atrial orifice of the right hepatic veins. This feature of the anatomy was discovered intraoperatively at the time of surgical repair. Systemic venous anatomy also included interrupted inferior vena cava (IVC) with azygos continuation to a left-sided SVC draining directly into the left side of the atrium. The successful surgical procedure included tunneling of the left-sided SVC to the right side of the common atrium and atrial septation with a patch.